We present a 62-year-old female with no significant past medical history until 1992, when she was diagnosed with breast cancer. She was initially treated with surgery plus adjuvant chemo and radiation therapy. Contralateral disease was detected in 2010 with progressive node and pulmonary involvement. The patient proved intolerant to second-line chemotherapy (carboplatin plus paclitaxel) and was started on VNR in August 2014. The first cycle was uneventful, and a second course of treatment was administered in September. Four days later, she began complaining of exercise intolerance and lack of strength in the lower limbs, followed by dyspnoea, and was seen at our emergency department 11 days after the second cycle. She was afebrile and reported no sputum production. Blood gas analysis showed severe hypoxemia (PO2/FiO2 ratio ~100) with concomitant respiratory acidosis. A contrast-enhanced chest computed tomography (CT) revealed diffuse ground-glass opacities in both lung fields, with no images suggesting pneumonia or any change in the previously known lung metastasis. Two sets of blood cultures were taken, and she was started on ceftriaxone plus azithromycin. Urinary antigens for Streptococcus pneumoniae and Legionella were negative, as was a nasopharyngeal swab for the influenza virus. On the next day, she presented severe respiratory distress despite administration of high-flow oxygen. Echocardiographic assessment of left ventricular function revealed no abnormalities. Because she maintained a good functional status, she was admitted for a trial of non-invasive bi-level ventilation (NIV), with a positive inspiratory pressure of 18 cm H2O and expiratory pressure of 10 cm H2O. She required FiO2 between 0.7 and 1 to achieve saturations of 89–91%. VNR toxicity was considered, and steroid therapy (prednisolone 1 mg/kg/day) was added. Due to persistent respiratory failure, antibiotic therapy was empirically changed to ceftazidime and vancomycin, with no improvement. A bronchoscopic evaluation was considered but discarded due to poor risk:benefit ratio. A second chest CT on the 10th day after hospital admission revealed persistent ground-glass opacities. Steroid therapy was intensified to methylprednisolone 1 g/day for three consecutive days. Yet there was no consistent improvement, and she remained fully dependent on NIV. A new chest CT on the 22nd day of hospitalization revealed no significant change in ground-glass opacities (Fig. ). In this setting, considering the absence of additional organ dysfunction or infection, we decided to start cyclophosphamide (CYC). She received a first dose of 1 g on the 24th day. Progressive improvement was noted, with decreasing respiratory rate and improving gas exchange (PO2/FiO2 ~150). Weaning from NIV was started five days after CYC. Chest CT scanning after 13 days showed improvement of the ground-glass infiltrates (Fig. ), and 14 days after CYC, she was breathing independently, requiring only oxygen by face mask. She was transferred to the ward 44 days after being admitted to the intermediate care unit with improving respiratory function. A second dose of CYC was administered one week later, and she was discharged home eight days after that with a fraction of inspired oxygen of 31% (PO2/FiO2 ~ 230). Her chest X-ray, on discharge, was clearer. One month later, she was seen in the outpatient clinic and remained well. In early January, 2015, she was admitted with a respiratory infection and aggravated hypoxemia, with no signs of pneumonia in the chest X-ray. Although responding initially to antibiotics, she developed a respiratory nosocomial infection and died after 19 days of inpatient hospital care.


A 57-year-old male with an extensive history of tobacco use was transferred to our institution with a left middle cerebral artery (MCA) syndrome and computed tomography (CT) angiogram evidence of a carotid terminus and M1 segment occlusion. The patient received intravenous tissue plasminogen activator at the outside institution within 90 min of his stroke onset. On arrival to our facility, within 2½ h of symptom onset, the patient's National Institutes of Health Stroke Scale score was 21. The initial CT of the head at the outside institution revealed an Alberta Stroke Program Early CT Score of 10.
The patient was taken emergently to the angiography suite for attempts at mechanical thrombectomy. After placement of an 8 French femoral sheath, the left ICA was accessed with an 8 French Flowgate balloon guide catheter (Stryker Neurovascular; Fremont, California) over a 6 French Penumbra Select Berenstein catheter (Penumbra Inc.; Alameda, California) over a Glidewire (Terumo; Somerset, New Jersey). The balloon guide catheter was positioned within the proximal cervical ICA. After removal of the Penumbra Select catheter and Glidewire, initial angiograms demonstrated a left supraclinoid ICA occlusion [] without evidence of cervical ICA injury during catheter manipulations [Figure and ].
The balloon was inflated, and proximal aspiration thrombectomy was attempted twice using manual aspiration with a 60 mL syringe through the balloon guide catheter as described in the initial case report.[] A follow-up angiogram after these two attempts demonstrated minimal recanalization of the ICA terminus; however, there was evidence of a new iatrogenic dissection within the ICA just proximal to the skull base [Figure and ]. We proceeded with multiple attempts at mechanical thrombectomy using a combination of stent retrievers and direct aspiration at the clot face but were only able to achieve a modified thrombolysis in cerebral infarction (mTICI) grade 1 recanalization of the ICA terminus and anterior cerebral artery distribution []. There was no significant flow past the M1 segment.
Following the procedure, the patient was admitted to the neurocritical care unit for observation. A follow-up CT head demonstrated a large ischemic territory within the left MCA distribution consistent with the continued M1 segment occlusion []. The patient's examination findings remained poor, with a dense left MCA syndrome. There was no significant improvement in the patient's neurological status during the hospitalization. After lengthy discussions with the family, the patient was placed on comfort care, and he died on the poststroke day 6. Patient consent for publication as a single case report was waived by the institutional review board.


A 33-year-old woman referred to the emergency ward of Vali-Asr Hospital with complaints of amenorrhea and irregular spotting. Her past medical history was unremarkable. The patient had one cesarean delivery and one spontaneous abortion 3- and 1-year ago, respectively. She was at 7 weeks of gestation with last menstrual period date. Her pregnancy was spontaneous and twins. A recent transvaginal ultrasound revealed dichorionic diamniotic twin pregnancy with normal fetal heart rate. She experienced a sudden vaginal bleeding and one fetus was aborted.
Vaginal ultrasound was performed and the demised fetus was detected in the lower segment of the uterus with a subchorionic hematoma. Uterine curettage revealed products of conception. She had amenorrhea for 3 months after curettage, but irregular spotting occurred and continued for 1 month. Consequently, the patient was referred to Vali-Asr Hospital. On admission, there was no significant abnormality on her physical examination and vital signs were stable. Only spotting from closed cervix was detected on speculum examination. The laboratory results were as follows: Hemoglobin 11/2 mg/dl (11-14), hematocrite 34/1 (34-38), and beta-hCG 67 mIU/ml.
A Doppler vaginal ultrasound showed that the uterus size was 35×49 mm and the endometrial thickness was 5 mm. There was a heteroechoic and hypervascular mass from previous cesarean section scar (). Serum beta-hCG level also raised to 93 mIU/ml one week later. There were several differential diagnoses, such as cesarean scar pregnancy, retention placenta accreta, and gestational trophoblastic disease. Actinomycine was prescribed to the patient because it was believed that the above-mentioned differential diagnoses were sensitive to chemotherapy. Four courses of actinomycine 2 mg were administered in consecutive 2 weeks. The patient had a strong desire to save her fertility and did not accept surgery. After three courses of actinomycine, the serum beta-hCG level returned to zero. Hence, the remaining course of chemotherapy was continued. Two weeks later, the patient presented severe vaginal bleeding. On admission, the blood pressure was 100/70 mm Hg, pulse rate 110 per minute, and the cervix was closed. However, there was severe vaginal bleeding on speculum examination. The uterus and adnexes appeared normal size. The laboratory tests were as follows: Hemoglobin 8/3 mg/dl, hematocrite 23/7, platelet count 153,000 and coagulation profile was normal, and beta-hCG was 0/7mU/ml.
The patient received 2000 cc normal saline infusion and 2 units packed red blood cells. Doppler ultrasound exhibited hypervascular heteroechoic mass 33×41 mm, similar to previous cesarean section scar. There was no evidence of regression in vascularity or size of the mass located in the cesarean section scar in comparison with the imaging before chemotherapy. Vascular resistance index (RI) was 0/37 and the sinologist suggested placental site trophoblastic tumor. There were no metastatic lesions in other parts of the body, such as liver, lung, and brain. After consultation, the patient agreed with hysterectomy. Laparatomy was performed and no significant lesion in abdominopelvic exploration was observed. There was a normal size uterus with a regular surface, except for the previous cesarean section scar location, which was slightly enlarged and protruded. Total abdominal hysterectomy was performed and the ovaries were preserved to save the endocrine function. On the cut section of the specimen, a brownish irregular friable mass was detected (). The operation and the recovery period were uneventful and the patient was discharged after 4 days.
On microscopic examination, histopathological dominant pattern was the large trophoblastic cells with eosinophilic cytoplasm and pleomorphic nuclei that invaded the myometrium (interdigitating pattern of myometrial invasion) with strong immunoreactivity for human placental lactogen (HPL) and focal immunoreactivity for beta-hCG absence of immunoreactivity for p63 (a useful feature in the differential diagnosis with epithelioid trophoblastic tumor). The Ki67 (a proliferating marker) was up to 12%. The depth of invasion to myometrium was 85%. The mitotic rate was 7 per 10 high power fields. Finally, PSTT was confirmed (). Serum beta-hCG and HPL were checked consecutively for 8 months. All measurements were in the normal range and the patient is presently symptom-free without any evidence of the disease. Written informed consent was obtained from the patient.
A 33-year-old woman referred to Vali-Asr Hospital with complaints of irregular abnormal uterine bleeding. She had two pregnancies within 7 years. The first was a cesarean delivery due to malpresentation and the second (9 months before admission) was a miscarriage that led to curettage at 8 weeks of gestation. Her past medical history was unremarkable. On admission, the patient was stable and there were no significant issues in her routine examination. In vaginal speculum test, cervix was mid and closed with short spotting from it. The uterus and adenxes were not tender in deep bimanual palpation. The laboratory results were as follows: Hemoglobin 12/3 mg/dl, hematocrite 35/8, platelet count 143,000, and beta-hCG 3,664 mIU/ml.
Vaginal sonography revealed an empty uterus cavity with a closed cervix. Endometrial thickness was 12 mm. There were no abnormalities in the tubes or ovaries, but there was a focal heterogeneity 41×30 mm in the previous cesarean section scar that had a low resistance index (RI) in Doppler waves (RI: 0/29). Magnetic resonance imaging (MRI), with and without contrast, also confirmed the mass 40×30 mm in the previous cesarean section scar.
Based on laboratory tests and imaging evidence, cesarean scar pregnancy (CSP) was diagnosed. In the absence of CSP treatment, according to a standard protocol, chemotherapy was recommended. Beforehand, the patient was debriefed regarding the advantages and disadvantages of chemotherapy. Methotrexate 1 mg/kg (68 mg) was administered intravenously every 48 hours and the serum beta-hCG level was measured sequentially. On the first day of the chemotherapy, the serum beta-hCG level was 3,664 mIU/ml; however, it was reduced to 794 mIU/ml on the 8th day and was checked again a week later. Unfortunately, despite a good response to methotrexate along with 4 courses of chemotherapy, the serum beta-hCG level raised to 4,325 mIU/ml 7 days later. Ultrasonography was performed and there was no change in the size of the mass and was still hypervascular. The distance to the serosal surface was 2 mm and deep penetration to myometrium was detected. Considering the ineffectiveness of chemotherapy, a surgery was planned for the definite treatment of CSP. The patient was consulted regarding the consequence; however, she had no desire to preserve her fertility and accepted the inevitability of hysterectomy during the operation. A midline incision was used to access the abdominal cavity. There were no abnormalities in the abdominopelvic exploration. The ovaries and tubes appeared normal and there was a bulged (largest size: 5 cm) in the anterior and inferior segments of the uterus just below the bladder reflection. The parametrium was fragile, particularly on the left side. The serosal surface was intact, but due to the location of the mass, uterus preservation seemed impossible. Consequently, total abdominal hysterectomy was performed. Considering the patient’s age, the ovaries were saved to preserve endocrine function. The dissected uterus is shown in . The recovery period was uneventful and the patient was discharged 13 days after hospitalization. The serum beta-hCG level on the third day after hysterectomy was 30 mIU/ml.
Histopathological examination revealed soft, dark-red, and hemorrhagic mass invading the uterus cervix and lower segment measuring 4×5×3.5 cm. It filled the full thickness of the uterus cervix wall and parametrial tissue and all surgical margins were free. Microscopically, there was plexiform proliferation of cytotrophoblasts and cyncytiotrophoblasts with the frequent presence of hemorrhage and necrosis without any villi. On immunohistochemistry (IHC) staining, it was positive for the beta-hCG marker and Ki67 (a proliferation marker) was 85%. Hence, as shown in , cervicoisthmic choriocarcinoma was diagnosed.
Brain, lung, and liver cut scans were performed to detect metastatic lesions. Based on the international federation of gynecology and obstetrics (FIGO) staging system for GTD, the patient was in stage 1 and the score was 5 (low-risk). Therefore, single-agent chemotherapy was administered. After the first course of actinomycine, the serum beta-hCG level returned to zero and thus the chemotherapy was continued for 3 additional courses. Currently, the patient is asymptomatic and followed up by a monthly measure of the serum beta-hCG level. Written informed consent was obtained from the patient.


A 43-year-old male with EC was referred for a 1-month history of decreased vision in his left eye. The patient had symptoms of dysphagia and weight loss for 4 months. The specimen was confirmed as ESCC from the esophagogastroduodenoscopy. Computed tomography showed a tumor in the middle esophagus and enlargement of lymph nodes without evidence of adjacent invasion or distant metastasis. With the diagnosis of ESCC, Stage IIIB, the patient received preoperative concurrent chemoradiotherapy, followed by total esophagectomy and reconstruction. He denied any history of neither ocular disease nor intraocular surgery.
The patient presented with visual deterioration in the left eye 1 month after surgery. At presentation, his best-corrected visual acuity was 20/20 OD and 20/100 OS. The anterior segment evaluation revealed no inflammation. Dilated fundus examination showed normal appearance in the right eye. In the left eye, however, vertical concentric folds between disc and mottled macula with diminished foveal reflex were found. An elevated yellow choroidal tumor, in size of seven-disc diameters, occupied posterior pole extending through temporal quadrant. There was neither vitreous opacity nor disc edema in the left eye [].
The inferiotemporally located choroidal tumor had an width of 17.0 mm and a thickness of 5.7 mm measured by B-scan ultrasonography (Echoscan US-4000; NIDEK, Gamagori, Japan) []. Spectral-domain optical coherence tomography (OCT) (SPECTRALIS OCT; Heidelberg Engineering GmbH., Heidelberg, Germany) showed a bulky choroidal mass with subretinal fluid []. Fluorescein angiography (SPECTRALIS scanning laser angiography; Heidelberg Engineering GmbH., Heidelberg, Germany) demonstrated pinpoint hyperfluorescent dye leakage of the choroidal tumor in the early phase, and fluorescein pooling into subretinal space in the late frames [Figure and ]. Indocyanine green angiography showed a blockage of background staining over the tumor area [].
Magnetic resonance imaging depicted a hypointense T1- and T2-weighted focal lesions on the walls of the left eyeball with contrast enhancement, and an abnormal diffusion restriction on diffusion-weighted imaging and apparent diffusion coefficient map []. Positron emission tomography and computed tomography showed a singular hypermetabolic focus in the left eye []. With the above findings, left choroidal metastasis from EC was impressed.
After discussion with his oncologist, palliative chemotherapy with cisplatin and fluorouracil was started. Concerning the rareness of distant metastatic site, in addition, radiation of 57.60 gray (Gy) in 48 fractions covering the field of choroidal metastatic tumor was also applied through tomotherapy. Other critical organs were within the normal range of a tolerable dose. After 3 months of treatments, the size of choroidal tumor reduced, the surface of the lesion flattened, and pigmentary change of retinal pigment epithelium (RPE) over the tumor area was noted []. Cup-to-disc ratio in the left eye remained 0.2, and the neuroretinal rim had a uniform width without pallor. No further elevated lesion was seen by B scan []. OCT scan revealed attenuation of outer retinal layers with indistinguishable ellipsoid zone and interdigitation zone and irregular clumps of RPE/Bruch's membrane complex []. His visual acuity remained 20/20 OD and 20/100 OS. There was neither recurrence nor new choroidal lesion during 5 months of follow-up.


A 10-year-old male patient reported to our institution with the chief complaint of painless progressive swelling in the anterior region of the maxillary jaw since one and a half year. The preliminary case history revealed that the patient had undergone surgical excision of a small swelling in the maxillary anterior region of the jaw 2 years back at a private hospital. On histopathological examination, the lesion was diagnosed as PsJOF. After one and a half years, the patient developed swelling in the same region, which was rapidly increasing in size. At the time the patient reported to our department, the lesion was of massive size. Elicited medical and family history did not reveal significant findings. Extra-orally, upon inspection, the swelling extended anteriorly up to the right canine region, posteriorly up to the left side zygomatic bone, superiorly up to left infraorbital margin and inferiorly up to the alveolus and measured 6x8 cm in size. The overlying skin appeared normal (). Palpatory findings revealed the swelling was hard in consistency and non-tender. On intra-oral inspection, a huge swelling extending from the maxillary right canine up to the maxillary left first molar obliterating the nasolabial fold with no visible secondary surface changes was noted (). On palpation, the swelling was hard in consistency, non-tender, and had no palpable pulsations. Tooth mobility was noted in relation to 21, 22, C, 11 and 12.
The orthopantomogram revealed a ground glass appearance with ill-defined borders over the anterior region of the maxilla extending to the left quadrant (). The maxillary central and lateral incisors showed displacement of the crowns as well as roots. Furthermore, the un-erupted maxillary right canine and left premolar showed evidence of displacement. The computed tomography scan showed an expansile lesion involving the alveolar arch of the left maxilla and extending into the pre-maxillary region and hard palate. The central lesional area showed focally distributed radio-dense areas. The lesional soft tissue was found to be extended into the left nasal cavity (). Fine needle aspiration cytology showed numerous red blood cells and occasional spindle-shaped cells suggesting benign nature of the lesion. The histopathological examination of incisional biopsy showed a highly cellular lesion predominantly composed of fibroblast-like spindle cells interspersed with varying degree of mineralization in the form of spherical ossicles. The spherical ossicles were acellular and had a basophilic center and an eosinophilic peripheral fringe resembling psammoma bodies (). Large vascular and sinusoidal spaces engorged with red blood cells and devoid of endothelial cell lining were also noted (). Based on the clinico-pathological correlation, a final diagnosis of recurrent PsJOF with secondary ABC was made.
Under general anesthesia, infiltration of 2% xylocaine with 1:100,000 adrenaline was given after the marking the Weber Ferguson’s incision. Lateral rhinotomy incision with horizontal infraorbital component and midline lip-split was done. The sublabial incision was performed after splitting upper lip in the midline to facilitate elevation of the flap from the anterior wall of the maxilla. The incision was extended through the entire buccogingival sulcus up to maxillary tuberosity. The horizontal component of Weber Ferguson’s incision was performed 1 mm below the infraorbital rim and the flap was reflected. The bilateral palatal cut was given after the reflection of palatal mucosa and zygoma cuts were given to disarticulate the bilateral anterior maxilla and the complete tumor was removed. The palatal prosthesis was inserted and fixed to posterior maxilla at the permanent molar region bilaterally. The closure was done in layers. The histopathological evaluation of the surgically excised specimen was consistent with the diagnosis of PsJOF with secondary ABC.
The obturator placed intra-operatively in the maxillectomy cavity was removed after 5 days. Following the first week after the surgery, healed facial incision was noted. A transient obturator with artificial denture was placed in the cavity with the support of the remaining teeth after 3 weeks of surgery. After six weeks of surgery, the patient was given an artificial denture as an obturator with missing anterior teeth. Thereafter, the patient is in regular followup for past 3 years with no recurrence.


A 73-year-old male was referred to our hospital presenting with bilateral vocal fold palsy and a rapidly growing anterior neck mass. We performed ultrasound sonography, revealing a bulky tumor in the thyroid gland and multiple bilateral cervical lymphadenopathies. We performed fine-needle aspiration cytology from both the thyroid tumor and the cervical lymph node, both of which revealed SCC. We found no primary tumor in the pharynx, larynx, or esophagus with laryngoscopy and upper gastrointestinal endoscopy, nor could we discover any primary tumor in the trachea using bronchoscopy. We discovered a bulky thyroid tumor and bilateral cervical lymph nodes involved in the common carotid artery and brachiocephalic artery using computed tomography (CT) with contrast effect (Fig. ). Using FDG-positron emission tomography/CT, we found FDG uptake in the thyroid, the bilateral cervical lymph nodes, the lungs, the mediastinal lymph nodes, and the vertebra (Fig. ). We diagnosed the patient with stage IVC primary SCC in the thyroid and initiated treatment with weekly paclitaxel. He received chemotherapy weekly at 90 mg/m2 according to a 6 weeks on/2 weeks off schedule.
Four months after the initiation of treatment, the objective response obtained from CT presented a stable disease. However, a slightly enlarged tumor resulted in dysphagia and aspiration pneumonia, which led to the patient's death. We performed an autopsy according to the patient's wishes. On a macroscopic level, we found that the greater part of the thyroid was replaced by carcinoma. The tumor and the cervical lymph node metastases involved the common carotid artery and the brachiocephalic artery, but not the trachea or larynx (Fig. ). The tumor had slightly invaded the external muscle layer of the esophagus; the mucosa of the esophagus, however, was intact. We observed multiple tumor nodules in the bilateral lungs and mediastinal lymphadenopathies. These macroscopic findings indicated primary thyroid cancer with distant metastases.
On examining the histopathology of all of the carcinomas in the thyroid, cervical lymph nodes, and lungs, we observed a palisade arrangement, intercellular bridges, and keratinization with a cancer pearl (Fig. ). We interpreted these histopathological features as pure highly differentiated SCC. Consequently, only in the thyroid was primary SCC proven and never in any other organ. We performed immunohistochemical examinations of the primary thyroid cancer, the cervical lymph node metastases, and the lung metastases; all of which were negative for TTF-1, Tg, and PAX8 (Fig. ).


We conducted this study in compliance with the principles of the Declaration of Helsinki.
A 64-year-old woman underwent a bilateral blepharoplasty of the upper eyelids combined with bilateral ptosis repair. The preoperative ophthalmologic examination showed no abnormalities. Visual acuity was 0.7 (20/30) and 0.6 (20/35) in the right and left eye, respectively. The intraocular pressures were within normal ranges, at 17 mm Hg and 18 mm Hg in the left and right eye, respectively.
The procedure was performed under local anesthesia by infiltrating 1% xylocaine (lidocaine HCl; Aspen Pharma Trading Limited, Aspen Netherlands B.V., Oss, the Netherlands) and 1:200,000 adrenaline with sodium bicarbonate. In addition, 0.04% topical oxybuprocaine (4 mg/mL; Bausch and Lomb OPS B.V., Schiphol-Rijk, the Netherlands) drops were administered. Postoperatively, the upper eyelids were cooled for 20 minutes every hour. The patient was discharged the same day. Hypromellose drops (0.3% hypromellose drops; several manufacturers) and carbomer eye gel (2 mg/g; Bausch and Lomb OPS B.V.) were prescribed and the patient was advised to take oral analgesics as needed.
Two days after the procedure, the patient presented to the Maxima Medical Centre (the Netherlands) with blurred vision preceded by a sudden sting in the right eye and a subsequent persisting unilateral headache on the right side. Upon clinical examination, irritated conjunctivae and redness of the right eye were observed. Pupil reactions were not tested due to the mild presentation. Conjunctivitis was suspected, so Tobradex ointment (1 mg/g of dexamethasone, 3 mg/g of tobramycin; Alcon Nederland B.V., Breda, the Netherlands) and oral analgesics were prescribed. Two days later, the periocular pain had decreased, but the patient’s vision had deteriorated to the sole ability of light and dark discrimination. Upon an ophthalmologic examination conducted by an ophthalmologist, corneal edema and a fixed dilated pupil of the right eye were observed. Visual acuity was 0.01 on the right side. The intraocular pressure of the right eye had increased to 55 mm Hg and a shallow anterior chamber was seen. Acute ACG of the right eye was diagnosed. The glaucoma was immediately treated pharmacologically, followed by an iridectomy 3 days later.
Upon follow-up 2 weeks later, the patient’s vision in the right eye was still poor. In the following weeks, an iridectomy was also performed in the left eye due to a shallow anterior chamber angle, in order to prevent future acute ACG. At a 1-year follow-up examination, the patient’s eyesight had been regained.


A 39-year-old Caucasian female had been diagnosed with juvenile-onset recurrent respiratory papillomatosis (JORRP) at the age of 3 years, and several polypoid lesions’ resections were performed at follow-up every 3 months from then on. She denied smoking and drinking alcoholics. She started presenting with shortness of breath and chest pain over the last three years, when the diagnosis of the pulmonary involvement by the papillomatosis was made with the aid of a thoracic computed tomography (CT). At her last hospitalization — 6 days before her death — she presented to the emergency room complaining of worsening of shortness of breath, chest pain and weight loss. A new CT scan was performed showing a worsening pattern of the pulmonary invasion, and a working diagnosis of bronchopneumonia. She was prescribed antibiotics, and a transbronchial biopsy was made. Her clinical status worsened with progressive respiratory failure accompanied by sepsis and death.


A 15-year-old girl sought the Pediatric Surgery Clinic complaining of a non-tender, slow-growing mass on her right supraclavicular fossa, which, on the physical examination revealed to be enlarged lymph nodes, which were hard and immobile ().
Her past medical history included an adenoidectomy at the age of 13 years due to a cleft palate and a nasopharyngeal stricture. A supraclavicular lymph node lesion was diagnosed, and a biopsy was performed at the external center; however, the patient revisited the center 4 months later with a regrowth of the lesion. This was surgically removed, but 2 years later the lesion had regrown and was surgically removed for the third time.
Histology results revealed the diagnosis of CD associated with CFT. The patient’s physical examination was normal except for the bulging mass over the supraclavicular fossa. Considering her past history, the working diagnosis was a relapse of the CD. Therefore, another surgical procedure was undertaken to remove the new tumor, which measured 5 × 5 × 2.5 cm. At this time, the histopathological findings of the lymph node sections showed increased lymphoid follicles and atretic germinal centers, as well as a proliferation of the internal and external vessels inside and outside the follicles. A concentric mantle zone consisting of small lymphocytes was observed around the follicles ( and ).
The histochemical and immunohistochemical examination showed the tumor cells of the CFT component staining focally with SMA and CD34, while the collagen fibers were stained diffusively with Masson’s trichrome (). The tumor cells diffusively expressed vimentin, the plasma cells CD38, the histiocytes CD68, the endothelium CD31, the mast cells stained with Toluidine Blue, and the connective tissue with PAS. EMA, ALK, Elastic stain, and Congo red were negative. The morphological, histochemical, and immunohistochemical findings altogether supported the diagnosis of CFT associated with CD.


A 57-year-old woman presented with a history of upper abdominal pain and distention for 5 mo. She had no parasitic infestations, allergic diseases, signs and symptoms of eosinophil-mediated tissue injury, and personal or family history of gynecologic malignancy. Various treatment modalities including proton pump inhibitor, antibiotics, and antispasmodics could not relieve her symptoms. The patient was transferred to our hospital for further evaluation and treatment. On physical examination, the abdomen was distended and tender with diffusely shifting dullness present and slight upper abdominal tenderness; there was no sign of rebound tenderness. Laboratory investigations showed the following values: Hgb, 119 g/dL; PLT, 343 k/mL; WBC, 12.8 k/mL; differential: segmentonuclear neutrophils, 10.0%; lymphocytes, 21.2%; monocytes, 2.7%; eosinophils, 65.6%. Serum electrolytes, coagulation studies, and thyroid tests were normal. Parasitic infestation was excluded by repeated negative stool examinations. Chest X-ray examination and ECG were negative. Abdominal ultrasonography showed massive ascites without any organ abnormalities, including the uterus and ovaries. Abdominal paracentesis revealed 1.8 L of hemorrhagic peritoneal fluid with a low serum albumin-ascitic gradient. Microscopy showed abundant white cell counts in the fluid, which were predominantly eosinophils. The ascitic fluid cytology was negative for malignancy, and cultures were negative for acid-fast bacilli, and bacterial and fungal infections. For diagnostic purposes, endoscopy of the upper gastrointestinal tract was performed, which showed erosions, exudates, and mucosal rings in the duodenal mucosa (Figure ). Simultaneously, the rectal mucosal exhibited erosions, hyperemia, and swelling on colonoscopy (Figure ). Colonoscopy examination revealed no lesions in the proximal colon and the ileum. Biopsies were taken from the duodenal lesion. Histological examination demonstrated characteristic histological findings of eosinophilic infiltrate at approximately 25 eosinophils per high power field in the duodenal mucosa (Figure ). The patient was negative for Helicobacter pylori determined using 13C-urea breath testing. Unfortunately, there was no pathological examination of the rectal lesions. Computed tomography (CT) imaging also showed peritoneal fluid, but, more importantly, an accompanying local mild thickening of the right rear rectum wall (Figure ).
Based on our findings and taking into account possible differential diagnoses, we diagnosed the patient with EG characterized by simultaneous mucosal involvement of the duodenum and serosal involvement of the rectum, which rarely presents with massive hemorrhagic ascites. Since the patient refused steroid treatment, ketotifen 1 mg bid po was administered for 1 mo and the patients rapidly responded with a complete resolution of ascites after 2 wk. Moreover, there was a remarkable decline in peripheral eosinophil counts. The patient recovered well and was free from gastrointestinal symptoms and had no recurrence of ascites during the 1-year follow-up period.


We report the case of an 18-year-old female who presented with chief complaints of swelling over the upper back since birth and spastic weakness of bilateral lower limbs with an inability to walk since 2 years. On examination, the swelling was soft-to-firm, nontender, midline in location, which started increasing in size at puberty and progressed since then to the present size of 14 cm × 12 cm []. Three-dimensional (3D) computed tomography scan of the dorsal spine was suggestive of a wide bony defect in the posterior spinal elements from D3 to D9 vertebrae. Diastematomyelia was also seen from D3 to D7 vertebrae []. Magnetic resonance imaging (MRI) of the dorsal spine was suggestive of a complex spinal dysraphism with lipomeningomyelocele and diastematomyelia []. At surgery, the patient's accessory breast was removed, lipomatous tissue and bony septum were excised, and dural repair was done. An elliptical vertical skin incision was taken incorporating the reductant skin along with the three nipples. The entry of the fibrolipomatous stalk through the fascial defect was identified. A large lipomatous mass along with the accessory breast was amputated at the level of the stalk to help in the visualization of the spinal cord–lipoma interface. Two hemicords with a dorsal type lipomeningomyelocele was identified []. The penetrating intradural lipomatous tract was identified all around. The osseocartilaginous median septum was drilled. Normal dura of both the hemicords was opened and the openings were carried caudally towards the penetrating tract. Circumferential dissection around the lipomatous tract was continued until the dura was separated completely. The dural opening was then continued caudally. The fatty mass was resected with the help of cavitron ultrasonic surgical aspirator (CUSA). Neurulation of the neural placode was then done. Water tight dural closure (duraplasty) was done for both the hemicords separately with the help of a graft. Histopathological examination was consistent with accessory ectopic breast with lipomeningomyelocele [].
The postoperative period was uneventful. Spasticity and power in the lower limbs improved and the patient was able to walk with support at the time of discharge.


A 65-year-old previously healthy female was admitted to the hospital with chief complaints of high-grade fever, generalized body ache, and decreased appetite for 3 days, followed by breathlessness and altered sensorium for 2 days. Her vital parameters recorded on examination were pulse – 140/min, blood pressure – 90/60 mmHg, respiratory rate – 60/min, temperature 102°F, and pulse oximetry 60% on room air. She also had diffuse erythematous macular rash on the trunk and peripheral extremities. Neurological examination revealed Glasgow Coma Score (GCS) of 10 without any signs of meningeal irritation. Pupillary size and reaction were normal with bilateral extensors plantars. Other systemic examinations were unremarkable. She was found to have anemia (hemoglobin 8 g/dl), hematocrit 41%, and thrombocytopenia (90,000/cm3) with normal total leukocyte count and coagulation profile. Dengue nonstructural (NS1) antigen (ELISA kit, PanBio Diagnostics, Brisbane, Australia) as well as IgM dengue antibody (Immunochromatographic kit, Standard Diagnostics Inc., Yongin, Korea) tests were detected positive in blood, whereas IgG antibody was negative. Serologies for malaria, chikungunya, leptospira, typhoid, and scrub typhus were negative. Rest all laboratory investigations were unremarkable. Her chest skiagram revealed bilateral infiltrates involving all zones suggestive of acute respiratory distress syndrome (ARDS). She was immediately placed on invasive mechanical ventilation in view of acute hypoxemic respiratory failure. The working diagnosis of ARDS secondary to dengue infection was established. Fluid resuscitation was started along with hemodynamic monitoring followed by subsequent resuscitation with vasopressor support. She was managed with ventilator strategy that comprised low tidal volume and high positive end-expiratory pressure settings with target fraction of inspired oxygen (FiO2) ≤0.6 to maintain SaO2 above 90%. On the 2nd day of hospital stay, her general condition further worsened as her GCS dropped to 5, bilateral pupils sluggishly reacting to light, and vasopressor requirement further increased. She also had two episodes of generalized tonic–clonic seizure. Serial platelet count was 65,000/cm3. She was given one unit of packed red blood cells and six units of random donor platelets. Emergency noncontrast computed tomography revealed dilated bilateral lateral, third, and fourth ventricles showing hyperdense content of blood attenuation suggestive of intraventricular hemorrhage along with linear hyperdensities along bilateral sulcal spaces diffusely and along tentorium cerebelli as well as falx cerebrum suggestive of subarachnoid hemorrhage shown in –. There was no midline shift. Fundus examination revealed no evidence of papilledema. Contrast magnetic resonance imaging including angiography excluded other sources of ICH, such as arteriovenous malformations, aneurysm, or dural fistulas. The patient was managed conservatively to maintain hemodynamic stability and repeat platelet transfusions were done. However, there was progressive deterioration and succumbed to death on the 3rd day of hospital stay.


A 68-year-old Caucasian male presented to the emergency department (ED) with a painless, draining left buttock lesion. He reported that the wound had originated as a pimple a few months earlier, but had been growing over the last three weeks with foul-smelling discharge. There was no history of trauma to the area. His past medical history included a deep vein thrombosis/pulmonary embolism 20 years prior for which he was on warfarin, hyperlipidemia and tonsillectomy. On physical exam, the patient’s vitals were significant for blood pressure elevated to 153/87. On the left gluteus, a 10 × 10 cm2 non-tender lesion was noted with malodorous discharge. Labs were only significant for a therapeutic international normalized ratio of 3.07. A computed tomography (CT) of the pelvis was read as having a 9 × 10.8 × 4.2 cm3 left gluteal abscess and bulky left inguinal lymphadenopathy (Fig. ). The patient was admitted, started on intravenous antibiotics and taken to the operating room (OR) for unroofing, debridement and incisional biopsy (Fig. ). Pathology revealed ulcerated, necrotic skin fragments with MCC and cultures grew Enterobacter cloacae, Haemophilus parainfluenzae, Peptostreptococcus anaerobius, Peptostreptococcus asaccharolyticus. Antibiotics were de-escalated and the patient was discharged with sodium hypochlorite wound dressing changes. A chest CT was performed and showed no evidence of distant metastasis. One month later, the patient then underwent complete operative excision of the lesion down to the gluteus fascia with 2 cm margins and left groin lymph node dissection with lymphadenectomy. The resulting 13 × 15 cm2 defect was closed with a combination of fasciocutaneous advancement flaps and split-thickness skin grafting (Fig. ). Pathological analysis showed a completely excised 11.6 cm ulcerated MCC lesion with negative margins and positive lymphovascular invasion (2/2 positive lymph nodes)—stage pT3 pN1b (Fig. ).
A hematology oncology consult was appreciated and per their recommendation the patient completed a course of 6120 cGy pelvic to buttock radiation over 2 months. Hyperbaric oxygen therapy, an established treatment for non-healing wounds within a radiated field [, ], was offered, but the patient declined. The buttock wound was noted to be healed at the 5-month postoperative follow up (Fig. ). A follow-up CT of the chest, abdomen and pelvis displayed increasing supraclavicular, mediastinal and retroperitoneal lymphadenopathy. The patient elected to follow up at a specialized clinical trials center and received pembrolizumab.


A 76-year-old male with a history of hypertension and diabetes mellitus presented with New York Heart Association Class III chest pain. Body mass index was 30.48 kg/m2. He was diagnosed to have triple-vessel disease. Preoperatively, room air ABG showed PO2 of 66 mmHg and PCO2 was 44 mmHg. Serum lipids were within normal range. Creatinine was 1.3 mg/dl. Electrocardiography (ECG) showed incomplete right bundle branch block (RBBB). Two-dimensional echo revealed basal inferior wall mild hypokinesia, Grade 3 diastolic dysfunction and LV ejection fraction was 55%. He was on telmisartan, atorvastatin, metoprolol, oral hypoglycemic agents, and aspirin (150 mg). Aspirin was given till the day of surgery. He had undergone off-pump CABG surgery with four grafts (left internal mammary artery to left anterior descending artery, radial artery to obtuse major one and obtuse major two, and saphenous venous graft to distal right coronary artery [RCA]). Intraoperatively, transesophageal echocardiography (TEE) was done; findings were similar to preoperative echo findings. During surgery, patient had stable hemodynamic. Blood sugar levels were maintained below 180 mg/dl. He was shifted to Intensive Care Unit on ventilator with dobutamine 5 mcg/kg/min. Intermittent pneumatic compression devices were applied for 5 days to both lower limbs to prevent deep venous thrombosis (DVT). Aspirin was given within 3 h after surgery. He was extubated after 12 h. Blood drainage in first 12 h was 800 and 600 ml in next 24 h. Patient had renal dysfunction and respiratory distress with basal atelectasis of lungs. He was kept on noninvasive ventilation after extubation for 4 days. Nasogastric tube was passed through nostril to avoid aerophagia on Bi-pap, but patient had nasal trauma and bleeding while inserting nasogastric tube. Hence, we did not give heparin for DVT prophylaxis. On 5th day postsurgery, he was hemodynamically stable, off inotropes and off oxygen. Patient was mobilized in chair. Suddenly, he experienced breathing difficulty, perspiration, tachycardia, and desaturation. He was re-intubated. His systolic blood pressure (BP) dropped down to 50 mmHg and saturation to 60% with FIO2. Initially, heart rate increased to 130 and then dropped to 50 beats/min. ECG showed incomplete RBBB without any ST changes. This was followed by electromechanical dissociation. Chest compressions were given. Peak airway pressures were low (Paw 14 cmH2O) with 600 ml tidal volumes. There were no secretions in endotracheal tube. Central venous pressure was 14 mmHg. Patient was kept on high inotropic supports. Saturation never increased beyond 70% during cardiopulmonary resuscitation. Cardiopulmonary resuscitation was continued for 45 min. BP never came up above 50 mmHg. Hence, patient was supported with intra-aortic balloon pump (IABP). Patient slowly improved in 10 min. TEE showed right atrial and ventricular dilatation with severe right ventricular (RV) hypokinesia and severe global hypokinesia of left ventricle with akinesia of basal inferior wall but no thrombus in the right atrium or in pulmonary artery. There was no evidence of cardiac tamponade. Troponin T Card test was positive, so RCA graft dysfunction was thought. But looking at clinical presentation, APE was suspected. D-dimer levels were checked and found to be very high 10,600 ng/ml. X-ray chest showed haziness on the left side. Postresuscitation, patient was in comatose state for 4 h; hence, computed tomography pulmonary angiography (CTPA) was delayed. He had dense left-sided hemiplegia on arousal. His CTPA showed thrombus in left segmental pulmonary arteries [] and CT brain showed right middle cerebral artery infarct. Patient was treated initially with unfractionated heparin and later with Warfarin. He was extubated after 3 days. Patient improved neurologically and was able to sit in chair. He was discharged from hospital on 20th day. Patient came walking at 3-month follow-up.


A 31-year-old male patient presented in the Emergency Department complaining of abdominal pain of rapid onset during the last 24 h, located mainly in the right scrotum, and mildly in the right iliac fossa. He mentioned mild nausea, but no vomiting or bowel dysfunction, and normal body temperature. His medical history included bilateral open inguinal hernia repair as an infant.
Clinical examination revealed tenderness in the right scrotum, with no palpable masses in the inguino-scrotal area; no specific signs for acute appendicitis were present. Laboratory blood examination showed an elevated white blood cell count (15.13 × 103/μL), as well as CRP levels (4.56 mg/dL). Ultrasound was negative for testicular torsion and fluid in the rectovesical pouch, but confirmed epididymitis, therefore, the patient was admitted to the Urology Department for the administration of antibiotics and analgesics. The following day the patient’s condition deteriorated, showing a further increase in CRP levels (17.52 mg/dl). Clinical examination revealed a positive McBurney’s sign and rebound tenderness. A second ultrasound was requested, whose findings were supportive of appendicitis, and the decision of surgical treatment was made.
Initially, under general anesthesia, a McBurney incision was performed, but an ischemic inflammatory mass and serosanguineous fluid were found; therefore, exploratory laparotomy through midline incision was considered necessary. The mass, identified as twisted omentum (Fig. ), was dissected after ligation of the omental pedicle (Fig. ). Additional appendectomy was performed to prevent any future diagnostic problems, while the rest of the abdominal cavity showed no underlying pathology or signs of adhesions.
Post-operative recovery was uneventful and the patient was discharged 5 days after the surgery in good health condition. Histopathologic examination of the dissected omental mass showed extended hemorrhagic necrosis, accompanied by inflammatory infiltration, whereas the appendix (of 6.5 cm in length), presented serosal appendicitis secondary to the intra-abdominal inflammation.


A 17-year-old girl with a body mass index of 23 and menarche at age 13 presented to her primary paediatrician’s office for abnormal uterine bleeding since age 14. Her menstrual bleeding occurred 1–3 times per month, lasted 3–7 days and was painful. At age 15, she was referred to a gynaecologist for management because her irregularities worsened over time. A pelvic ultrasound, von Willebrand factor, complete blood count, platelet, anaemia profile, thyroid stimulating hormone, prothrombin time, partial thrombin time, factor 8, follicular stimulating hormone, luteinising hormone, oestradiol, 17-progesterone were all normal. Her Dehydroepiandrosterone (DHEA) sulfate was mildly elevated 411 (normal 37–307). For the following 15 months, she was treated serially with Depo-Provera injections, OrthoEvra (ethinyl estradiol and norelgestromin) patches without improvement, and Estarylla contraceptive pill (CP) was started 1 month prior to presentation without improvement.
At the time of re-presentation to her paediatrician’s office, despite being on a CP, she had four periods of bleeding in the month prior, with breaks lasting 1–4 days in between periods. She used up to 10 heavy pads per day, with frequent flooding. She reported pain of 8/10 with menstruation, large clots up to 2 cm in size, headache, dizziness and five episodes of syncope. At the time of presentation, she shared that despite good compliance with medication, she had noted no improvement in her menses and was becoming hopeless about ever improving her symptoms. She asked what else she could try to regulate her menstruation.
At this time, the author, who is the patient’s paediatrician and is also fully trained and dual licensed in traditional Chinese medicine (TCM), offered acupuncture treatment to manage her DUB with severe dysmenorrhoea. Based on TCM diagnostic criteria, the patient was diagnosed with abnormal uterine bleeding due to ‘heat and phlegm with blood stasis’. The treatment principle is to ‘clear heat, reduce phlegm’, reduce stress, ‘invigorate blood’ and ‘fill her conception (menstrual) channels’. The patient had not heard of acupuncture prior to the treatment, but after a trial of one needle, she was willing to commit to the treatment.


A 34-year-old male patient was admitted to the Oral and Maxillofacial Surgery Service of the Adriano Jorge Hospital with a history of increased volume in the left infraorbital region [] and painful complaint. At the physical examination, a firm, palpable, sessile, adherent nodule with a slow evolution of about 2 years was observed.
Puncture had a negative result. An incisional biopsy of the lesion was performed under local anesthesia [], where it was obtained a suggestive diagnosis of malignant schwannoma.
After requesting complementary examinations of bone scintigraphy and CT [Figures and ], extensive bone remodeling was observed in the left maxilla. Due to the clinical characteristics and histopathological examination suggestive of a malignant tumor and in combination with the findings of the complementary examinations, total left maxillectomy was performed by Weber Ferguson access under general anesthesia and orotracheal intubation [Figures –]. The surgical specimen was sent for histopathological analysis.
Histopathological examination showed densely collagenous connective tissue infiltrated by a great number of large pleomorphic, sometimes multinucleated, histiocytoid cells with nuclei of varying sizes. Fragments of bone tissue were also observed with innumerable areas of active resorption [].
The patient is about 3 years of preservation and underwent multidisciplinary treatment with the manufacture of bucomaxillary prosthesis and awaits new surgery for rehabilitation [Figures –].


A 12-year-old entire male domestic shorthair cat was referred with a 5 week history
of intermittent vomiting, loss of appetite and weight loss. A mid-abdominal mass had
been identified at the referring practice on abdominal radiographs and ultrasound,
with the origin not determined. The cat’s previous history included chronic
arthritis being treated intermittently with the non-steroidal anti-inflammatory drug
(NSAID) meloxicam (Metacam; Boehringer Ingelheim), which was stopped by the
referring practice prior to presentation. The cat had been in remission from
diabetes mellitus for 2 years.
Blood work performed at the referring practice showed mild anaemia (haematocrit [HCT]
25.7 l/l; reference interval [RI] 30–45 l/l) that was not further characterised as
blood film examination or reticulocyte evaluation was not performed. A limited
biochemistry profile showed azotaemia, with creatinine at 254 μmol/l (RI 53–177
μmol/l) and urea of 11.5 mmol/l (RI 5.4–12.5 mmol/l). A urinalysis was not
performed.
An in-house SNAP ELISA for feline leukaemia virus and feline immunodeficiency virus
was negative. Thoracic radiographs were unremarkable. An ultrasound-guided
fine-needle aspirate of the mass was performed, which showed heavy blood
contamination with a moderate number of large lymphocytes and reactive macrophages.
The sample was considered non-diagnostic.
On presentation at the University Teaching Hospital, Sydney, the cat was bright and
responsive, weighed 5.2 kg (body condition score 2/5) and had lost 900 g of weight
over the previous month. On physical examination, a grade III/VI parasternal
systolic heart murmur was auscultated. A large (6 × 4 cm), slightly irregular and
firm mass was palpated in the mid-abdomen. The left kidney was palpably smaller than
the right. The remainder of the physical examination was unremarkable.
A repeat abdominal ultrasound (Phillips EPIQ5; Phillips Medical Systems Netherlands)
using linear array and curvilinear array (5 Mhz) transducers identified a 50 × 67 mm
lobulated heterogeneous mass in the mid-abdomen adjacent to the mesenteric vessels
(). The
ileocolic lymph node was mildly enlarged (short axis 6 mm) with normal sonographic
appearance. Nephrolithiasis and a decrease in size of the left kidney and presumed
compensatory hypertrophy of the right kidney were identified. Cytological
examination of the mass and the enlarged ileocolic lymph node by ultrasound-guided
fine-needle aspirate showed blood contamination and was considered inconclusive.
Ultrasound-guided core biopsies were obtained under sedation using a 14 G Tru-cut
biopsy needle (Becton Dickinson Australia).
Histopathological evaluation of the Tru-cut biopsy revealed a non-encapsulated
proliferation of irregular bands of pleomorphic spindle cells forming irregular
blood-filled spaces and interspersed with multiple foci of lymphoid tissue (). The cells had large
round-to-angular, hyperchromic basophilic nuclei with 1–2 nucleoli, displaying
marked anisokaryosis and a mitotic index of 2/10 high-power fields (). The cells had a
moderate amount of pale eosinophilic cytoplasm with indistinct cell boundaries. The
diagnosis of HSA was made based on the cytological features of malignancy in the
cell population forming vascular spaces.
The cat was started on oral metronomic chemotherapy with compounded 5 mg oral
cyclophosphamide (Bova Compounding Chemist, 16.6 mg/m2 daily). The cat’s
owner reported that the cat did well at home after starting treatment. There was one
single vomit and loss of appetite for 2 days that resolved on temporary
discontinuation of medication for 3 days. Four weeks after treatment started, the
cat’s appetite increased with only occasional vomiting (1–2 times a month). The cat
started gaining weight 6 weeks after commencing cyclophosphamide and maintained this
for 8 months. The abdominal mass was palpated to be similar size on physical
examination.
A complete blood count performed every 4–8 weeks revealed a mild, non-regenerative
microcytic, normochromic anaemia (HCT 24–28 l/l [RI 30–45 l/l]; mean cell volume
34–37 fl [RI 40–45 fl]), mild persistent thrombocytopenia (217–289 ×
109/l; RI 300–700 × 109/l) with lymphocytopenia (0.3–0.68 ×
109/l; RI 1.6–7 × 109/l). A biochemistry panel performed 6
weeks post-treatment revealed persistent but stable azotaemia (creatinine 256 μmol/l
[RI 90–180 μmol/l], urea 15 mmol/l [RI 7.2–10.7 mmol/l]) and hyperphosphataemia
(2.82 mmol/l [RI 1.3–2.3], not measured previously). The cat was clinically stable
at this stage and was diagnosed with International Renal Interest Society stage 3
chronic kidney disease, based on its creatinine level. A commercial kidney diet
(Hill’s Prescription Diet k/d) was recommended.
Three months into treatment, the cat developed acute onset of sneezing, which
resolved spontaneously with a presumptive diagnosis of viral upper respiratory
disease. The cat developed alopecia from self-mutilation, over the flanks, 4 weeks
into treatment; the alopecia resolved spontaneously. Eight months after treatment
started, a repeat abdominal ultrasound was performed, which showed that the
abdominal mass was similar in size (53 × 45 mm) with mild progression of
mineralisation and heterogeneity (). There was enlargement of multiple mildly heterogeneous
ileocolic lymph nodes (largest one 20 × 30 mm), suggestive of metastatic disease.
There was mild free peritoneal fluid and also mild progression of chronic changes in
both kidneys. The cat continued to be clinically well until 10 months after
diagnosis, when it lost 300 g within a month. The cat presented for acute onset of
anorexia and weakness 10 months after treatment started. On physical examination,
the cat was tachycardic and tachypnoeic with weak femoral pulses. There was also
evidence of fluid wave in the abdomen suspected to be due to haemorrhage. The cat
was euthanased 6 days later owing to failure of improvement in clinical signs.
Post-mortem examination of the cat was declined by the owner.


A 23-year-old woman was diagnosed with AA by the end of 2010. In recent years, she has not had any immune system-related serious illnesses and conditions, such as asthma, allergies, and atopic dermatitis. Because of her condition, she experienced psychological changes in the workplace. She was initially treated with mometasone furoate, a topical corticosteroid, and oral cepharanthine to enhance blood flow. Despite compliance to treatment, AA progressed to diffuse AA. Initially, she was treated with intralesional triamcinolone acetonide (40 mg) five times per week between January and February 2011; triamcinolone acetonide has a potency that is five times that of methylprednisolone. However, her symptoms persisted.
She was referred to the dermatology department of our university hospital in June 2012. The result of the pull test at the margin of the alopecic lesions was positive. As further treatment to resolve alopecia, narrowband ultraviolet B phototherapy and squaric acid dibutylester (SADBE) as a local autoimmune treatment were administered as an outpatient for four courses between June 2011 and March 2013. Her response was erratic. Therefore, systemic corticosteroid pulse therapy was administered (). The first course of 1,500 mg corticosteroid therapy in the hospital was administered in May 2013. The first course comprised 500 mg methylprednisolone administered intravenously for 3 days. However, the clinical recurrence of extensive AA, nearly alopecia universalis (), was observed 3 months after the first course. She was administered repeated corticosteroid pulse therapy until the end of 2013. A second course of 1,500 mg corticosteroid therapy was administered for 3 days, but her symptoms did not improve. After the third course of the same corticosteroid pulse therapy, extensive alopecia finally improved to small diffuse alopecia. In March 2014, SADBE treatment was started again as an outpatient. By September 2015, SADBE treatment was replaced by administration of 25 mg/day prednisolone, which was gradually tapered down to 5 mg/day. Her alopecia was resolved through the multidisciplinary approach to the treatment of AA.
In February 2016, she complained of right groin pain immediately after sustaining injury in the right leg after almost falling off her bicycle. She was referred to our department for orthopedic evaluation 1 month after the incident. Steroid-induced ONFH was suspected because of prior repeated systemic corticosteroid pulse therapy. Physical examination and MRI scan of the bilateral hip joints showed abnormal signs. The range of motion of the right hip joint was restricted in the direction of flexion, abduction, and internal rotation. Pain in the right groin increased when standing or climbing up the stairs. The T1-weighted MRI of the hip showed the characteristic band pattern on the right and a large diffused low signal area in the left femoral head. Based on these findings, she was diagnosed with stage three postcollapse ONFH on the right and stage one precollapse ONFH on the left (). Thereafter, she complained of continuous and severe right hip joint pain. THA was scheduled and performed for the right ONFH in March 2017. For the left ONFH, she wanted a joint-preserving surgery, wherein core decompression and autologous bone graft were performed (). One year postoperatively, three clinical scores in her hip improved (University of California, Los Angeles [UCLA] activity score, 4; Oxford hip score, 19 points; and Harris hip score [HHS], right: 90.0 points, left: 85.5 points) compared with those preoperatively (UCLA activity score, 2; Oxford hip score, 41 points; HHS, right: 36.0 points, left: 46.0 points). The postoperative course went well, and the patient became ambulatory with a single cane and was discharged after rehabilitation therapy. At the Department of Dermatology, 5 mg/day corticosteroid was administered as maintenance dose for AA. The patient was carefully followed up as an outpatient if the symptoms on the left side worsened.


A 51-year-old male living in a rural part of western Norway was hospitalized after 2 weeks of influenza-like symptoms including fever, mild headache and a reduced general condition. During the last week before hospitalization, his wife had noted abdominal distention. Otherwise, no focal symptoms were noted. Two days prior to admittance, he started oral penicillin because of bacterial sinusitis. Previous medical history was significant for alcohol abuse, pancreatitis and alcoholic liver cirrhosis.
At admittance, he had memory and orientation impairment. Physical examination revealed blood pressure 133/85 mmHg, heart rate 107 beats per minute, temperature 40.3°C, respiratory rate 24 per minute and oxygen saturation 97%. He had scleral icterus and the abdomen was significantly distended although he denied abdominal pain. Laboratory tests showed leukocytes 8.8 × 109/l (references: 4.3–10 × 109/l), platelet count 26 × 109/l (references: 145–348 × 109/l), hemoglobin 13.8 g/dl (references: 13.4–17.0 g/dl), international normalized ratio (INR) 1.1 (references: <1.1), activated partial thromboplastin time 41 s (references: 28–48 s), sodium 133 mmol/l (references: 137–145 mmol/l), potassium 3.2 mmol/l (references: 3.5–5.0 mmol/l), magnesium 0.56 mmol/l (references: 0.71–0.94 mmol/l), creatinine 58 μmol/l (references: 60–105 μmol/l), alanine aminotransferase 59 U/l (references: <70 U/l), alkaline phosphatase 171 U/l (references: <105 U/l), C-reactive protein (CRP) 123 mg/l (references: <5 mg/l), gamma glutamyl transferase 2016 U/l (references: <115 U/l), amylase 59 U/l (references: >120 U/l), bilirubin 42 μmol/l (references: 5–25 μmo/l). Screening for human immunodeficiency virus, hepatitis B and C antibodies were all negative and serum antinuclear antibodies and antineutrophil cytoplasmic antibodies were not detected. Computer tomography (CT) of the skull and brain revealed signs of sinusitis. Ultrasound of the liver was consistent with cirrhosis and ascites. Blood cultures were taken before cefotaxime was prescribed for his sinusitis and a potential spontaneous bacterial peritonitis. In addition, he was given thiamine prophylaxis against Wernicke’s encephalopathy.
Over the next 6 days, he developed decompensated liver failure, CRP increased to 200 mg/l, bilirubin to 159 μmol/l and INR to 1.5. Microbiological cultures were negative. A systemic autoimmune condition was considered unlikely because of negative immunological tests. He was persistently febrile, confused and developed an increasing oxygen demand. A CT scan of his thorax and abdomen showed bilateral pneumonia and right-sided pleural effusion. Thoracentesis revealed one liter of bloody pleural fluid and a bronchoscopy including a bronchial alveolar lavage (BAL) was performed. Because of liver cirrhosis, he was considered to be at risk for infections with atypical and opportunistic microbes. However, BAL polymerase chain reaction (PCR) was negative for Pneumocystis jiroveci and Legionella pneumophila. Blood cultures were repeated and incubated for an extended time period together with samples from the pleural effusions. The antibiotics were changed to meropenem and ciprofloxacin to better cover gram negative and intracellular microbes. Additionally, fluconazole was added to provide coverage for the possibility of infection with Candida species. However, his condition did not improve and he developed progressive multi-organ failure and increasing encephalopathy. Due to poor compliance and ongoing alcohol abuse, he was not considered to be a candidate for liver transplantation. Death occurred 12 days after admittance.
On the following day, there was growth of gram-negative coccobacilli on the chocolate agar from the pleural fluid specimen and later also in the blood cultures, after incubation for 5 and 8 days, respectively. The isolates were identified as F. tularensis by matrix-assisted laser desorption ionization-time of flight mass spectrometry (Maldi-Tof MS, Bruker Daltonics, Bremen). Species identity was later confirmed by real-time PCR at The Norwegian National Reference Laboratory as F. tularensis non-subspecies tularensis, although exact subspecies was not identified.


A 60-year-old male patient presented to the retina clinic with sudden painless diminution of vision in his left eye (LE) for 2 days. There was no associated redness or photophobia. He was a known diabetic for 20 years and had undergone allogenic-related renal transplant for chronic renal failure 2 years back. Currently, he was on oral azathioprine 150 mg and oral tacrolimus 6 mg for the same. Both eyes had received panretinal photocoagulation for proliferative diabetic retinopathy 6 months ago []. Right eye (RE) had undergone uneventful phacoemulsification with posterior chamber intraocular lens (PCIOL) implantation 4 months ago. His LE was subjected to phacoemulsification with PCIOL implantation along with IO injection for DME and Grade 3 posterior capsular cataract, 2 months ago. His preoperative best-corrected visual acuity (BCVA) was 6/18 in the RE and counting fingers at 3 m in the LE. Optical coherence tomography revealed epiretinal membrane with lamellar macular hole in his RE and cystoid macular edema with subfoveal serous fluid in the LE.
On ocular examination, he had a BCVA of 6/18 and counting fingers close to face in his RE and LE, respectively. The intraocular pressure was 20 mmHg in his RE and 22 mmHg in his LE. Anterior segment examination of the LE revealed conjunctival congestion, clear cornea, 1+ cells and flare, well-centered PCIOL, and no evidence of neovascularization of the iris. Fundus examination of the LE revealed 1+ vitreous cells with media clarity of Grade 1, laser photocoagulation scars in the retinal periphery, and yellowish white area of necrotizing retinitis with retinal hemorrhage involving the fovea []. Anterior segment examination of the RE was unremarkable and fundus examination showed media clarity of Grade 1 with laser photocoagulation scars in the retinal periphery with no evidence of DME.
A clinical diagnosis of CMVR was made, and a vitreous tap was taken from the LE to test for CMV, herpes simplex virus, and VZV. Qualitative polymerase chain reaction (PCR) confirmed the presence of CMV and the patient received intravitreal ganciclovir (2 mg/0.1 ml) along with topical steroids. Blood sample for quantitative CMV DNA-PCR revealed 1.6 × 106 copies of CMV DNA. The patient was started on 175 mg intravenous infusion of ganciclovir by the renal transplant department. The patient showed healing of CMVR lesion and received three additional intravitreal ganciclovir injections over the following 2 weeks. The macular retinitis lesion healed over the ensuing 2 weeks with BCVA improving to 6/60 []. Intravenous ganciclovir was continued for 14 days, following which a repeat blood sample for CMV DNA revealed undetectable copies. Oral valganciclovir (450 mg once a day) was started. Over the course of 3 months, CMVR lesion healed completely and the BCVA improved to 6/24 []. Oral valganciclovir was stopped after 3 months. Over a follow-up of 9 months, the patient maintained BCVA of 6/24 in his LE and had no recurrence of the infection [].


A 25-year-old male with no previous comorbidities was admitted in a hospital with a history of high-grade fever for 6 days, diffuse abdominal pain for 2 days, and one episode of hematemesis. Laboratory workup revealed anemia, thrombocytopenia, hyperbilirubinemia with raised transaminases, normal coagulation profile and renal function (hemoglobin 10 g/dl, total leukocyte count 5300/cumm, platelet count 15,000/cumm, serum creatinine 1.0 mg/dl, bilirubin (total/direct) 1.9/1.0 mg/dl, SGOT/SGPT/ALP3390/1150/93 IU/L, INR 1.0) dengue NS1 antigen positive, malaria antigen and smear negative, chest radiograph, and computed tomography (CT) scan [] showed dense bilateral lower lobe consolidation. In the next 2 days, he developed acute respiratory distress syndrome (ARDS) requiring intubation and mechanical ventilation and septic shock, for which broad-spectrum antimicrobials (meropenem and doxycycline) were started, and he was referred to Intensive Care Unit (ICU) of our institute for further management. At the time of admission in ICU, he had fever (core temp 40°C), heart rate of 130/min, blood pressure (invasive) 130/70 mmHg on norepinephrine infusion (0.1 μg/kg/min), sedated with midazolam (3 mg/h) and fentanyl (100 μg/h), and on mechanical ventilation (PC/PEEP: 20/10 cm H2O, respiratory rate 30/min, FiO2:0.8). ICU severity scores were as follows: APACHE II 20 and SOFA 12. Initial laboratory workup at admission showed improving thrombocytopenia and liver function tests. Workup for tropical infections revealed positive dengue IgM and positive scrub typhus serology. Procalcitonin was elevated at 6.5 ng/ml. Due to severe ARDS (PaO2/FiO2 ratio 100), he was proned for 12 h, after which his oxygenation status improved (PaO2/FiO2 ratio 220). Broad-spectrum antimicrobials were continued. However, he had persistent high-grade fever (core temperature 40°C) for the next 3 days despite recovering from septic shock. Three blood cultures drawn at admission grew B. cepacia which was resistant to amikacin (MIC >32), aztreonam (MIC >16), ceftazidime (MIC >16), and cefoperazone–sulbactam (MIC 16:64) and sensitive to levofloxacin (MIC: 2), meropenem (MIC: 2), and trimethoprim–sulfamethoxazole (MIC <0.5/9). Furthermore, endotracheal aspirates at ICU admission revealed the growth of methicillin-resistant Staphylococcus aureus (sensitive to linezolid and vancomycin). Antimicrobials were changed to doripenem, linezolid, and cotrimoxazole. Over 24–36 h following change of antimicrobials, fever reduced in intensity, which was followed by successful extubation 48 h later. Repeat blood cultures after 1 week of therapy with cotrimoxazole were sterile. Procalcitonin decreased to 0.5 ng/ml. CT imaging at discharge showed cavitatory lesions with bronchiectatic changes in the lungs [] which resolved after few days.


A 26-year-old white British woman travelled to Mexico in August 2015 and developed chikungunya infection while touring a jungle area. The infection was confirmed by a local doctor, as the patient had the classic chikungunya symptoms of fever, joint pain and skin eruption. On returning to the UK, the patient consulted a rheumatologist for having persistent joint pains. Chikungunya infection was confirmed by RT-PCR in November 2015. All other possible rheumatological diseases were excluded by further tests. Chest X-ray, Anticnuclear antibody (ANA), anti nuclear cytoplasmic antibody (ANCA) rheumatoid factor, full blood count and C reactive protein were normal.
In July 2016, the patient presented to the urgent care department in ophthalmology with gradual loss of vision in her left eye for the past 1 week. Her Snellen visual acuity was 6/6, right eye, and 6/12, left eye. Ocular examination of the right eye was normal. In the left eye, anterior segment examination and intraocular pressure by Goldmann applanation tonometry were normal. There was no afferent pupillary defect. Posterior segment examination revealed an inflammatory foveal lesion (). High-definition Optical coherence tomography (OCT) scan of the left eye showed increased reflectivity of the neurosensory retina with a cleft of underlying subretinal fluid (). Angio-OCT scan showed areas of capillary dropout on the nasal aspect of the foveal avascular zone (). Fluorescein angiography () showed an area of leakage in the fovea suggesting focal foveal capillary vasculitis, whereas indocyanine green (ICG) angiography () failed to show active choroidal inflammation.
The patient was not on any medication, and there was no ocular history of relevance.


In February 2018, a 60-year-old woman with fistulizing CD for about 40 years came to our attention because of the appearance of worsening asthenia and abdominal pain. Her past medical history was positive for left colon resection with colostomy packaged about 20 years before, for ileocecectomy carried out in 2005 due to adhesion syndrome, and for fistulotomy in 2015 because of multiple rectovaginal and rectocutaneous fistula. The patient was considerably underweight (45 kg). Objective examination of heart, chest, and hypochondriac organs was negative. The abdomen was flat and manageable, painless with torpid peristalsis. The ostomy appeared retracted and stenotic into the left site were not evaluable. At the perianal level, it was possible to appreciate multiple fistulous ostia with presence of minimal granulation tissue and abundant mucous-purulent secretions that also involved the labia majora. The perineum appeared completely destructured. The patient regularly took prednisone (5 mg), lansoprazole (30 mg), and ciprofloxacin (500 mg). She had suspended mesalazine (800 mg) because of the appearance of a probably drug-related skin rash. Abdominal CT, carried out in order to restage the disease and evaluate perianal involvement, showed, on the lateral and posterolateral right side of the anal canal, a 21 × 21 mm hyperdense thickening also extending to the levator ani muscle of the omolateral side, probably representing an abscess. In addition, at the anal level there was a fluid and gaseous collection of 27 × 81 mm, extending also to the vagina and major labia. MRI of the lower abdomen showed two subtle striae hyperintense on T2-weighted sequences fat-sat, localized posteriorly and in the left subsphincter region; these findings were evaluated as fistulas. After evaluating the objective exam as well as hematochemical and instrumental examinations, we proposed to the patient, in agreement with our gastroenterologists, a multidisciplinary therapeutic program that included initial surgical drainage of the perianal fistulas with the Miles technique and repackaging of the retracted ostomy, followed by medical therapy. The surgery involved two stages, one abdominal and the other perineal. The surgeon accessed the abdominal cavity through a xipho-pubic incision. After viscerolysis the sigmoid ostomy was isolated and dissected. We identified the rectosigmoid stump that was isolated up to the rectum wings. A left ovarosalpingectomy was necessary due to adherences which evolved in an abscess probably coming from the rectal stump. Then 10 cm of sigma were resected after mobilization of the splenic flexure which involved a necessary splenectomy. The remaining colon was mobilized up to half of the transverse colon. The ostomy was packaged on the distal transverse side at the level of the right iliac fossa because of the presence of multiple abscesses and cutaneous ulcers at the site of the previous ostomy. The reconstruction of the pelvic floor and the excision of the preexisting ostomy were carried out with biopsy of the peristomal cutaneous lesion. Two drainages were applied: an upper one in the splenic loggia and a lower one in the neo-Douglas. After repeated washings and hemostasis control, the surgical breaches were synthesized and the ostomy was opened. Later we performed an excision of the anorectal canal as the perineum was deconstructed. We proceeded to the partial closure of the ischiorectal excavation with placement of a perineal drainage. The removed material was sent to the anatomopathologists (Fig. ). The postoperative course was regular. The final histological examination showed normal findings as concerned the ovary, the spleen, the ostomy, the colon, and the parastomal skin, while it showed adenocarcinoma of the large intestine as regards the sigmarectoanal canal specimen. This was an adenocarcinoma arising from flat low-grade and high-grade dysplasia, infiltrating the wall up to the subserous adipose tissue (pT3) with a maximum extension of 3 mm from the outer limit of the muscle membrane in the perivisceral adipose tissue. The neoplasia showed infiltrative growth with abundant single cells and poor peri- and intratumoral lymphocytic infiltration. In the wall and in the adipose tissue underlying the neoplasia, numerous lymphoid aggregates and gigantocellular granulomas (compatible with CD) were observed. On the serosa, bleeding as well as granulocytic and histiocytic inflammatory infiltrates could be observed to configure a picture of localized peritonitis. Neoplastic invasion of the sphincter muscle was not observed. As regards grading, the neoplasia was classified as G3 and according to Dukes' classification as B2. High-grade tumor budding was present and was evaluated with anticytokine antibodies AE1 and AE3. Vascular invasion was not documented while perineural invasion was present and widespread. The expression of the protein product encoded by DNA repair genes was also evaluated: MSH-6 and PMS-2 were conserved, while CDX-2 had a partial loss of nuclear positivity (Fig. ). The eight isolated pericolic lymph nodes were disease-free. The proximal margin and the distal margin sampled on the operating piece were disease-free. The radial margin showed a distance of 1 mm from the neoplasm. After evaluating the new clinical situation, we contacted our oncologist for postoperative staging of the neoplasm and evaluation of a possible antitumor therapy. In consideration of stage II of the disease, the most appropriate therapy involved postoperative radiotherapy (45 Gy) associated with capecitabine chemotherapy 1,650 mg/m2/day within 2.5 months after surgery. The patient refused any kind of treatment and only accepted perineum dressings.


A 73-year-old woman with a 5-week history of abdominal swelling was referred to hepatology clinic. She had no history of liver disease. Her medical history included hypothyroidism and a remote total abdominal hysterectomy and bilateral salpingo-oophorectomy for uterine leiomyomata. She had no history of smoking, alcohol abuse, or direct exposure to asbestos. Her most recent mammogram and colonoscopy were normal. Her mother and sister had melanoma, her brother had brain cancer, and her father had colon, prostate, and bladder cancer. Physical examination of the patient revealed a nondistended abdomen and no lower extremity edema. A recent abdominal ultrasound revealed a moderate volume of ascites but no splenomegaly or other signs of portal hypertension. Basic chemistry panel and liver chemistries were completely normal, though a complete blood count was notable for a mild thrombocytosis (platelet count of 432,000/mm3). C-reactive protein was elevated to 3.5 mg/dL, suggesting systemic inflammation. Serum Quantiferon Gold was negative.
A diagnostic paracentesis led to removal of 2 L of serous fluid characterized by serum-ascites albumin gradient (SAAG) of 0.5 g/dL, total protein of 6.4 g/dL, normal lactate dehydrogenase (103 U/L), and normal glucose of 99 mg/dL. Ascitic fluid cell count revealed 695 WBC/mm3 with 4$ neutrophils and 73$ lymphocytes. Gram stain of the fluid did not reveal any pathogenic bacteria, and cytology was negative for malignancy. Ascitic fluid adenosine deaminase, bilirubin, and amylase were also normal.
Imaging tests were unrevealing as to the cause of her ascites. Transthoracic echocardiogram showed a normal left ventricular ejection fraction and no evidence of right heart failure. Magnetic resonance imaging (MRI) of the abdomen with and without contrast did not reveal an intra-abdominal malignancy. A noncontrast CT of the chest (contrast was held due to shellfish allergy) revealed biapical scarring with pleural and subpleural nodularities that were stable compared to prior imaging. Subsequent positron emission tomography scan revealed no fludeoxyglucose uptake of these nodules, suggesting they were benign. A diagnostic upper endoscopy revealed no varices or obvious malignancy, and random duodenal biopsies were normal. A repeat diagnostic colonoscopy was normal. Transjugular liver biopsy revealed normal liver histology without fibrosis. Recurrent paracenteses were required to control her abdominal ascites despite use of oral diuretics.
Finally, a diagnostic laparoscopy was performed and revealed diffuse peritoneal studding with mucinous deposits, more concentrated in the upper abdomen, and a fluid-filled mass associated with the appendix. Peritoneal biopsies revealed low-grade malignancy consistent with mesothelioma. Chemotherapy and possible cytoreductive surgery are planned.


A 53-year-old female presented with abdominal distension and pain localized to the right upper abdominal quadrant radiating to the back. It was partially controlled with oral analgesia and was associated with fever. Due to pain progression, the patient was treated at the emergency department three months later for diaphoresis and dyspnea on exertion associated with right chest wall pain that irradiated to ipsilateral shoulder. On examination, the patient presented with abdominal distension and a painful palpable mass in the right hypochondrium. Laboratory studies indicated leukocytosis and neutrophilia, while the liver tests were normal. Multiphase contrast-enhanced computed tomography scan showed a liver with an occupying hypodense mass in the right lobule and heterogeneous reinforcement in the periphery, a central necrotic component and satellite lesions in the 6th and 8th segments. Importantly, a 39 mm left auricular multi-lobulated mass associated with a lytic lesion was detected in the fourth right costal arch, both of which suggested secondary deposits ().
The patient was hospitalized for extensive work-up and initiated with intravenous antibiotics. Evaluation of intracardiac lesion included a transthoracic echocardiogram, which demonstrated a multinodular image attached to left auricle causing intermittent obstruction with a gradient of 32 mm/Hg. The right ventricle was dilated (43 mm) with tricuspid annular plane systolic excursion (TAPSE) of 18 mm suggestive of right heart failure and pulmonary hypertension confirmed by a pulmonary artery systolic pressure (PASP) of 87 mmHg. Biopsy of the hepatic lesion revealed no complications and revealed undifferentiated embryonal sarcoma. Throughout the follow-up, the patient manifested inflammatory response syndrome characterized by fever, leukocytosis, and tachycardia. No infectious focus was identified and the patient's urine and blood culture tested negative. Due to the poor prognosis, palliative radiotherapy and escalated intravenous analgesia were initiated without clinical improvement. Finally, the patient presented refractory hypoxemia resulting in cardiac arrest and death.


A 41-year-old woman presented to the emergency room with 1 month-long right upper quadrant abdominal pain associated with chills, nausea and emesis. Physical examination showed tachycardia and hepatomegaly with tenderness on palpation, and the remainder of the examination was normal. Laboratory studies showed normal levels of complete blood count and liver function parameters. Alpha-fetoprotein was within normal levels. An abdominal computed tomography showed a 20×16 cm-sized cystic lesion in the right hepatic lobule, with multiple septae and hypervascularization. Doppler ultrasound confirmed the finding and showed compression of intrahepatic vascular structures including portal vein ().
The patient underwent extended right hepatectomy without complications. Histopathological study reported high-grade undifferentiated embryonal sarcoma with negative margins and without lymphovascular and perineural invasion, or gangliona metastasis. The patient was discharged without pain and remained stable for two years, when a cystic lesion on the surgical site was documented during the follow-up. The lesion was located close to one of the main branches of the portal vein, and contraindicated for surgical resection.
Due to disease recurrence, chemotherapy with doxorubicin and isophosphamide was scheduled; however, only three cycles were administered due to hematological toxicity manifesting as neutropenia and fever. The lesion remained stable until three years when an increase in size and appearance of new lesions suggesting disease progression were observed (). After five years from the initial diagnosis, the patient was scheduled to receive chemotherapy with gemcitabine and docetaxel.


A 39 year old woman was admitted to our department because of intermittent bloody stools without vomiting, abdominal pain, diarrhea, skin flushes, etc. The patient had suffered from a slightly curved spinal column since childhood, with the abnormal curvature becoming noticeable 13 years prior. Systemic skin pimples then occurred gradually without pain or itching. Physical examination showed multiple hemispherical subcutaneous nodules with varying sizes and with soft and clear boundaries on the chest and abdomen. There were coffee pigment spots with varied sizes and colors between these nodules, with a maximal size of 3 cm × 2 cm (Figure ). The patient’s father also had definitive NF-1. Blood examination showed the hemoglobin level of the patient was 101 g/L, with no other abnormalities. Computed tomography and magnetic resonance imaging (MRI) of the chest revealed enlarged mediastinal lymph nodes, dermatologic nodules with long T1 and T2 values, uniform densities, clear boundaries, diameters of < 10 mm (Figure ), thoracolumbar scoliosis and thoracic deformities (Figure -D). A pelvic MRI detected segmental thickening of the right external iliac vein, with a thickness of 27.4 mm and a sausage-like appearance (Figure ). The middle and lower rectal mucosae were irregularly thickened, with 26.5 mm at the widest point and an irregular signal with long T1 and slightly longer T2 values. Obvious uneven enhancement was noted in the post-contrast arterial phase, while separation and necrosis were visible in parts. These presentations suggested a diagnosis of multiple rectal lesions (Figure and B). No obvious abnormalities were noted in the computed tomography of the head. Colonoscopy revealed multiple yellow-white nodular uplifts under the rectal mucosa at approximately 1-10 cm from the anal verge. These uplifts varied between 0.3-2.5 cm in diameter and presented with a patchy distribution. The lesion involved the entire rectal lumen (Figure and D). The uplifted surface was smooth but congestive, and blood vessels were apparent on the surface of the nodules under the narrow-band imaging (Figure ). Endoscopic ultrasound revealed multiple hypoechoic lesions in the mucosa and submucosa, with enlarged lymph nodes in the outer membrane (Figure ). Pathohistological and immunohistochemical examinations (n = 10) at many sites from rectal samples showed that tumor cells were present in the lesions and mutually linked to form cord, nest, or gland-like structures. The tumor cells were round, oval or columnar, of varying sizes, with round nuclei, and without obvious mitosis. Cells were CD117 (-), CD56 (+), CK (+), CgA (+), Syn (+), and TTF-1 (-), with a Ki-67 index of < 2%, thus supporting the diagnosis of a grade 1 rectal neuroendocrine tumor (Figure -B). Specimens from many nodules were taken throughout the body and were examined by pathohistology and immunohistochemistry. The subdermal nerve fibers were in a disordered arrangement, and the cells were elongated, spindle-shaped and oddly distributed in the light-stained collagen matrix. Immune staining revealed CD34 (+) and S-100 (+) expression, deep and S-shaped nuclei, and scattered mast cells. These pathological features corresponded to a diagnosis of type I neurofibromatosis (Figure -E). This patient had multiple rectal neuroendocrine tumors with a diameter > 20 mm. The probability of lymph node metastasis and distant metastasis was considered to be very high. Surgical intervention was advised, however the patient rejected surgery and favored surveillance by regular follow-ups every 3-6 mo.


In October 2009, inflammatory breast cancer with small cutaneous nodules and lymphadenopathy in the axillary and supraclavicular regions was diagnosed in a 63-year-old woman. Histopathological examination proved ER/PgR (Allred scores 7/7) and HER2 (IHC 3+, FISH+) positive adenocarcinoma. Thoracic CT revealed further pathologically enlarged lymph nodes around the brachiocephalic artery and aortic arch. No other metastatic manifestations were detected.
The patient received three lines of trastuzumab-based chemotherapy, which controlled the disease for 15 months (). The first-line therapy with paclitaxel-trastuzumab resulted in partial response, but had to be stopped due to grade 3 sensory neuropathy. After two cycles of maintenance trastuzumab monotherapy, the skin metastases rapidly progressed, thus the treatment was supplemented with docetaxel. After sensory neuropathy deteriorated, docetaxel was immediately switched to letrozole. Then, a further three lines of chemotherapy, palliative irradiation to the right breast, chest wall and lymphatic regions, and an additional two lines of HER2-targeted therapy (lapatinib and trastuzumab-emtansine [T-DM1]) were given. These all produced limited or no effect, the disease progressed and involved >50% of the chest wall resulting in the typical appearance of “carcinoma en cuirasse”. Having no additional anti-HER2 therapy options, fulvestrant (loading and then high-dose schedule with 500 mg every 14/28 days) was started. All cancer manifestations improved, and an almost complete regression was maintained for 2 years. After that, the introduction of letrozole had no effect, while consequently cyclophosphamide/methotrexate/5-fluorouracil chemotherapy resulted in partial regression which lasted for 8 months. Finally, she died in December 2016 ().


In July 2010, a 41-year-old premenopausal woman with shortness of breath and low back pain was diagnosed with metastatic breast cancer. For the past year she had noticed deformation of the right breast. Imaging studies indicated locoregionally advanced disease with distant metastases in the lymph nodes, liver, and bones. Core biopsy from the breast tumor indicated grade 2 invasive ductal carcinoma, ER/PgR (Allred scores 8/4) and HER2 (IHC 3+, FISH+) positive.
As first-line therapy, paclitaxel-trastuzumab followed by trastuzumab maintenance therapy was given, which resulted in major partial regression at all metastatic sites, lasting 19 months. Then the patient developed dizziness and vomiting, and in addition to skeletal progression, multiple brain and leptomeningeal metastases were revealed. After whole-brain irradiation with simultaneous integrated boost (15× 2.2+0.7 Gy) the central nervous system (CNS) metastases completely disappeared.
Following radiotherapy, the patient received eight cycles of cyclophosphamide-epirubicine-5-fluorouracil (CEF), followed by tamoxifen as maintenance therapy. This resulted in stable disease for 16 months altogether. Then, CNS progression was detected, and the patient (with postmenopausal hormone levels) was given fulvestrant (loading and then high-dose schedule, 500 mg every 14/28 days). During the treatment, the lesions in the brain decreased significantly (), and all the other manifestations were stable. After 1 year of fulvestrant treatment, the disease progressed in the brain, which was re-irradiated after which T-DM1 treatment was started. As a result, complete regression of the brain metastases associated with partial regression of the other manifestations was observed. The administration of T-DM1 for almost 1 year was subsequently followed by letrozole, which showed no activity. Therapy was continued with capecitabine plus lapatinib, but brain metastases still progressed. Almost 7 years after the first diagnosis, the patient died in January 2017 ().


In January 2012, a 50-year-old postmenopausal woman was referred to our department, for the past 2 years she had noticed a palpable node in her right breast. Extensive stage T3N3 breast cancer with multiple bone metastases was diagnosed. Histopathological examination revealed grade 3 invasive ductal cancer, ER/PgR (Allred scores 8/8) and HER2 IHC 3+ positive. As first-line treatment, letrozole plus trastuzumab was initiated. During treatment, the tumor markers (CA15-3 and CEA) increased and 5 months later skeletal progression was detected. Then, following four courses of doxorubicin plus cyclophosphamide, paclitaxel (once every 3 weeks) was initiated. Tumor markers decreased to normal levels and partial response was detected at all metastatic sites, however, due to grade 3 peripheral neuropathy, paclitaxel treatment was terminated after 18 cycles. Then, fulvestrant (loading and then high-dose schedule, 500 mg every 14/28 days) was introduced as maintenance therapy. After 19 months of fulvestrant therapy, the patient presented with CNS symptoms; brain CT verified multiple metastatic lesions in the cerebellum and parieto-occipital region. Whole brain irradiation with simultaneous integrated boost (14× 2.2 Gy+14× 0.8 Gy) was applied. Since all the extracranial tumor manifestations were controlled with fulvestrant, the patient remained on the systemic therapy; following another 14 months of fulvestrant therapy, no progression was detected until June 2017. At that time, although the brain metastases remained stable, new metastases in the liver and spleen, as well as progression of the bone metastases were diagnosed. The patient’s general condition deteriorated continuously and she died in September 2017 ().


A 53-year-old morbidly obese female (body mass index [BMI] – 45.2) with co-morbidities of hypertension, diabetes and obstructive sleep apnoea with failed attempts to lose weight came to our private hospital for BS. There was a past surgical history of open mesh hernioplasty performed 2 years back for VH. On clinical examination, recurrence of VH was noticeable. The patient was explained and counselled about delayed repair of VH after LRYGB. Intraoperatively, VH was seen with 2.5 cm defect [], having omentum as content along with bowel adhesion to abdominal wall. To proceed for LRYGB, intraoperatively, omentum was reduced and bowel adhesions were removed. The patient underwent LRYGB for her morbid obesity in which side-to-side GJ and JJ anastomosis were done using single linear stapler and hand-sewn closure of enterotomy. Intraoperative methylene blue leak test was negative; VH defect was closed using 1–0 prolene as figure of eight suture. The patient was stable and was ambulatory on the same day.
Oral methylene blue test was negative on 1st post-operative day. The patient was started on clear liquid diet and recovery was uneventful. On 3rd post-operative day, the patient developed tachycardia, abdominal pain with fever and episodes of vomiting subsequently. Her blood parameters showed WBC count of 19,000 with 91% polymorphs. The drain colour suggested enteric content. Immediately, re-laparoscopy was planned. On re-laparoscopy, it was found that sutures used for closing hernial defect had given away. Small bowel was seen herniating through umbilical defect causing intestinal obstruction []. There was an evidence of peritonitis due to disruption of GJ anastomosis with major collection in peri-gastric region []. The obstructed small bowel was reduced and found to be gangrenous []. Hence, resection anastomosis of the bowel was done laparoscopically.
Thorough peritoneal lavage was done, and drains were placed in peri-gastric and pelvic region. In view of friable gastric and jejunal tissue, no sutures were taken on GJ leak. A feeding gastrostomy was done in the remnant stomach for decompression of remnant gastric pouch and enteral feeding. The VH defect was closed by multiple interrupted non-absorbable sutures.
Postoperatively, the patient was shifted to intensive care unit and was kept on intravenous antibiotics and parenteral nutrition. Gastrostomy feeding was started on 4th post-operative day and the patient was shifted to ward. On 12th post-operative day, pelvic drain was removed, and the patient was discharged with peri-gastric drain and gastrostomy for enteral feeds. There was a gradual decline in drain output to around 20–30 ml at 6 weeks. Gastrografin study was done at 6 weeks which showed no evidence of leak. The ultrasonography of the abdomen showed no residual collection. The patient was started on oral liquids after 7 week along with gastrostomy feeds. Drain output remained low for the next 2 weeks. At 9 weeks post-surgery, peri-gastric drain and gastrostomy were removed. The patient was gradually shifted to soft diet and then to normal diet in the next 3 weeks. Further follow-ups and tests on the patient in the next visits were normal. Patient blood sugar levels were under control with oral hypoglycaemic agents. BS resulted in good weight reduction of the patient and BMI of 30.8 was achieved. The patient has also been advised to undergo proper mesh repair for VH at a later date.