A 64-year-old female presented with an incidentally-discovered right posterior inferior cerebellar artery (PICA) aneurysm initially treated by coiling. Follow-up demonstrated recanalization with significant coil compaction that required retreatment. We thought that further coiling might compromise the flow through the PICA and that it would be very probable in the future to have a similar recanalization with the same treatment strategy with coils. The PED Shield was uneventfully used from the basilar to vertebral artery across the origin of PICA and the coiled aneurysm. The patient received dual antiplatelet therapy one week before the procedure. At 8-weeks, clinical and imaging follow-ups were obtained. There were no thromboembolic complications; on digital subtraction imaging (DSA), the aneurysm was stable with preserved blood flow through the PICA (). OCT imaging was planned to evaluate the degree of neointimal growth over the stent to assess the possibility of removal of one of the antiplatelet agents in favor of PICA aneurysm occlusion.
After obtaining the patient’s consent, our endovascular access used a radial artery approach. A 6 F Sofia distal access catheter (Microvention/Terumo, Tustin, CA, USA) over a 0.014” Traxcess microguidewire (Microvention/Terumo) was advanced through the right vertebral artery into the basilar artery using the standard technique. During the procedure, we used a continuous saline flush in our catheters. The patient was heparinized (5,000 IU) and under general anesthesia.
The C7 Dragonfly, a monorail 2.7 F OCT catheter (St. Jude Medical Inc., St. Paul, MN, USA), was advanced inside the distal access catheter (). The OCT image was generated as the optical fiber in the catheter moved using an automated pullback system during simultaneous contrast infusion at a rate of approximately 3 mL/sec. Imaging of about a 5 cm segment of the vessel was achieved with a 15 mL contrast injection necessary to obtain clear images. The OCT system C7 Dragonfly Intravascular Imaging Catheter is more rigid compared to conventional neurointervention systems and potentially traumatic if not carefully navigated. The fiber optic inside the catheter is sensitive to manipulation and presents a risk of fracture during navigation in tortuous vessels, but does not present a risk of catheter fracture or intravascular migration. There were no complications during the procedure.
OCT images demonstrated excellent visualization of PED Shield struts, device wall apposition, and neointima development. Concentric neointimal growth over the PED Shield braid was evident in all areas that the device was well apposed against the vessel wall. No neointima was seen at this 8-week follow-up in areas of device malapposition as well as areas with no contact with the endothelial wall such as the vertebra-basilar junction, perforator, and PICA origin. No thrombi were found on the exposed part of the PED Shield. Normal arterial wall imaging was evident both proximal and distal to the flow diverter. No dissection, intimal tear, or stent stenosis were found ().


The patient is a 50-year-old Caucasian female with a history of Ph+ CML. She was initially treated with hydroxyurea and was switched a week later to dasatinib (a BCR-ABL tyrosine kinase inhibitor (TKI)). She remained on dasatinib therapy on and off for 4 years. Breaks in treatment were required due to thrombocytopenia and pleural effusions. Due to a persistent pleural effusion, she was eventually switched to bosutinib by mouth. Early side effects from bosutinib treatment included diarrhea and “a pruritic”, diffuse rash on the trunk and extremities that appeared a week after beginning treatment. The rash resolved within a week after a methylprednisolone taper and oral diphenhydramine.
After 2 months of starting bosutinib treatment, the patient developed tender papules on her forearms and the medial aspect of her feet. Her oncologist referred her to the dermatology clinic. On physical exam, the patient had erythematous, infiltrated papules and plaques on the forearms and tender, erythematous dusky papules and plaques along the medial aspect of the feet (). The lesions were not pruritic. A punch biopsy of the right forearm was performed. Diffuse interstitial granulomatous infiltrates consisting of histiocytes amid thickened collagen accompanied by eosinophils were present on histopathology. Interface dermatitis was present focally. Special stains (Fite acid fast, gram, and Grocott’s methenamine silver stain) were negative for fungi and bacteria, including mycobacteria. Features were consistent with IGDR (). She was given clobetasol 0.05% cream to be applied to her lesions twice daily and reported mild improvement on follow-up. She subsequently developed new lesions on her thighs (), but denied any joint pain.


A 62-year-old male patient reported with a non-healing ulcer on the roof of mouth on left side. The ulcer appeared 2 months back and it was sudden in onset, associated with occasional bleeding.
Intra-oral examination revealed 1.5 cm x 1.5 cm superficial ulcer on left side of hard palate extending till the junction of hard and soft palate posteriorly, and edentulous alveolar ridge laterally (). Patient was completely edentulous in upper and lower arches, with severe trismus and maximal mouth opening of 1 cm. Right hemi-mandible was absent and reconstructed with pectoralis major myocutaneous (PMMC) flap. There was a mandibular deviation with PMMC flap obscuring palate on right side. Antero-inferiorly, the flap was extending till right oral commissure leading to microstomia. It showed signs of sub-cutaneous stricture formation and fibrosis, thus compromising orofacial musculature (). Neck examination did not reveal evidence of cervical adenopathy.
Patient had been diagnosed with moderately differentiated squamous cell carcinoma (MDSCC) of the lip with synchronous well-differentiated squamous cell carcinoma (WDSCC) of the tongue in 2005. He had undergone wide excision of upper lip-buccal mucosa and tongue followed by adjuvant external beam radiotherapy (EBRT) to face and neck to a total dose of 50 Gy/25 fractions till July 2005. He developed recurrence and was diagnosed with WDSCC of the tongue in 2008. He underwent wide local excision and commisuroplasty but did not receive any adjuvant radiotherapy or chemotherapy. In 2012, the patient presented with second primary lesion (MDSCC) involving the right buccal mucosa, extending till the mandible. He underwent extended right hemi-mandibulectomy and modified neck dissection followed by reconstruction with PMMC flap. He received further adjuvant EBRT to face and neck to a total dose of 60 Gy/30 fractions till August 2012. Subsequently, he was lost to follow-up and did not report to the hospital till 2016.
Histopathologic examination of palatal ulcerative lesion revealed fragments of hyperplastic squamous epithelium with focal areas of dysplasia compatible with squamous carcinoma. Contrast-enhanced computed topographical (CECT) examination did not reveal erosion of underlying left maxilla (). Similarly, 18-fluoro-desoxyglucose positron-emitted topographical scan (FDG PET) did not reveal any abnormal focal increased tracer uptake in head and neck region or elsewhere in the body.
In view of morbidity of previous surgeries and radiotherapy, salvage re-surgery and EBRT were not feasible. HDR brachytherapy was prescribed keeping in mind superficial nature of disease and long disease-free interval (DFI). However, fabrication of conventional surface mould was considered improbable due to completely edentulous maxilla coupled with severe trismus and microstomia.
An alternate mode of retention in the form of dental implant was proposed for surface mould. An orthopantograph (OPG) examination showed adequate bone depth in left maxillary premolar-molar region. Primary impression was made with irreversible hydrocolloid material (Zelgan Plus, DentsplyTM). Custom tray was constructed and subsequently, muscle trimming was carried out using low-fusing impression compound (DPITM Pinnacle tracing stick) to accurately register the borders of the surface mould. A corrective wash impression was made using medium body elastomeric impression material (Aquasil impression paste, DentsplyTM) () to obtain master cast, and customized surface mould was fabricated using heat-polymerized acrylic resin.
Subsequently, two Nobel ActiveTM (Nobel BiocareTM) implants each measuring 3.5 mm x 8 mm were placed in left maxillary 1st and 2nd pre-molar region, using flapless technique under local anesthesia and antibiotic cover. LocatorTM abutments (Nobel BiocareTM) were placed (), and implants were immediately loaded with radiation-carrier prosthesis ().
Primary healing was uneventful and CT scan was carried out after 24-48 hours to verify intimate fit of prosthesis. Once satisfactory, surface mould was removed and softened modeling wax was adapted onto the polished surface. Four 6-F nylon catheters were placed 1 cm apart and secured in wax (). Radiation simulation was carried out using Oncentra® Brachyplanning software (Elekta technologies). The reference isodose was chosen based on coverage of a predetermined target volume. Subsequently, remote after-loading 91Ir HDR brachytherapy, 3.5 Gy twice daily fractions minimum 6 hours apart were administered to a total dose of 49 Gy over 7 days (). The patient was put on nasogastric feed to prevent any untoward masticatory force on the immediately loaded implants. On completion of radiotherapy, the prosthesis was removed, and locator abutments were replaced with cover screws and implants were submerged under oral mucosa.
Patient has been on regular follow-up and exhibited good loco-regional control at the end of six months ().


A 15-year-old male patient referred to our outpatient clinic with refractory epilepsy. Medical history revealed complex partial seizures that first began at 1.5 years of age, which was treated with carbamazepine for 2.5 years. As seizures stopped, the drug was ceased gradually, resulting in no more seizures for 3 years. Although carbamazepine was restarted 3 years later because of recurrence of seizures, the patient had seizures 3–4 times in a month and even several times during a day on some days.
The parents reported that he was born in hospital by spontaneous vaginal birth, he had no prenatal and postnatal health problems, he is successful in the school and he also had a healthy brother. And the parents had no consanguineous relationship.
On physical examination, neuromotor development was found to be appropriate for his age. Neurological examination revealed no pathological findings. Complete blood count and biochemical tests revealed normal findings. On the other hand, electroencephalography showed slow spike and wave complexes arising from the posterior regions of the right hemisphere. Cerebral MRI obtained to determine the etiology of seizures revealed left cerebellar dysgenesis and bilateral peritrigonal and temporo-occipital heterotropia (suggestive of posterior PNH) []. Because of ongoing epileptic seizures, levetiracetam (40 mg/kg/day) and zonisamide (5 mg/kg/day) were added to carbamazepine (30 mg/kg/day) treatment, respectively. FLNA gene mutation was found to be negative. The patient was asked to follow- up every 3 months at outpatient clinic, and he reported no more seizures for the last 2 years.


A 10-year-old girl first presented with early onset of low progressive difficulty in walking and slurred speech. She walked at the age of 18 months but was clumsy, fell frequently, had problems with her balance, and had poor fine motor skills. Neurologic examination at 4 years showed an action tremor of her hands, spasticity in the legs, with hyperactive knee reflexes, and ankle clonus. A diagnosis of spastic paraparesis was suggested in another hospital. First magnetic resonance imaging (MRI) scan of the brain and spinal cord was normal at 4 years. Her history revealed a normal pregnancy and delivery in the mother. Her parents were consanguineous. A second MRI scan of the brain at the age of 6 years showed atrophy of the cerebellar vermis.
On neurological examination in our hospital, she had ataxia, dysarthria, nystagmus, spasticity in lower limbs, global hyperreflexia, and pes cavus. Plantar responses were extensor. Her cognitive function and ophthalmologic evaluation were normal. The MRI showed atrophy of the superior cerebellar vermis and linear hypodensity in the pons []. Electroneuromyography showed sensorial and motor axonal peripheral neuropathy. Previous investigations in other clinics and our hospital showed negative genetic test for Friedreich’s ataxia, normal vitamin E status, alpha-fetoprotein, immunoglobulins, urine amino acids, and organic acids and negative molecular tests for spinocerebellar ataxias type 1, 2, 3, 6, 7, and 10. The clinical and radiological findings suggested ARSACS. We detected homozygous mutation (c.2182C>T) (p.Arg728) (p.R728) in the SACS gene. Written informed consent was obtained from the parents of the patient who participated in this case.


A 6-month-old boy was referred to the Pediatric Clinic Policlinico-Vittorio Emanuele hospital, Catania (Italy), for presenting with drug-resistant seizures with the diagnosis of MDS. He is the second born of healthy, unrelated Italian parents. At the time of gestation, the mother was aged 34 and the father 38 years. The sister (aged 3 years) is healthy. The mother felt normal fetal movements during the gestation. A diagnosis of brain malformation was recorded during the 6 months of gestation as the result of intrauterine ultrasound (US), which disclosed ventriculomegaly, absence of corpus callosum, and intrauterine growth restriction. The mother denied having had any infection diseases or having used drugs or alcohol during her pregnancy. The boy was born at 37 weeks of gestation by spontaneous vaginal hasty delivery. The birth weight was 1800 g, length 45 cm, and head circumference 32 cm. The apgar scores were 5 and 8 at 1 and 5 minutes, respectively. Soon after birth, he was admitted to the neonatal intensive care unit in another city hospital for treatment and clinical evaluation related to facial dimorphisms, cerebral malformation, low weight, and episodes of cyanosis and bradycardia. During this hospitalization, treatment for his respiratory distress and poor alimentation was started. During this period, lasted 26 days, he was submitted to several laboratory investigations, genetic analysis, and neurological investigations. Routine laboratory findings disclosed the presence of mild metabolic acidosis, which was immediately corrected. Brain magnetic resonance imaging (MRI) showed severe LIS consisting of wide-spread agyria and restricted areas of pachygyria ]. The EEG showed poorly organized background and the presence of spikes, mainly in the temporo–central–occipital regions []. At US, colpocephaly and absence of normal cortical gyration with simplified sulci and cerebral scissors were noticed. The parents did not refer anomalous movements from birth to the first 4 months of life when some daily episodes of slow tonic movements of the upper limbs were noticed. General hypotonia and others signs of development delay were still present, and difficulty in maintaining the head upright and persisting episodes of slow movements in the upper limbs were reported by the general pediatrician. During this period, the baby showed a good growth improvement.
At the age of 6 months, he was admitted for the first time for our observation, due to slow tonic seizures sometimes associated to short phase of desaturation prevalently during the sleep. His weight was 6 kg (third percentile); length 62 cm (>third percentile), and head circumference 40 cm (<third percentile). Mild facial dysmorphism was noticed and consisted of high forehead, slight bitemporal hollowing, epicanthus, small upturned nose, low-set and abnormally shaped ears, slim lips with edge under-down, and retrognathia. The neurological examination showed severe hypotonia with difficulty in maintaining the sitting position and poor reactivity. The anterior fontanel was open 0.5×0.5. Partial bilateral ptosis was noticed. The seizures were mainly localized at the face with blinking and slow tonic movements of the upper limbs with a slow rotation of the trunk afterward the left side appearing prevalently before sleeping. The intercritical video-EEG showed a pattern of modified hypsarrhythmia (MH) consisting of random high-voltage spikes and slow waves predominant in the occipital areas and a lack of synchronism with a chaotic appearance []. The critical video-EEG showed a typical electrical suppression during an epileptic spasm: the background was variable and mainly consisted of the combination of hypervoltage slow waves with a bout of rapid low amplitude activity or a diffuse attenuation of the trace.
At 4 weeks later, whereas the intercritical EEG remained unchanged [], the types of seizures showed different features, presenting with brief and synchronous movements of the head, trunk, and limbs, typical of ISs []. These anomalous movements usually lasted about a few seconds and frequently occurred in clusters. The critical video-EEG showed a typical electrical suppression during the epileptic spasms. As the seizures persisted with high frequency, treatment with valproic acid (40 mg/kg/day), levetiracetam (50 mg/kg/day), and vitamin B6 (5 mg/kg/day) was started. Also, the administration of a ketogenic diet failed to give good response. With the gradual addition of midazolam to the treatment regimen, a reduction of the frequency of seizures was achieved, but the seizures still persisted. Routine laboratory analyzes, including blood count, coagulation testing, blood lactate, pyruvate, glucose and ketones, creatine kinase, copper, vitamin D, ceruloplasmin, plasma and urine amino acids, organic urinary acids, purine and pyrimidine, 7-dehydrocholesterol, and total cholesterol were normal. Renal and abdominal US investigations were also normal.
The child was discharged from the hospital and followed up in day hospital. At the age of 8 months, the typical IS seizures were still present and correlated with an EEG intercritical pattern of hypsarrhythmia []. From the age of 8 to 12 months, both clinical phenotypes and EEG recording unchanged, aside from the onset of focal seizures (see ).


A female patient, 64 years old, felt pain in the left upper quadrant of abdomen with no obvious cause in December 2015. CA19.9 level increased to 170.0 μ/mL, and CEA, CA125, and CA50 levels were tested normal on December 18, 2015. ECOG (Eastern Cooperative Oncology Group) performance status score =1. 18-fluoride positron emission tomography-computed tomography scan (18F-PET/CT) showed that there was a mass (about 5 cm in diameter) in the left lobe of the liver and multiple retroperitoneal lymph nodes, which were deemed metastatic on December 20, 2015. The patient underwent a liver biopsy on December 28, 2015. The pathology showed moderately differentiated tubular adenocarcinoma. Immunohistochemically, the tumor cells tested positive for cytokeratin 7 (CK7), cytokeratin 19 (CK19), CDX2, Ki-67 (labeling index =15%), MUC1, and MUC5AC and the tumor cells were negative for cytokeratin 20 (CK20), CEA, MUC2, and P53. Based on the pathologic results presented earlier, the patient was diagnosed with ICCs. She underwent treatment of transcatheter arterial chemoembolization (TACE) for the first time with agents of epirubicin (50 mL), fluorouracil (5-FU 1.0 g), and liquid lipiodol (10 mL) on December 30, 2015. She accepted the accurate cyberknife treatment to liver tumor (DT 42.5 GY/4F) from January 15, 2016 to January 19, 2016 and CA19.9 level decreased to 89.3 μ/mL after radiotherapy.
On May 20, 2016, the patient’s CA19.9 level increased to 132.9 μ/mL. Magnetic resonance imaging (MRI) of upper abdomen showed the remained left hepatic lobe lesions, multiple disseminated metastatic lesions in the right hepatic lobe, and multiple abdominal metastatic lymph nodes on May 25, 2016. She accepted the additional treatment of TACE for three times followed with gemcitabine (800 mg), oxaliplatin (100 mg), and liquid lipiodol (10 mL) on June 24, 2016, September 15, 2016, and October 31, 2016, respectively. CA19.9 level decreased to 77.6 μ/mL after treatments of TACE.
The patient experienced persistent pain in the lower back in January 2017. CA19.9 level increased to 466.6 μ/mL, CA125 level increased to 108.0 μ/mL, and CA50 level increased to 91.4 μ/mL on February 22, 2017. 18F-PET/CT showed that left clavicular area, mediastinum, and right cardiophrenic angle lymph nodes (SUVmax =5.1), multiple low-density shadows in the liver (SUVmax =3.8), and multiple retroperitoneal lymph nodes (SUVmax =4.0), whose FDG metabolism all increased on February 24, 2017. Peripheral blood (method: next generation sequencing) has been tested in March 2017. The gene mutation was negative for epithelial growth factor receptor (EGFR) exons 18–21, KRAS codons 12–13, 61, and 146, NRAS codons 12–13 and 61, KIT exons 9, 11, 13, and 17, PDGFRA exons 12 and 18, ALK, ROS1, C-MET, RET, HER-2, fibroblast growth factor receptor 1–4 (FGFR1–4), or isocitrate dehydrogenase 2 (IDH2). The gene mutation was positive for BRCA2 (c.8009C.A, p>S2670*, heterozygous germline mutation). She received helical tomotherapy (DT50GY/25F) from March 23, 2017 to April 26, 2017, aimed at left supraclavicular, mediastinal, retroperitoneal metastatic lymph nodes and liver metastatic lesions. The lower back pain relieved after radiotherapy. CA19.9 level decreased to 79.3 μ/mL, CA125 level decreased to 43.4 μ/mL, and CA50 level decreased to 55.8 μ/mL after radiotherapy on May 10, 2017.
The patient developed upper abdominal pain and lower back pain in June 2017. CT of the chest and abdomen showed multiple nodular annular enhancement lesions in the left and right lobes of the liver, and left supraclavicular swollen lymph nodes, which considered metastasis; bilateral neck, hepatic gastric space, and retroperitoneal multiple lymph nodes were smaller than before (March 15, 2017); uterine rectal fossa nodules were considered enlarged lymph node (1.5×2.3 cm) () on June 2, 2017. MRI of the upper abdomen showed that multiple nodules in the liver may be intrahepatic metastases on June 2, 2017, in which a new lesion (arrowhead in ) in the right lobe of the liver was found (0.8×0.6 cm). Due to the new pelvic swollen lymph node and the new lesion in the liver, the therapeutic evaluation is progressive disease. CA19.9 level increased to 109.0 μ/mL, CA125 level increased to 74.6 μ/mL, and CA50 level increased to 63.9 μ/mL on June 20, 2017. The patient’s liver biopsy specimens tested negative for programmed death-ligand 1 (PD-L1) and pMMR on June 16, 2017.
The patient started to take olaparib (400 mg bid po) from June 26, 2017. There have been grade 2 fatigue, grade 1 nausea and vomit, grade 4 leukopenia, grade 4 neutropenia, grade 3 anemia, and grade 3 thrombocytopenia during the treatment. On January 23, 2018, white blood cell count decreased to 0.9×109/L, neutrophil count decreased to 0.5×109/L, hemoglobin decreased to 71 g/L, and platelet count decreased to 47×109/L. She stopped taking olaparib for 7 days and got relief after taking positive measures, such as granulocyte-colony-stimulating factor, thrombopoietin, and transfusion. She resumed olaparib at a reduced dosage (200 mg bid po). During the treatment, CA19.9 level decreased to 91.1 μ/mL, CA125 level decreased to 60.1 μ/mL, and CA50 level decreased to 40.3 μ/mL on July 10, 2017. CA19.9, CA125, and CA50 all returned to normal level on August 25, 2017. CT of the chest and abdomen showed that multiple low-density lesions in the liver are the same as before (), the left supraclavicular fossa, left hilus pulmonis, hepatic gastric space, and para-aortic lymph nodes are the same as before; the former uterine rectal fossa nodules disappeared () on March 29, 2018. The therapeutic evaluation is partial response (PR). The patient’s upper abdominal pain was relieved during the treatment. The ECOG performance status declined from 2 scores to 1 score. Currently, the patient is still undergoing treatment.


A 60-year-old male presented with a headache for few months. Carotid Doppler revealed high-grade atherosclerotic stenosis of the proximal right ICA with poststenotic parvus–tardus waveforms []. Left carotid vessel showed high-resistance waveform with the absence of normal ICA waveform which suggested agenesis of the left ICA [].
The patient underwent computed tomography (CT) angiography imaging of the carotid vessels and circle of Willis, which showed absent ICA on the left side and collateral flow to the left hemisphere through the circle of Willis [Figures –]. The absence of the left carotid canal was also noted at bone window setting of CT, which confirmed the congenital nature of the nonvisualization of left ICA []. Maximum intensity projection reconstruction revealed that the left middle cerebral artery (MCA) was supplied by the basilar artery through a dilated left posterior communicating artery (PCOM) [Figures and ].
The patient subsequently underwent magnetic resonance (MR) imaging of the circle of Willis, which showed the absence of flow-related signal intensity within the left ICA []. Saccular aneurysmal dilatation of the anterior communicating artery (ACOM) was detected on CT angiography (CTA) and MR angiography [Figures and ]. A1 segment of the left anterior cerebral artery (ACA) and right PCOM were not seen opacified on CTA and showed the absence of flow-related signal intensity suggested aplasia []. The remaining portion of left ACA was supplied by ACOM aneurysm.


A 34-year-old male presented to our hospital with recurrent left upper quadrant pain for 1 month. Findings of physical examination were unremarkable. The hematology laboratory tests were also unremarkable, including the hemoglobin level was 154 g/L, the white blood cell (WBC) count was 7.4 × 109/L, and the platelet count was 219 × 109/L. The liver function and coagulation tests were normal. Abdominal ultrasound showed splenomegaly and multiple hypoechoic masses about 1.0 × 1.2 cm scattering in the spleen. Unenhanced computed tomography (CT) revealed multiple nodular lesions located at the spleen, and these lesions demonstrated marginally marked enhancement on contrast-enhanced CT. On magnetic resonance imaging (MRI), these lesions revealed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, and lymphoma of the spleen was presumed. No malignant infiltration was found in the bone marrow examination.
The patient underwent splenectomy. Multiple nodules measuring 1.0 to 1.5 cm in diameter were presented as the surgical specimen. Histopathologic examination revealed the proliferation of Langerhans cells with ovoid nuclei, which have a longitudinal nuclear groove, imparting a “coffee-bean” appearance and infiltration of eosinophils (Fig. A, B). Immunohistochemical staining revealed that cells of the tumor were positive for S-100, CD1a, CD45RO, and Vimentin (Fig. A∼D) and negative for CD20, CD79a, CD3, CD23, CD35, and EMA. Taking the morphological and immunohistochemical features into account, the diagnosis of LCH was confirmed. In order to determine LCH was isolated or not, the patient completed further examination as follows. Thyroid hormone levels as well as luteinizing hormone (LH), follicle-stimulating hormone (FSH), prolactin, and cortisol were all within normal limits. Urine osmolarity was normal, and chest CT and head MRI scan were unremarkable. Whole-body bone scintigraphy did not show any specific lesion. Being no evidence of diseases beyond the spleen, a definitive diagnosis of isolated LCH of the spleen was made. The patient received no follow-up adjuvant treatment and had remained tumor free for 9 years after surgery.
The Ethics Review Board of the First Affiliated Hospital of Nanchang University approved this study. Written informed consent was obtained from the patient for publication of this case report and any accompanying images.


A 78-year-old man presented with complaints of abdominal pain, nausea and emesis. CT imaging showed findings consistent with GOO. A year prior in July 2016 he had presented to our facility with obstructive jaundice secondary to a pancreatic head mass (3 mm × 2 mm) with sonographic evidence suggesting both superior mesenteric artery and portal vein invasion. He underwent endoscopic retrograde cholangiopancreatography (ERCP) and successful dilatation of the CBD with the placement of a 10 mm × 6 cm BMS approximately 5 cm into the CBD (Figure ). Biopsy of the mass confirmed adenocarcinoma.
During hospitalization in October 2017, esophagogastroduodenoscopy (EGD) showed retained fluid in the gastric body. There was a malignant appearing, intrinsic moderate stenosis in the second part of the duodenum suggesting type II GOO. The biopsy showed active duodenitis with gastric metaplasia and inflammatory exudates consistent with an ulcer. This area was traversed and stented with a 22 mm × 12 cm WallFlex stent using fluoroscopic guidance (Figure ). Three days later the patient underwent repeat EGD for acute, new onset jaundice and failure to respond to medical treatment. Endoscopic evaluation showed a patent WallFlex SEMS without any migration. Endoscopic retrograde cholangiopancreatography (ERCP) with fluoroscopy was simultaneously performed and confirmed the previously placed duodenal and biliary stents. The scope was passed through the duodenal stent with precision fluoroscopic guidance and the bile duct containing the previously placed CBD stent (10 mm × 6 cm BMS) was deeply cannulated with the short-nosed traction auto-tome and guidewire. Contrast was injected and ductal flow of contrast was adequate. Contrast extended to the main bile duct; however, the lower third of the main bile duct, the middle third of the main bile duct and CBD was completely obstructed by what appeared to be a mass with tumor ingrowth (the same mass that had eroded and obstructed the duodenum previously). A 0.035-inch × 260 cm straight guidewire (Hydra Jag wire) was passed into the biliary tree. Dilatation of the duodenal stent side was accomplished with a Hurricane 10 mm × 4 cm balloon dilator and was successful. One 10 mm × 4 cm covered metal stent (CMS) was placed 3 cm into the previous 10 mm × 6 cm BMS within the CBD. Bile and clear fluid flowed through the stent and the stent was in proper position (Figure ). The patient’s total bilirubin dropped from 5.7 to 3.5 the next day. Four days later, his total bilirubin was 1.5, his acute symptoms had resolved and he was discharged from the hospital.
Relevant patient information: BMI 26.6, non-smoker. History: Coronary heart disease and percutaneous coronary intervention, cerebrovascular accident, atrial fibrillation, pulmonary embolism, nonresectable pancreatic adenocarcinoma, hypothyroidism, depression and hypertension.


A 50-year-old Senegalese male presented to the emergency unit, with acute abdominal pain initially localized in the right lower quadrant, then generalized to the whole abdomen, lasting for 5 days. The pain was associated with vomiting related to food intake, and alteration of bowel habits. There was neither fever, nor relieving factors and he had a non-contributory past medical history, apart from a recent minimal weight loss.
Physical examination revealed a minimal dehydration with an abdominal distension. The rectal examination was normal. The vital signs including the temperature, the pulse, the blood pressure and the respiratory frequency, were within normal limits.
A plain abdominal radiography suggested a bowel obstruction, by showing multiple air-fluid levels. Biological results were the following: hemoglobin: 13,5 g dl−1, hematocrit rate: 42,5%, white blood cell count: 8×103 mm−3, creatinine = 18 mg L−1, serum Na: 139 m Eq L−1, serum K: 3.4 m Eq L−1. The contrast-enhanced CT showed a target mass localized in the right iliac fossa, and was suggestive of ileocecal intussusception without specifying the underlying cause (Fig. ). While coronal reconstruction showed a « sausage-shaped mass » (Fig. ). Moreover, there was some lymphadenopathies surrounding the target mass, and a small amount of abdominal effusion.
Upon laparotomy, we found an ileocecal intussusception, with mesenteric lymphadenopathies and a dilation of the whole small bowel (Fig. ). We performed a right hemicolectomy, without attempting to reduce the intussusception, and an end-to-end ileotransverse anastomosis.
The post-operative outcomes were favorable. The pathological examination of the surgical specimen found an adenomatous ileal polyp with a high grade dysplasia on the terminal ileum being the cause of the ileocecal intussusception (Fig. ).


A 61-year-old Japanese male visited our clinic with a pretibial edema which had already persisted for a few days. He did not show any symptoms suggestive of viral infection and did not have any chronic illness and allergic disease. He had a history of nephrotic syndrome that initially developed when he was 5 years old. His nephrotic syndrome was corticosteroid dependent, i.e., his urinary protein level rapidly reduced with corticosteroids, but relapses were common after the reduction or discontinuation of corticosteroids. He had not experienced a relapse since he was 9 years old. He could recall undergoing a renal biopsy but not its outcomes. Although his medical records were not available, we assumed that he had MCNS. He was not treated with immunosuppressive agents at that time because they were not available in Japan.
Physical examination revealed a pitting edema of his lower extremities (Fig. ). His blood pressure was 176/100 mm Hg, his height was 169.8 cm, and his weight was 72.0 kg. The laboratory test results were as follows: hemoglobin, 14.9 g/dL; red blood cell count, 518 × 104/mm3; white blood cell count, 7,990/mm3; platelet count, 28.9 × 104/mm3; total protein, 5.5 g/dL; serum albumin, 2.7 g/dL; Na, 139 mEq/L; K, 4.2 mEq/L; Cl, 104 mEq/L; serum creatinine, 0.92 mg/dL; eGFR, 65.3 mL/min/1.73m2; AST, 23 IU/L; ALT, 21 IU/L; cholesterol, 318 mg/dL; triglyceride, 219 mg/dL; blood glucose, 94 mg/dL; and HbA1c 5.4%. Urinalysis showed urinary protein 3+, negative glucose, and 5–9/HPF red blood cells in the sediment. The amount of urinary protein was 13.3 g/g creatinine.
The patient was diagnosed with relapsed MCNS and was treated with 40 mg of prednisolone, 40 mg of furosemide, 75 mg of spironolactone, and 20 mg of azilsartan daily. In 1 week, his pretibial edema and urinary protein level reduced, and his blood pressure became normal. In 3 weeks, his serum total protein level was 6.1 g/dL and his serum albumin level was 3.6 g/dL. Subsequently, furosemide and spironolactone were discontinued, and the dose of prednisolone was reduced. After 6 months, prednisolone was discontinued; no relapse has been noticed till now.
A renal biopsy was not performed because the proteinuria quickly responded to the corticosteroids, which is a characteristic of MCNS and is unusual in other causes of glomerulonephritis.


A 33-year-old male patient reported to us with the chief complaint of a painless swelling involving right half of the palate for 5 years from the date of reporting. The swelling initially appeared as a small mass in the central palatal region and then grew over time to involve the entire right half of the hard palate []. The patient gave a history of being treated for RS which had manifested as a nasal swelling associated with chronic rhinitis 3 years back and reported a similar lesion at the same site 6 months back which was suggestive of recurrence.
General examination did not reveal any deviation from the routine findings. Upon extraoral examination, slight facial asymmetry was noticed owing to the nasal swelling on the right side which resulted in obliteration of nasolacrimal fold.
Intraorally, the lesion presented as an ovoid swelling involving the right side extending from the palatal area corresponding to right canine up to approximately 1 cm beyond the third molar and transversely from the gingival margins of the teeth till midline of the palate. The swelling was grossly 4 cm × 2 cm in size with regular, well-demarcated margins, and the overlying surface was smooth and of the same color as the adjacent palatal mucosa. On palpation, the swelling was nontender and soft to firm in consistency.
Based on the history and clinical findings, a provisional diagnosis of palatal extension of RS was made.
Minor salivary gland tumor, radicular cyst, and periapical abscess were considered in differential diagnosis. Subsequently, radicular cyst and periapical abscess were ruled out the due absence of any dental involvement.
Pulp vitality test – The teeth were tested vital during the electric pulp testing.
Radiographic investigations – The patient was subjected to radiographic investigations, wherein the panoramic radiograph was deemed insufficient to comment on the lesion and the patient was further advised cone-beam computed tomography (CBCT). CBCT revealed a palatal swelling appeared as an isodense mass on the right side of the palate measuring 37.4 mm × 20.6 mm in size. In the coronal section, radiopaque structures suggestive of the stents placed to correct the nasal obstruction were present bilaterally, around which soft-tissue masses appeared to grow. On sagittal view, areas of bone erosion were apparent in the posterior palate and floor of the nose indicating continuity in nasal and palatal lesion [].
On hematological examination, the patient was found to have hypochromic microcytic anemia. Preanesthetic evaluation was conducted to rule out other systemic malfunctions such as diabetes mellitus and immunosuppressive diseases.
Cytopathology – Cytological evaluation of the aspirate revealed clumps of red blood cells and few inflammatory cells.
Microbial colony culture – No microbial colonies were observed after 48 h of incubation on MacConkey's agar.
Incisional biopsy of the lesion gave the impression of a chronic granulomatous inflammation. Haematoxylin and eosin stained section exhibited fibrocellular connective tissue with severe degree of chronic inflammatory cell infiltrate with lymphoplasmacytic predominance and moderate degree of vascularity. Scattered at places were several large macrophages (foamy histiocytes) having a central nucleus and vacuolated cytoplasm with rod-shaped bacilli at places resembling the Miculicz cells (MCs). Also evident were hyalinized plasma cells which were seen either discretely (Russell bodies) or in clusters (Mott cells) [].
Histopathology was consistent with the clinical diagnosis and further confirmation was done using special staining techniques to demonstrate the microorganisms. Gram staining showed the presence of Gram-negative rod-shaped bacilli. Periodic acid-Schiff (PAS) and Giemsa stain were also employed for demonstration of the Klebsiella spp. The organisms were stained magenta with PAS and light blue with Giemsa stain [].
Based on the histopathological findings, the diagnosis was given in favor of RS.
The patient was referred to the department of Ear–Nose–Throat surgery for further treatment. The lesion was surgically excised and antibiotic therapy with tetracycline one gram twice a day along with Ciprofloxacin 500 mg/day for 1 month was started and continued as deemed fit by the physician. No recurrence has been observed up to the 6-month follow-up. The patient is kept under regular observation to monitor any incidence of recurrence.


A 72-year-old gentleman, receiving treatment with ipilimumab for advanced metastatic melanoma, presented with a one-week history of a widespread blistering pruritic cutaneous eruption. In January 2016, he was diagnosed with a stage IIIB primary malignant melanoma of his upper back which was treated with wide local excision and completion lymph node dissection. A Positron Emission Tomography – Computed Tomography scan, undertaken in June 2016 for surveillance as part of his follow-up, showed multiple areas of uptake in the lung, supraclavicular fossa and right scapular. He was subsequently commenced on pembrolizumab and completed six cycles. Restaging Positron Emission Tomography – Computed Tomography scan showed disease progression and the patient was commenced on ipilimumab, six weeks after the last dose of pembrolizumab.
One week after commencing the ipilimumab, the patient developed grade 4 diarrhoea. He was treated for ipilimumab-associated colitis with prednisolone 60 mg once mane, which was slowly tapered over the following two months once daily. Ipilimumab was therefore withheld and a palliative approach adopted. Within a week of cessation of the prednisolone for the colitis, the patient developed a cutaneous eruption on his limbs and trunk. Clinical examination revealed a widespread erythematous urticated and blistering eruption. No mucosal involvement was demonstrated ( and ()).
A skin biopsy from the left anterior thigh showed a sub-epidermal blister, with the dermis comprising of perivascular lymphocytic infiltrates mixed with multiple eosinophils. Direct immunofluorescence revealed linear deposition of the IgG and C3 at the basement membrane zone. Elisa for BP180 was elevated at 86 U/mL and indirect immunofluorescence demonstrated anti-basement membrane zone antibodies detected at a titre of 1/200, with antibodies localising to the roof on split-skin substrate, all in keeping with a diagnosis of bullous pemphigoid ( and ()).
The patient was commenced on 40 mg prednisolone once daily and topical clobetasol propionate 0.05% ointment. He is currently being considered for a secondary immunosuppressive therapy, following relapse when the prednisolone dosage was tapered.


A 16-year-old virginal female was referred from her primary care provider (PCP) for complaints of copious vaginal discharge. As part of the initial workup, her PCP noted a pelvic mass and ordered a magnetic resonance imaging (MRI) of the pelvis. The MRI revealed an 8-cm pelvic mass in the lower cervix/upper vagina connected to the myometrium of the left uterine body by a vascular stalk, measuring 2.3 cm in diameter []. The patient had a history of normal monthly menses; however, the month before presentation, she experienced an abnormally heavy period soaking 7 pads per day for 6 days, followed by copious amounts of clear vaginal discharge. Laboratory values revealed a hemoglobin of 6.9 g/dL. The patient was virginal and had no significant medical or surgical history.
The patient was counseled and offered a vaginal examination in clinic versus an examination under anesthesia (EUA) in the operating room. The patient consented to a single-digit vaginal examination in the clinic. On examination, a large, smooth, firm mass was palpated in the midvagina. While a malignant process could not be ruled out, the heterogeneous appearance and presence of internal hemorrhage suggested the most likely diagnosis of an aborting, degenerating fibroid.
After the physical examination and review of the images, the recommendation was made for surgical management. Together with her guardian, we recommended an EUA with removal of the aborting mass by operative hysteroscopy versus vaginal myomectomy with possible dilation and curettage. The patient and her guardian were also counseled extensively regarding the risk for life-threatening intraoperative hemorrhage. In addition, the patient was consented for the unlikely possibility of the uterine occlusion balloon, exploratory laparotomy, and possible hysterectomy in the case of life-threatening hemorrhage. Finally, they were counseled on the possibility of additional surgery or treatment in the unlikely event of malignancy. In preparation for the procedure, a blood transfusion was recommended due to her preoperative anemia, symptoms, and risk of bleeding during the surgery. Due to the fact that her cervix was dilated and the perceived risk of ascending infection, the decision was made to initiate twice-daily doxycycline to reduce the risk of infection until surgery could be performed. The patient was consented and booked for an outpatient procedure including an EUA, vaginal myomectomy, uterine curettage, and a bedside abdominal ultrasound, performed under general anesthesia.
Preoperative surgical planning ensured that any necessary medications and equipment were present in the operating room at the time of surgery. Uterotonic agents including methergine and misoprostol tablets were brought to the operating room. A 30-cc Foley balloon, as well as hemostatic agents including oxidized regenerated cellulose (SURGICEL SNoW, Ethicon, Cincinnati, OH, USA) and thrombin in a gelatin matrix (Floseal, Baxter, Healthcare Corp, Deerfield, IL, USA), along with an abdominal hysterectomy tray, was brought into the operating room in the event of hemorrhage. Next, a Pederson speculum was gently placed into the vagina and an 8-cm fibroid was noted to be on a 3-cm stalk. The cervix was noted to be dilated 4 cm. A suture was placed around the base of the stalk in an attempt to prevent bleeding. This can be done with a free tie or with a laparoscopic pretied suture loop. Dilute vasopressin (20 units in 100 mL of normal saline), a neurohypophysial hormone that constricts blood vessels, decreasing intraoperative blood loss, increasing postoperative hemoglobin, and decreasing blood transfusion rate, was injected into the mass in four locations, with special attention to the base of the stalk.[] The mass was grasped with a Lahey clamp and manually debulked with a scalpel from the most distal portion of the mass into retrievable pieces []. The morcellation was continued until the stay suture was reached at the stalk. The stalk was made flush with the lower uterine segment wall using a sharp curettage. Minimal bleeding was encountered throughout the entire procedure. The uterine cavity began to contract at this time without the use of uterotonic agents. A bedside abdominal ultrasound was performed to confirm that the mass was removed in its entirety and a thin endometrial stripe was noted. The estimated blood loss was 25 mL. Pathology revealed tissue consistent with a submucosal uterine leiomyoma with focal hemorrhage.


A 46-year-old female underwent DSEK for pseudophakic corneal edema in her left eye. The treatment was performed under sterile conditions using preoperative topical anesthesia. On postoperative day one, slit lamp examination revealed well attached lenticule. Topical steroids (prednisolone acetate 1%) was prescribed for six times daily for 1 week and then tapered. The patient's immediate postoperative course was uneventful.
Ten weeks after the procedure, the patient presented with complaints of watering, blurred vision, redness, and pain in the treated eye. On examination, the best corrected visual acuity was 20/400. Slit lamp examination revealed white, nonsuppurative, crystalline corneal infiltrates that exhibited branching needle-like opacities. Size of corneal infiltrate was 1.3 × 1 mm (slit lamp scale was used to measure the infiltrate's size) located in anterior corneal stroma []. Fluorescein staining revealed a corneal epithelium defect. Size of corneal epithelial defect was 1.3 × 1 mm.
Corneal scrapings were obtained from the base and edge of the ulcer using a sterile surgical blade (# 15 on a Bard Parker handle) under topical anesthesia (0.5% proparacaine hydrochloride) and slit lamp magnification. Gram stain and 10% potassium hydroxide mount were included as a part of the standard protocol for microscopic evaluation of corneal smears. Gram-stained smears were examined at ×400 and ×1000 magnification and the potassium hydroxide preparations were examined at ×200 and ×400 magnification under light microscope. Scrapings for smears were collected prior to those for culture. Gram-positive cocci was identified on Gram stain. Fortified cefazolin (5%) with ciprofloxacin (0.3%) was started as a standard empirical therapy on half-hourly basis.
The culture was inoculated on sheep blood agar, chocolate agar, brain heart infusion broth, Sabouraud dextrose agar, and thioglycolate media. The samples were inoculated directly onto the solid culture media by making a row of ‘C’ marks. For inoculation into the liquid media, the blades were swirled directly in the culture fluid. Streptococcus viridans was isolated on blood agar, chocolate agar, brain heart infusion, and thioglycolate media.
Isolated S. viridans was sensitive to co-trimoxazole, linezolid, and netillin. Based on antibiotic sensitivity report, the patient was treated with hourly topical linezolid (0.2%) fortified eye drops. The antibiotic was reduced to five times daily 3 days after presentation and slowly tapered after the infiltrates began to resolve and the epithelial defect healed. The patient responded well to the treatment. Ten weeks after presentation, slit lamp examination revealed deep corneal scarring with neovascularization []. Complete resolution of corneal infiltrate was seen at 10 weeks. Best corrected visual acuity at 10 weeks follow up was counting fingers at 1 meter with accurate projection of rays.


The 74-year old woman was referred to hospital due to recurrent symptoms of heart failure which twice caused hospitalization during the previous 12 months. The patient had a history of anterior myocardial infarction that was treated with primary PCI 7 years ago. Other comorbidities included diabetes mellitus, chronic kidney disease grade III and a history of colorectal cancer successfully treated 3 years ago. Recent coronary angiography did not reveal obstructive disease that required revascularization. The transthoracic echocardiography (TTE) showed left ventricular enlargement (left ventricular end diastolic diameter = 61 mm), depressed systolic function (EF = 25%), left atrial enlargement, severe functional mitral regurgitation, moderate tricuspid regurgitation and right ventricular systolic pressure of 70 mm Hg. The therapy for a heart failure in accordance with the guidelines was prescribed to the patient and consisted of full doses of β-blockers, angiotensin-converting enzyme inhibitor (ACE) and eplerenone. A cardioverter-defibrillator was implanted in the patient for the primary prevention of sudden cardiac death. There was no indication for resynchronization therapy. Due to the fact that heart failure symptoms were persistent, the decision was made to treat mitral regurgitation interventionally. Regards the operation risk, the calculated STS score for mortality was 6%. Following the Heart Team discussion and considering the patient’s preferences, MitraClip device implantation was decided. The pre-procedural transesophageal echocardiography (TEE) confirmed significant mitral regurgitation and the mitral valve anatomy was suitable for the MitraClip therapy ().
The MitraClip procedure was performed under general anesthesia. In addition fluoroscopy and TEE were used to guide the procedure. The first MitraClip was implanted into the central aspect of the mitral valve (A2-P2 area). There was substantial residual MR following the implantation of the first clip () and therefore the decision was made to implant the second clip laterally. After positioning to achieve perpendicularity and the proper axial pathway, clip arms were closed and the device was advanced into the left ventricle. Afterwards, the clip was re-opened and slowly upraised towards the leaflets. Nevertheless, unexpected resistance occurred due to the clip’s entanglement within the tendinous chords. The clip was opened to the inverted position in order to remove it safely into the left atrium. However, the subsequent attempts to retract the clip continued to fail despite the various maneuvers. Finally, the clip was retracted into the left atrium, but, unfortunately, the TEE revealed a new large regurgitation jet which originated from the perforation of the anterior leaflet (). To assess and size the perforation, 3D TEE with volume rendering was used. The patient’s hemodynamics deteriorated and i.v. inotropic support with dobutamine infusion was required. Following the discussion with cardiac surgeons, the decision was made to continue with a percutaneous procedure in view of the extremely high risk of emergency surgery. The bail-out plan was implemented and the second clip was placed laterally, next to the first one, in order to stabilize the position of both devices. The implantation of the second clip was successful without additional complications. Therefore, after two-dimensional and three-dimensional echocardiographic measurements the decision was made to use the 11 mm ASO device to cover residual mitral regurgitation caused by the perforation of the anterior leaflet. Using the MitraClip delivery sheath, the catheter and the guidewire were inserted into the left ventricle through the lateral orifice of the mitral valve. Over the guidewire the ASO device was delivered into the left ventricle, positioned between the lateral clip and the lateral commissure, and deployed appropriately. A few attempts to grasp the anterior leaflet with the perforation between the discs of the ASO device were performed; nonetheless, its position was suboptimal. The part of the posterior leaflet was not captured between the retention discs (). A new coaptation line was created between the posterior leaflet and the ASO device. Overall, the grade of mitral regurgitation was substantially reduced without causing a significant transvalvular gradient. The trans-mitral gradient immediately following completion of the procedure was 6.3/3.5 mm Hg in TEE measurement. As the hemodynamic parameters were stabilized, the patient was extubated. However, during the first hours after the procedure severe hemolysis developed and required massive blood transfusions to the patient. The transthoracic echo showed a stable position of both clips and ASO with the coexistence of moderate mitral regurgitation and a trans-mitral gradient of 8.2/2.6 mm Hg. Despite the treatment with intravenous fluids and bicarbonates, on the second day continuous venovenous hemofiltration was necessary as the subject developed acute renal failure with anuria as a complication of severe hemolysis (). Following an additional Heart Team meeting the patient was qualified for urgent high-risk mitral valve replacement. The procedure was performed with implantation of a valve bioprosthesis (Hancock II 29 mm, Medtronic). After the surgery the patient was extubated but required extracorporeal membrane oxygenation support for the hemodynamic instability that was unresponsive to the high doses of ionotropic drugs. The renal replacement therapy was continued. Despite the intensive therapy the signs of multi-organ failure were worsening and on the ninth day after surgery the patient died.


Patient is a 34-year-old female without past medical history. Two years before she sought medical attention for the first time, she noticed a mass in her upper abdomen that has grown to gigantic proportions, however, due to a lack of adequate access to healthcare facilities in her geographic location she did not visit a healthcare professional. In the 4 months previous to seeking medical attention, she noticed pain around the mass, severe weight loss and recurrent episodes of postprandial vomit, which prompted her family to take her to the emergency room. On clinical examination, a malnourished and dehydrated patient with a giant mass in the upper abdominal was discovered. She reported a slight pain on palpation, but no tenderness was encountered. Blood work was normal and due to the size of the mass, an abdominal contrast-enhanced computerized tomography (CT) was requested. It revealed a huge 40 × 28 × 10 cm3 encapsulated retroperitoneal tumor with a soft tissue density (19–30 UH), and an area containing a calcification (Fig. A). The mass extended from the right flank to the pelvis. It displaced the liver, right kidney and most of the bowel (Fig. A). Yet, it did not invade any adjacent structures and no other masses were observed. Due to these findings and after adequate reanimation, surgery was decided. At laparotomy, a 40 × 30 × 10 cm3 reddish soft consistency retroperitoneal mass was encountered (Figs A and B), the mass was firmly attached to the third portion of the duodenum, right kidney and ureter. Nevertheless, it did not invade any of the latter (Fig. B). No other masses or lymph nodes were encountered. From there surgical decision was straightforward, and complete excision of the mass was performed. During the procedure, an adhesion between the mass and the third portion of the duodenum was tractioned, and a small traumatic duodenal injury occurred. However, due to prompt intervention, the lesion was identified and repaired. A drain was left near the duodenal injury and another one in the retroperitoneal space. After this, the remainder of the procedure continued without any complications (Fig. B).
Pathology reported a DDL, the tumor was composed of mature adipocytes with some enlarged atypical nuclei (Fig. C). In some areas, the liposarcoma transitioned towards a fusiform cell sarcoma and in one area it showed a benign ossification (Fig. C). The resection margins were free of tumor tissue.
The postoperative period of the patient was uneventful, 7 days after surgery, the nasoenteric tube was withdrawn and sips of liquids were initiated. Attaining good oral tolerance, antibiotics were withdrawn, abdominal drains were removed and a soft diet was initiated.
The patient was discharged in good conditions. On follow-up controls the patient is doing well and is undergoing periodic controls with an oncology specialist (Fig. C).


A 55-year-old female presented with left flank pain, frequency, dysuria, and intermittent hydatiduria (passage of grape-like vesicles in the urine).
Ultrasonography (USG) revealed Grade IV hydronephrosis of the left kidney with ballooned out renal pelvis. An oval lesion of mixed echogenicity was arising from the renal pelvis and extending into the dilated inferior calyx of the left kidney [].
Contrast-enhanced computed tomography (CT) of the abdomen and pelvis showed left sided Grade IV hydronephrosis with a large complex intra-renal-pelvic cyst extending into the inferior calyx [].
T2 weighted Magnetic resonance (MR) imaging of the abdomen revealed a large cystic mass with a low signal intensity rim, a high-signal-intensity internal matrix which was arising from the left renal pelvis and was extending into the dilated inferior calyx of the kidney [Figure and ]. T1-weighted images showed low signal intensity rim with no enhancement of the cyst on post-contrast study []. On the basis of these findings, the mass was believed to be intra-renal-pelvic hydatid cyst.
The patient underwent open nephrectomy which revealed a grossly hydronephrotic kidney with papery thin cortex with a large hydatid cyst occupying the renal pelvis and the inferior calyx.


A 57-year-old male came with complaints of diffuse abdominal pain for the past 3 months with an increase in severity over the past 10 days. Routine blood investigations (complete hemogram, blood sugar, and serum creatinine) were normal. Liver function tests were normal. Amylase and lipase levels were within normal limits.
Contrast-enhanced computed tomography (CT) [] showed a well-defined cystic lesion arising from the head and neck of the pancreas with enhancing septations within. The lesion was partly exophytic anteriorly and seen causing mass effect on the main pancreatic duct with downstream dilatation of the main pancreatic duct in the body and tail []. The lesion was also seen exerting mass effect on the confluence of splenic and superior mesenteric vein and the adjacent portal vein. Endoscopic ultrasound (EUS) showed a heterogeneous lesion in the head of the pancreas which was predominantly isoechoic with few cysts in some portion of the lesion giving the appearance of “honeycomb” []. EUS-guided fine needle aspiration cytology (FNAC) was reported as well-differentiated adenocarcinoma of the pancreas.
The patient underwent pancreatectomy with cholecystectomy and splenectomy and the specimens were sent for histopathological examination. The patient recovered well after surgery and is under follow-up.
Grossly, the cut section of the pancreas showed a well-circumscribed lesion measuring ~4.0 × 4.0 × 3.5 cm, which appeared spongy because of the presence of multiple, tiny cysts ranging in size from 2 to 10 mm []. Intervening areas were gray-white in color.
On microscopy, the specimen showed an unencapsulated neoplasm in the pancreas composed of cysts of varying sizes lined by single layer of cuboidal to low columnar mucinous epithelium with occasional papillary processes []. Mild nuclear atypia was evident. Stroma showed fibrosis and collagenization. Histopathological diagnosis of mucinous cystadenoma of pancreas was made based on the above findings.


An 85-year-old man with a permanent DDDR pacemaker (implantation 25 years ago due to complete heart block) was admitted for differential diagnosis and treatment of a right atrial mass, revealed in a routine transthoracic echocardiogram (TTE), which was performed on an outpatient basis, for the evaluation of dyspnoea on exertion (New York Heart Association III stage). The patient had a prior medical history of arterial hypertension and three-vessel coronary artery disease under medical treatment. Coronary angiography was performed 2 years ago. Percutaneous coronary artery angioplasty was not feasible due to the complexity of the atheromatic lesions and the patient refused any surgical treatment. Six months prior to this hospitalization, he had undergone uncomplicated generator replacement due to battery depletion. On admission, the patient was afebrile with a heart rate of 60 b.p.m. and blood pressure 120/65 mmHg. The ECG showed pacing rhythm, and he was pacemaker-dependent. Physical examination of the heart, lung, and abdomen revealed no abnormalities and there were no signs of localized infection at the pacemaker pocket site.
Biochemical examinations (red and white blood cells count, C-reactive protein and erythrocyte sedimentation rate, liver function tests, thyroid function tests, urine analysis, and plasma markers of coagulation) were within normal range. The interrogation of the device revealed normal findings.
The TTE showed the left and right ventricle within normal size and contractility range. The apical four chamber view (Figure and B) revealed clearly two mobile masses (32 mm and 19 mm long) with a common stem, of low echogenicity and elliptic shape, attached to the ventricular lead of the pacemaker, prolapsing through the tricuspid valve in systole (ventricular lead type: Medtronic 4043–58, passive fixation, bipolar). Contrast-enhanced chest and computed tomography showed a pulmonary infarct in the right upper lobe. A Doppler study of the upper and lower extremity veins did not show any thrombi.
The patient was held on low molecular heparin (LMWH). Three sets of blood cultures and urine culture were negative. The patient remained in-hospital for a close echocardiographic follow-up and for the final identification of all blood cultures. Ten days after his admission, he presented low grade fever up to 37.5°C, normal white blood cell count and C-reactive protein at 70 mg/L (normal range <3 mg/L). A positive urine culture of Escherichia coli was detected. After 2 weeks of antimicrobial treatment (ciprofloxacin 200 mg bid) and under LMWH the patient was afebrile, with normal inflammatory markers and small decrease of the size of the cardiac masses in the TTE.
The patient was discharged on acenocoumarol. A TTE after 1 month did not visualize the cardiac masses (Figure ). Acenocoumarol with a goal INR of 2.0–2.5 was recommended to be continued for an additional 3 month period. After discontinuation of acenocoumarol, the patient received aspirin 100 mg daily, due to the coexisting coronary artery disease.
The patient did not receive any other anticoagulant or antiplatelet therapy and did not attend any other scheduled follow-up appointments.
Over 2 years after his 1st admission, the patient, without anticoaguland therapy anymore, was readmitted to our clinic due to a 7-day history of fever up to 38.5°C. Once more, physical examination revealed no signs of localized infection.
The TTE revealed two new vegetation-like masses, possibly attached to the same site of the ventricular lead. Transoesophageal echocardiogram (TOE) confirmed clearly the existence of the masses approximately of the same size as in the 1st hospitalization and their attachment to the pacemaker lead (Figure ). Once more, most of the biochemical examinations (red blood cells count, erythrocyte sedimentation rate, liver function tests, thyroid function tests, urine analysis, and plasma markers of coagulation) were within normal range, with only slight elevation of white blood cells count [11 000/μL (normal range 4000–9000/μL), 76% PMN (normal range 50–80%), and C-reactive protein (23 mg/L)]. Two out of five blood cultures yielded Staphylococcus epidermidis. Contrast-enhanced chest computed tomography showed no pulmonary infarcts. The patient was held on vancomycin 2 g daily and meropenem 1 g daily, combined with LMWH (enoxaparin 60 mg bid). Differential diagnosis between contaminated thrombus and vegetation was challenging. However, 18F-FDG PET/CT scan, which was performed 2 days after admission, demonstrated increased uptake of 18F-FDG in the outer part of the middle pulmonary lobe and in the sigmoid colon (Figure ). The diagnosis of vegetation was excluded and lead thrombosis was confirmed once more (relapse). Antibiotics were discontinued. A sigmoidoscopy was performed, which revealed polyposis of the sigmoid colon. Endoscopic polypectomy was performed one month afterwards. At that time, the TOE echocardiogram revealed total resolution of the masses (Figure ).
Since then, the patient remains asymptomatic on acenocoumarol therapy with a goal INR of 2.0 – 2.5, with no detectable intracardiac masses in a follow-up TTE, 6 months after his 2nd hospitalization. Repeated device interrogation did not detect any dysfunction. Of note, no atrial tachyarrhythmia was ever detected.


A 49-year-old woman was diagnosed with VSD at birth and underwent pulmonary artery banding during infancy. She was recently diagnosed with severe PAH and was referred to our hospital for treatment. The patient had dyspnoea. Her heart rate was 111 b.p.m., blood pressure 126/94 mmHg, and SpO2 92% with oxygen administration at 2 L/min. Cardiovascular examination revealed increased intensity of P2 and pansystolic murmur at the third right intercostal space on auscultation and parasternal heave. The lung field were clear. The remainder of the physical examination was unremarkable. Transthoracic echocardiography showed perimembranous-type VSD (34 × 34 mm) with a bidirectional shunt, a dilated right ventricle, and an estimated right ventricular systolic pressure of 108 mmHg (Figure –C; see also , Video S1). Right heart catheterization (RHC) revealed a mean pulmonary artery pressure (mPAP) of 76 mmHg, PVR index (PVRi) of 15.8 Wood units/m2, pulmonary blood flow-to-systemic blood flow ratio (Qp/Qs) of 1.68, and PVRi systemic vascular resistance index ratio (Rp/Rs) of 0.4 (Table ). Vasoreactivity testing with the patient breathing 100% oxygen for 10 min showed the following decreases: mPAP 73 mmHg, PVRi 12.5 Wood units/m2, Rp/Rs 0.34. There was also an increase in Qp/Qs of 2.34.
Because surgical closure of the VSD would be difficult, we started combination therapy with sildenafil 60 mg/day and ambrisentan 5 mg/day. Right heart catheterization performed 3 months after treatment showed mPAP 68 mmHg, PVRi 11.4 Wood units/m2, Qp/Qs 2.13, and Rp/Rs 0.35. We added epoprostenol and gradually increased the dose. Right heart catheterization at 1 year after treatment showed mPAP 58 mmHg, PVRi 8.8 Wood units/m2, Qp/Qs 2.2, and Rp/Rs 0.35. Vasoreactivity testing with 100% oxygen for 10 min showed mPAP 72 mmHg, PVRi 7.2 Wood units/m2, Qp/Qs 3.26, and Rp/Rs 0.31 (with the patient on sildenafil at 60 mg/day, ambrisentan at 5 mg/day, and epoprostenol at 30 ng/kg/min). The patient underwent surgery for the VSD with a patch and a flap valve. Transthoracic echocardiography 10 months post-operatively showed an estimated right ventricular systolic pressure of 50 mmHg (Figure –F; see also , Video S2). Right heart catheterization performed 10 months post-operatively showed mPAP 42 mmHg, PVRi 11.5 Wood units/m2 (with the patient on sildenafil 60 mg/day, ambrisentan 5 mg/day, epoprostenol 24 ng/kg/min).


A 20-year-old man had been diagnosed with VSD and PAH at birth in another hospital. Treatment with sildenafil 60 mg and ambrisentan 10 mg was started at age 5 years. The patient was referred to our hospital at age 20 years. The patient had slight dyspnoea. His heart rate was 58 b.p.m., blood pressure 97/52 mmHg, and SpO2 94% in room air. Cardiovascular examination revealed increased intensity of P2 and pansystolic murmur at the third right intercostal space on auscultation and parasternal heave. The lung field were clear. The remainder of the physical examination was unremarkable. Transthoracic echocardiography showed perimembranous-type VSD (18 × 18 mm) with left-to-right shunt, dilated right ventricle, and estimated right ventricular systolic pressure of 91 mmHg (Figure –C; see also , Video S3). Right heart catheterization showed mPAP 71 mmHg, PVRi 9.0 Wood units/m2, Qp/Qs 1.59, and Rp/Rs 0.41 (Table ). Vasoreactivity testing breathing 100% oxygen for 10 min showed no change in mPAP 73 mmHg but decreases in PVRi of 7.8 Wood units/m2, Qp/Qs 2.37, and Rp/Rs 0.30. We initiated administration of beraprost and changed sildenafil to tadarafil. Right heart catheterization performed 1 year later showed mPAP 76 mmHg, PVRi 11.8 Wood units/m2, Qp/Qs 1.34, and Rp/Rs of 0.61. Vasoreactivity testing breathing 100% oxygen for 10 min showed mPAP 72 mmHg, PVRi 7.7 Wood units/m2, Qp/Qs 2.05, and Rp/Rs 0.36 (with the patient on tadarafil at 20 mg/day, ambrisentan at 10 mg/day, and beraprost at 240 µg/day). The patient underwent surgery for patch closure of the VSD and small atrial septal fenestration. Transthoracic echocardiography 2 years later showed estimated right ventricular systolic pressure of 48 mmHg (Figure ; see also , Video S4). Right heart catheterization performed 2 years post-operatively showed mPAP 48 mmHg and PVRi 9.1 Wood units/m2 (with the patient on riociguat 7.5 mg/day, macitentan 5 mg/day, and treprostinil 15 ng/kg/min).


A 49-year-old woman was diagnosed with VSD and PAH at 2 years of age in another hospital. Left pulmonary artery banding was performed at age 4 years. Treatment with ambrisentan and warfarin was started at age 18 years. At age 49 years, the patient was referred to our hospital for possible VSD closure. The patient had slight dyspnoea. Her heart rate was 76 b.p.m., blood pressure 95/46 mmHg, and SpO2 95% breathing room air. Cardiovascular examination revealed pansystolic murmur at the third right intercostal space on auscultation. The lung field were clear. The remainder of the physical examination was unremarkable. Transthoracic echocardiography showed a perimembranous, muscular-type VSD (18 × 19 mm) with a bidirectional shunt, dilated right ventricle, and estimated right ventricular systolic pressure of 56 mmHg (Figure –C; see also , Video S5). Right heart catheterization showed mPAP 60 mmHg, PVRi 12 Wood units/m2, Qp/Qs 2.54, and Rp/Rs 0.29 (Table ). Vasoreactivity testing with the patient breathing 100% oxygen for 10 min did show decreased mPAP (at 60 mmHg) but was associated with decreased PVRi of 6.0 Wood units/m2 and Rp/Rs of 0.2 and increased Qp/Qs of 4.42. The initial strategy was to band the main pulmonary artery. Right heart catheterization performed 1 year after banding showed decreases in mPAP of 38 mmHg, PVRi of 6.5 Wood units/m2, and Rp/Rs of 0.21. Qp/Qs was increased by 2.87. Vasoreactivity testing breathing 100% oxygen for 10 min showed mPAP 43 mmHg, PVRi 5.8 Wood units/m2, Qp/Qs 3.72, and Rp/Rs 0.14 (with the patient on ambrisentan 10 mg/day). The patient underwent surgery for patch closure of the VSD and debanding of the main and left pulmonary arteries. Transthoracic echocardiography 6 months later showed an estimated right ventricular systolic pressure of 29 mmHg (Figure –F; see also , Video S6). Right heart catheterization performed 6 months after surgery showed mPAP 15 mmHg and PVRi 4 Wood units/m2 (with the patient on ambrisentan at 10 mg/day).


A 33-year-old man came to emergency department with chief complaint of fatigue and cold sweat in the morning while doing routine house chores. He also complained from retrosternal burning sensation 2 days ago. He had upper tract respiratory infection 1 week ago. He was not a professional athlete, however, from 2 days ago he began ‘light body building and aerobic exercise’. When detailed history was taken, retrosternal chest discomfort was associated with holding a 30 pounds weight at the first time. The pain did not exacerbate with walking and did not radiate to arms or jaw. At the first day of presentation, he had no chest pain or shortness of breath. Physical examination was unremarkable and vital sign was stable. He was non-diabetic and normotensive. Past medical history was negative. He did not use any kind of drug substance and cigarette.
After physical examination, electrocardiogram (ECG) and routine lab test were ordered.
ECG revealed normal sinus rhythm, normal axis, and no ST-T change (except for tall R in V1) (no dynamic change in serial ECGs), Figure . Right and posterior ECGs were normal. High sensitivity Troponin T scores (0 and 2 h protocol) were 87 ng/dL and 84 ng/dL, respectively, with upper limit of 24 ng/dL. D-dimer was negative. Erythrocyte sedimentation rate (ESR) and C-reactive peptide (CRP) were also negative. Transthoracic echocardiography (TTE) was performed; normal left ventricle and right ventricle size left ventricular ejection fraction 55%, good right ventricular function, no evident wall motion abnormality, and no significant valvular disease.
In this step, besides acute coronary syndrome (ACS), we thought about myopericarditis and also aortic dissection (AD). Physical examination, TTE, and D-dimer level were not in favour of AD. But we could not rule out myopericarditis or even ACS.
The patient received ASA 650 mg TDS, Pantoprazole 40 mg daily, Atorvastatin 40 daily, Clopidegrol 75 daily, Metoral 25 mg BD, and enoxaparin 80 mg SC BD. We decided to perform coronary multislice computed tomography angiogram (MDCT). It was done at heart rate 80 b.p.m. with prospective triggering method. Calcium score was zero, and coronary vessels were normal except mild stenosis in mid part of left anterior descending artery (LAD). The patient was asymptomatic and transmitted from coronary care unit (CCU) to the ward. He insisted on hospital discharge as soon as possible. At the day 3, we planned to discharge the patient and follow him medically. But, he complained from similar retrosternal pain while holding his 2-year-old child, which was brought to the hospital against hospital regulations to visit her father. The resident immediately visited the patient; fortunately serial ECGs and Troponin level were not remarkable. Hence, we changed the plan and scheduled him for coronary angiography (CAG). The next day, he underwent CAG, Figure . In addition to mild LAD lesion, multiple linear coronary dissections, and contrast dye staining within left circumflex artery (and its obtuse marginal branches) were seen. After consultation with interventionist the patient transferred to CCU again.
Connective tissue disease may cause SCAD. Anti-nuclear antibody, anti double-stranded DNA, and rheumatic factor were ordered and were negative. Colour Doppler sonogram of renal arteries was performed to evaluate probable fibromuscular dysplasia. All para-clinic results were normal.
We observed the patient for the next 2 days and discharged him with ASA 80 daily, Clopidegrol 75 daily, Pentoprazole 40 daily, Atorvastatin 20 qHS, and Metoral 25 mg BD.
Monthly follow-up was done for about 4 months. Restriction of isometric exercises was advised. After 3 months, he was asymptomatic except one similar episode. The night of Tehran earthquake, he held his child to escape from the apartment and experienced the same symptoms. He was revisited in our clinic and myocardial perfusion scan with exercise test was performed, which was negative for ischaemia.


A 37-year-old female with past medical history of morbid obesity (body mass index 56.65 kg/m2), severe obstructive sleep apnea, and tracheal stenosis from prior prolonged intubation due to penetrating neck injury of 3 weeks’ duration was admitted to our Intensive Care Unit for stridor. The patient's postintubation tracheal stenosis was not found to be appropriate for surgical correction and therefore she was initially managed with laser ablation and mechanical dilation; although due to persistent stridor and dyspnea, a straight silicon tracheal stent was placed. Following resolution of symptoms, the stent was removed 6 months later.
Shortly thereafter, she returned with increased dyspnea and nonproductive cough. Bronchoscopy revealed severe tracheal stenosis with malacia extending 42 mm from the cricoid cartilage []. The decision was made initially to place a Dumon® tracheal straight silicon stent (Novatech SA, France) which unfortunately collapsed during postoperative recovery and had to be exchanged 5 days later for a Dumon® Y-shaped silicon stent (dimensions: 16 mm × 13 mm × 13 mm). The procedure was described to be a difficult procedure by the implanting interventionalist, requiring multiple balloon dilations, rigid bronchoscope manipulations, use of forceps, and complicated by postoperative laryngeal edema and spasm, which improved with oral prednisone and the patient was eventually discharged from the hospital.
On postoperative day 7, she developed dyspnea and stridor resulting in re-admission to the Intensive Care Unit for close monitoring while awaiting bronchoscopy evaluation by interventional pulmonology. She had mild respiratory distress with associated mixed stridor, oxygen saturation levels of 96% on room air without pronounced respiratory rate or effort. Overnight, the patient developed increasing dyspnea and chest pain with oxygen saturation of 90%, initially responsive to conservative management. She progressed to worsen within the next few hours despite escalating doses of helium-oxygen therapy. A difficult airway team composed of anesthesiologists, otolaryngologists, and trauma surgeons was promptly called for advanced airway management. Upon evaluation at bedside, the patient was having increasing respiratory effort, with oxygen saturation on 80% through face mask with 100% supplemental oxygen.
Given impending respiratory decline, sedation and muscle relaxation were provided. Two-handed mask ventilation with oral airway was accomplished with difficulty. Following the American Society of Anesthesiologists’ difficult airway algorithm,[] the decision was made to proceed with endotracheal tube placement. Portable videolaryngoscope (size #4 blade) was used, providing a full visualization of the glottis (Cormack–Lehane view Grade 1). Difficulty in advancing a size 7.0 Fr endotracheal tube was encountered due to resistance distal to the vocal folds. A second attempt with a tracheal tube introducer under direct videolaryngoscope guidance also failed to advance due to similar airway resistance. Following this attempt, mask ventilation became more difficult.
A laryngeal mask airway (size #4) was placed; however, this also failed to provide adequate ventilation and oxygenation, necessitating a surgical airway. A cricothyroidotomy was performed with a palpable lumen across the incision through which a tracheal tube introducer was placed and a size 6.0 Fr endotracheal tube was threaded. Despite adequate colorimetric change, high ambu-bag resistance was encountered and the patient continued to have inadequate ventilation. At this point, attempted flexible fiberoptic evaluation of the patient's airway was difficult due to secretions and blood in the supraglottic space. The patient subsequently developed cardiac arrest and expired after multiple rounds of cardiopulmonary resuscitation.


A 64-year-old man presented with mild back pain and weight loss for 3 months. His medical, family, and psychosocial history was unremarkable. Computed tomography (CT) scan at admission revealed a mass (9.3 × 8.9 × 9.7 cm3) above the upper pole of the left kidney, a 7-mm nodule in the right lung, and a 3-mm nodule in the left hepatic lobe. Contrast-enhanced magnetic resonance imaging (MRI) demonstrated a mass, 9.9 × 9.7 × 9.1 cm3 in size, compressing against the left renal artery and vein. A biopsy revealed poorly differentiated cells with abundant eosinophilic cytoplasm and round nuclei, resembling hepatocellular carcinoma cells. The cells were arranged in acinar and cord-like patterns (Fig. ). The tumor was positive for CK8/18, CK19, CK7 and hepatocyte and negative for AFP, suggesting that these cells were hepatoid. No tumor mass was identified in the liver by contrast enhanced MRI. Lung cancer and neuroendocrine tumor were ruled out as the source of the adrenal tumor by immunostaining. Poorly differentiated adenocarcinoma with unknown origin was considered at this point of time.
Laboratory investigations showed elevations in carcinoembryonic antigen (95.75 ng/mL; normal range: 0–5 ng/mL), carbohydrate antigen (CA) 125 (1512 U/mL; normal: 0–35 U/mL), CA 15–3 (438.5 U/mL; normal: 0–25 U/mL), and CA19–9 (36.01 U/mL; normal: 0–27 U/mL). Plasma AFP, adrenocorticotropic hormone, and cortisol were normal. 18F-fluorodeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) showed a moderate increase in 18F-FDG uptake in the left adrenal gland with a maximum standardized uptake value (SUVmax) between 2.4 and 7.8 and a mean between 2.1 and 6.9. The tumor was 9.8 × 9.3 × 10.8 cm3 in size. A soft tissue lesion, 2.3 × 1.7 cm2 in size, with increased 18F-FDG uptake (SUVmax = 6.04) was detected in the retroperitoneal region (Fig. ).
Multiple nodules were noted in bilateral upper lungs as well as the oblique pleura. The largest nodule was 1.3 × 1.4 cm2 in size, with heterogeneous 18F-FDG uptake (SUVmax = 3.7, mean = 3.2). Lung metastasis was considered. Immunohistochemistry revealed that the tumor tissue was negative for vimentin and positive for Hep Par1, thus excluding adrenocortical carcinoma (Fig. ). Endoscopic ultrasound showed no tumor in the stomach. A diagnosis of hepatoid adenocarcinoma of the adrenal gland was established.
NGS of the primary adrenal tumor and blood using Illumina Hiseq (Geneseen, , reference genome: GRCh37/hg19) revealed the following mutations (Table ): ATM (c.A3078-2T), CDKN2A (D84H), epidermal growth factor receptor (EGFR) (P546L), serine/threonine kinase 11 (STK11) (p.331fs insertion frameshift mutation), TP53 (H193L), BIM (loss of heterozygosity), and MLH1 (V384D hybrid germline mutation). The following drug metabolism-related polymorphisms were detected: cytidine deaminase (CDA) (K27Q heterozygous polymorphism), glutathione S-transferase pi 1 (GSTP1) (I105 V homozygous polymorphism), glutathione S-transferase theta 1 (GSTT1) (homozygous gene deletion), and thiopurine S-methyltransferase (TPMT) (Y240C heterozygous polymorphism).
After 4 cycles of mFOLFOX6 regimen (oxaliplatin, leucovorin and fluorouracil), the tumor became larger (10 × 10 × 11 cm3 upon contrast-enhanced CT) and invaded the left kidney as well as the left psoas muscle. The patient then received transcatheter arterial chemoembolization (TACE) as well as apatinib (Hengrui Pharmaceuticals, Lianyungang, China) at an initial dose of 850 mg/day for 1 week, and then 500 mg/day. After 1 month, the patient refused apatinib therapy because of severe fatigue and declined further evaluation. The patient died 9 months after diagnosis.


A 61-year-old male patient with insignificant medical history presented to vascular clinic from orthopedic clinic with complaint of a mass on the volar aspect and radial side of his right wrist (). He started to notice this mass since 6 years. It was small and had gradually increased in size. He denied any history of trauma or previous cannulation at the same site. He is a barber and a nonsmoker. On physical examination, the mass was 3×3 cm, there were no skin changes, and it was pulsating. Allen’s test was positive. There was no evidence of other aneurysms in both upper and lower limbs by physical examination. A trial of needle puncture of the mass was performed at orthopedic clinic before referral and revealed arterial blood flow, and so he was referred to our vascular clinic.
Doppler ultrasound and computed tomography (CT) angiogram (CTA) showed a 3×3 cm isolated distal RAA with normal ulnar artery and intact both deep and superficial palmar arches ().
On patient request and taking into account the potential risks such as thromboembolic complications or even rupture, a surgical repair was decided and performed. Under local anesthesia, a 4 cm longitudinal incision above the aneurysm was done. A saccular aneurysm was identified at the distal part of the radial artery after the takeoff of the superficial palmar arch branch (). Then, the proximal, the distal radial artery, and the origin of the superficial palmar arch were controlled. Excision of the aneurysm was done, and the excised specimen was sent for histopathology. Arterial continuity was restored by primary end-to-end anastomosis between proximal and distal radial artery (). The postoperative course was uneventful, and patient was discharged on the same day on simple analgesia. The histopathology report showed a dilated arterial sac with intraluminal mural thrombus.


A 45-year-old female with no known comorbidities presented with acute onset breathlessness, face and neck swelling for which chest roentgenogram showed a right lung mass. CT scan chest showed a mass lesion right upper and middle lobe (RUL/RML) causing superior vena cava obstruction. Image-guided biopsy from the lung mass revealed adenocarcinoma with immunohistochemistry (IHC) positive for thyroid transcription factor-1 (TTF-1) and CK7 []. She was treated with radiotherapy to mediastinum to a dose of 20 Gy in 5 fractions which resulted in the resolution of symptoms. Magnetic resonance imaging (MRI) brain was normal, while whole-body 18FDG-PET/CT (WB-18FDG-PET/CT) showed FDG avid RUL/RML irregular marginated mass lesion with standard uptake value (SUV) of 7.36 [] and FDG avid soft-tissue lesion left gluteus muscle with a SUV of 9.83 [Figures and ], biopsy from which revealed metastatic deposit from adenocarcinoma lung with IHC staining positive for TTF-1 [] and negative for desmin, thus ruling out STS. She was started on palliative chemotherapy with cisplatin and pemetrexed, but after 3 cycles, she presented with severe lower back and pelvic pain. WB-18FDG-PET/CT showed new FDG avid metastases to bilateral supraspinatus [], psoas muscles, pelvis, and lumbar vertebrae suggestive of disease progression []. She was treated with lower hemi-body radiation to dose of 8 Gy in 2 fractions. However, despite pain relief, her condition kept on deteriorating and she finally succumbed to her illness within a span of 3 months from diagnosis.


A 54-year-old Saudi male, with a history of uncontrolled diabetes mellitus for the past 10 years presented to the King Fahd Hospital of the University, Al Khobar, Saudi Arabia, with a 6-week history of irregular fever pattern, dry cough, night sweats and weight loss. No other respiratory, genitourinary or gastrointestinal symptoms were reported. In the past 5 years, the patient traveled to the Philippines 2–3 times a year, with his last visit being during the rainy season 2 weeks before the onset of symptoms. The patient had no history of contact with animals or sick patients as well as had no extramarital sexual contacts, intravenous (IV) drug abuse or blood transfusions. Despite seeking medical advice and receiving multiple oral and IV antibiotics, the patient had no improvements.
Physical examination of the lungs revealed decreased air entry in the right upper and middle zone posteriorly, with minimal fine inspiratory crepitations. Complete blood count as well as renal and liver function tests were normal, except for white blood cells, which were 13.2 × 109/L with normal differential count. Complete bacteriology and virology screening were negative. Blood and sputum cultures were also negative.
Imaging in pan-computed tomography (CT) scan revealed multiple, bilateral pleural-based nodules and multiple splenic hypodensities, representing possible abscesses [Figures and ]. The patient underwent flexible bronchoscopy with right video-assisted thoracoscopic surgery. The lung biopsy showed noncaseating granuloma with negative malignant cells, and acid-fast bacilli and fungal cultures were negative. Minimal clinical improvement was observed during a 21-day course of empirical therapy with IV ceftriaxone, but fever continued to spike at lower grades.
Although definite diagnosis could not be made because of insufficient facility in our laboratory, a provisional diagnosis of melioidosis was made based on the cumulative clinical scenario. Accordingly, the patient was discharged on trimethoprim–sulfamethoxazole (TMP–SMX) and doxycycline for 3 months. At the outpatient follow-up, significant improvements were observed with respect to fever and inflammatory markers. For definitive diagnosis, the authors attempted to send another specimen to a more advanced laboratory; however, the patient was lost to follow-up.


A 56-year-old woman with carcinoma of the left breast (for 1 year) and metastasis in the vertebrae and liver was bedridden. Her pain was well controlled with oral tramadol and paracetamol. She had constipation and was on laxatives. She was on Foley's catheter for 9 months. The last catheter change was done 3 weeks back. The patient's daughter reported that the urinary bag was filled with purple-colored urine in the morning []. She had no other symptoms suggestive of UTI. The entire family was very much worried about this sudden change in urine color. They thought that it was due to cancer spreading to bladder and leading to some purple-colored discharge in the urine. The family refused bringing her to hospital for any further investigations or treatment. The family shared the pictures of urine bag and catheter on WhatsApp. She was hypertensive but not on any medications. A provisional diagnosis of PUBS was made and she was started on tablet nitrofurantoin 100 mg twice daily for 2 weeks. The family and the patient were counseled about the benign nature of the condition and the need to increase fluid intake. By 3rdday, the urine had become light colored [] and was back to normal by end of the week. She expired after 4 months at home.
A 75-year-old woman presented with non-Hodgkin's lymphoma for 2 years and moderately differentiated squamous cell carcinoma of the right parotid region (8 months). She had fall and fracture of the neck of the right femur for 7 years and was bedridden and on Foley's catheter. She experienced pain over the right side of the face (10/10) and maggots in the wound for 3 days. She was started on morphine, metoclopramide, bisacodyl, paracetamol, and pantoprazole. Three days later, she presented with purple-colored urine in the catheter and urinary bag. There were no other symptoms of UTI. The last catheter change was done 1 month back. She was started on tablet nitrofurantoin 100 mg twice daily for 2 weeks. The patient was counseled about the benign nature of the condition and the need to increase fluid intake. By 3rdday, the urine had become normal colored. She died 24 days later at home.


A 67-year-old hypertensive and diabetic male weighing 60-kg status postcoronary artery bypass graft surgery to left anterior descending and left circumflex arteries 4 years ago, presented to our institute with a history of chest pain, palpitations, and dyspnea since the last 30 days. Electrocardiogram revealed monomorphic VT during one such episode with a pulse of 170/min and a blood pressure of 110/70 mmHg. He was started on injection Amiodarone 150 mg intravenous (IV) bolus over 10 min followed by infusion of 1 mg/min IV for 6 h then 0.5 mg/min IV for 18 h. The arrhythmia did not revert with this. Injection Metoprolol 5 mg, injection Lignocaine 100 mg, and injection Magnesium sulfate 2 g IV were tried but to no avail. Transthoracic echocardiography (TTE) revealed hypokinesia of apex, anterior, posterior, and inferior walls and septum with a reduced ejection fraction of 25%. No clots were seen during this examination. No previous TTE report was available for comparison. Cardiac enzyme assay was normal and ruled out any active ischemia. He had stopped consuming his routine antiplatelet medications since the last fortnight.
Hypokalemia, hypomagnesemia, and hyperthyroidism were excluded as triggering factors for arrhythmias on laboratory investigations. He was subsequently given Direct current (DC) synchronized cardioversion with 100 J, and normal sinus rhythm (NSR) was achieved. He went into monomorphic VT 8 times on day 1 [] and was reverted only with increasing dose of synchronized cardioversion each time. Decision was made to try sympatholysis with a left-sided SGB (LSGB) on day 2. After checking coagulation parameters, explaining the procedure to the patient and relatives, and taking due consent, the procedure was performed under ultrasound guidance with 10 ml of 0.375% Ropivacaine which was deposited with the tip of the needle in the longus colli muscle after negative aspiration of blood. Spread of the drug was confirmed by bulging of the longus colli compartment and spread of drug on ultrasonography (USG). Horner's syndrome confirmed the success of the block. The patient showed an arrhythmia-free interval of 12 h, but the monomorphic VT continued thereafter with a frequency of 6–7 episodes per day only amenable to synchronized cardioversion. He was intubated during one of these episodes and put on intermittent-positive pressure ventilation. Hypotension ensued and his right internal jugular vein and right radial artery were cannulated, and injection Noradrenaline was started. Sedation with Fentanyl and Midazolam infusion was started. Day 4 prompted a decision to try sympatholysis with a thoracic epidural. After due consent, under all aseptic precautions, in the left lateral position at the T2–T3 interspace, a 18G Tuohy needle was used and the epidural space was encountered with a paramedian approach at 4 cm using loss of resistance to saline method. The catheter was fixed at 8 cm at skin surface and secured with sterile dressing after tunneling. During the next episode of monomorphic VT, 5 ml of 0.5% Lignocaine was injected with return to NSR within 3 min. After seeing this response, an infusion with 100 ml of 1% Lignocaine with 100 mic of fentanyl was started at 5 ml/h with an elastomeric pump. After commencement of the infusion, there was a dramatic reduction in the occurrence of the VT with only one episode on day 5 which settled on bolus of 5 ml of 0.5% lignocaine with 10 mic of Fentanyl through the epidural catheter. Plan was to do an electrophysiological (EP) study and catheter ablation of the arrhythmogenic pathway. Repeat TTE showed a LV clot of 2.3 cm × 2 cm size. The EP study was deferred, and injection Heparin 5000 IU bolus followed by 1000 IU/h infusion was started with regular monitoring of the activated partial thromboplastin time. The epidural catheter was kept in situ with only one episode of monomorphic VT on day 7 which settled on the abovementioned bolus. Relatives did not give consent for surgical left cardiac sympathetic denervation (LCSD). The patient suffered cardiac arrest with ventricular fibrillation on day 8 and succumbed.