A 44-year-old woman with MRKHS attended our outpatient clinic for further investigation and treatment of a pelvic mass. The patient had been diagnosed as having MRKHS at the age of 14 years, when she first sought medical advice for primary amenorrhea. She had a surgical history of open pulmonary valvotomy under cardiopulmonary bypass due to pulmonary valvar stenosis (at the age of 11), and creation of a neovagina in accordance with the Creatsas modification of Williams vaginoplasty (at the age of 16) []. As for the skeletal system, mild kyphosis had been diagnosed at the age of 15.
In 2016, she was admitted to our clinic because of a pelvic mass arising potentially from the ovaries as indicated by a pelvic ultrasound, accompanied with a constant, pelvic pain lasting for 3 months. No urinary or bowel complaints were mentioned. On physical examination, secondary sexual characteristics as well as the external genitalia were found to be well developed. Bimanual examination revealed a solid, rough, mobile, suprapubic mass of approximately 10 cm in diameter. Tumor markers were all within normal limits, and hormonal profile results indicated a normal ovarian function.
Pelvic magnetic resonance imaging (MRI) findings were consistent with a multinodular mass measuring 92×79 mm that was comprised of smaller, discrete masses. It was impossible to differentiate whether the mass arose from the ovaries or a hypoplastic uterus.
Exploratory laparotomy revealed two rudimentary uterine buds joined together in a midline fibrous band with multiple solid masses on it, grossly resembling fibroids (). Ovaries and fallopian tubes looked normal, whereas the cervix could not be palpated. Excision of the redundant piece of uterine tissue, including the tumors, as well as bilateral salpingo-oophorectomy was performed. After an uneventful recovery, the patient was discharged 6 days after surgery.
The histopathological report revealed normal ovaries and fallopian tubes, complete aplasia of the uterus, and multiple leiomyomas arising from either parametrial or paratubal tissue.


A 71-year-old male patient with history of cataract surgery (previous refraction: −12.5 sphere, −1.00 to 0º cylinder), with diffractive lens implant and high myopia (axial length of 30.19 mm) in the right eye, developed a MH 15 days after the cataract surgery, associated with metamorphopsia and decreased visual acuity (VA, count fingers). One month later, PPV with ILM peeling was performed achieving the closure of the MH. Three months later, the visual symptoms returned (metamorphopsia), the VA had not improved (count fingers), and a MH recurrence was observed (700 µm), associated with subretinal fluid, intraretinal hemorrhages, and pigment dispersion ().
A new surgery was carried out in order to achieve the MH closure. The PRGF-membrane was prepared according to Endoret® technology (BTI Biotechnology Institute, S.L., Miñano, Álava, Spain) The surgical procedure consisted in enlarging the peeling of the ILM, close to the optic nerve and the vascular arcade and tap the edges of the MH (to increase the probability of closing the MH, technique proposed by the retina surgeon: AFVS). Then, the PRGF membrane was introduced through the trocar and placed inside the MH (the thickness of the membrane was 100 µm), without pressure and without forcing the adhesion (PRGF fibrin membrane have adhesion properties when it comes into contact with the cell surface) trying to avoid the potential damage to the retinal pigment epithelium; subsequently three drops of liquid PRGF were placed in the MH, and a fluid-gas (C3F8) exchange was performed at the end of the surgery ().
To obtain the PRGF-membrane, 81 mL of the patient’s blood was extracted in 9 mL tubes with 3.8% sodium citrate as an anticoagulant. The blood was centrifuged at 580 g at room temperature for 8 mins (Endoret system®). The fraction 2 (F2) was defined as 2 mL of plasma just above the buffy coat and fraction 1 (F1) was defined as the remaining plasma above F2. To make each PRGF membrane, 5 mL of F2 was activated with 10% calcium chloride and then incubated in 35 mm diameter wells at 37°C for 30 mins. Once the fibrin clots were formed, they were placed in a shaping vessel and pressed for 30 s, yielding 100 µm thick PRGF-membrane. The F1 (liquid) was placed after the PRGF membrane was introduced into the MH. This preparation does not contain cells.
After two months of follow-up, the MH was completely closed. Anatomic recovery of the foveal depression (assessed by SD-OCT) was observed and the mild intraretinal hemorrhages and subretinal fluid disappear, improving the VA to 0.1. An alteration was observed in the outer layers of the retina in the foveal area, but no adverse reactions were associated with the PRGF. We did not have any new recurrences of the MH in a follow-up period of six months, and VA remained stable during this period ().


A 52-year-old (163 cm; 52 kg) male patient diagnosed with asymptomatic severe aortic stenosis (sclerodegenerative) in New York Heart Association Class II presented with a history of syncope. On general examination, the pulse rate was 98 per min, regular with blood pressure of 110/84 mmHg. On auscultation, S2 was single with ejection systolic murmur Grade III/IV. Transthoracic echocardiography revealed severe calcific aortic valve (annulus 26 mm) with peak systolic gradient of 72 mmHg and mean of 43 mmHg, mild aortic regurgitation, concentric left ventricular hypertrophy with 55% ejection fraction, and mild mitral regurgitation. Aortic angiography showed fusiform aneurysmal dilatation of the ascending aorta and arch of aorta to the origin of the left subclavian artery []. Carotid Doppler showed normal bilateral carotids with no significant block or atheroma. In preanesthetic assessment, all laboratory investigations were normal, blood pressure was 112/86 mmHg, and heart rate was 84 beats/min. He was accepted in the American Society of Anesthesiologists physical Grade IV under General Anaesthesia for Bentall debranching procedure.
Preinduction monitoring included electrocardiogram, pulse oximetry, bispectral index, and invasive blood pressure in the right radial and left femoral arteries. NIRS technique was used to measure cerebral oximetry. Two disposable SomaSensor transducers (Invos 5100C, Covidien, USA) capable of producing and detecting optical data from the patient were applied to the left and right sides of the patients’ forehead. Femoral vein and femoral artery were dissected for emergency femorofemoral bypass. After induction of anesthesia and endotracheal intubation, transesophageal echocardiography (TEE) was performed using two-dimensional TEE probe to confirm the diagnosis and to detect any additional findings. No plaque was noted in ascending aorta, arch, and descending aorta in TEE. After heparinization, CPB was commenced using high aortic and two-stage right atrial venous cannulation. After replacing the aortic valve and suturing the proximal end of the tube graft, cannulas were placed separately for the right and left carotid artery for antegrade cerebral perfusion. The distal end of the aortic arch and the left subclavian artery was sutured under deep hypothermic circulatory arrest (DHCA) at 20°C. During DHCA, the antegrade cerebral perfusion through the right and left carotid artery was continued at a rate of 800–1000 ml/min. The institutional protocol for brain protection during DHCA was followed which also included external brain cooling, pharmacological protection in the form of injection thiopentone 3 mg/kg during cooling and rewarming, injection phenytoin sodium 15 mg/kg, injection methylprednisolone 30 mg/kg, and injection lignocaine 2 mg/kg. Total bypass time was 330 min, aortic cross-clamp time of 243 min and DHCA time of 37 min. Monitored parameters (invasive blood pressure mean, cerebral NIRS, temperature, and bispectral index [BIS]) in operation room and intensive care unit are shown in . NIRS values were 72.9 ± 4 and 64.8 ± 4.9 for the left and right hemisphere, respectively. The values never changed more than 11% throughout the procedure.
The patient developed the right hemiparesis postoperative, confirmed with noncontrast computerized tomography head/magnetic resonance imaging which revealed the left middle cerebral artery territory stroke which was embolic []. He was managed conservatively and started recovering with supportive care. The patient was discharged from the hospital after 3 weeks with right hemiparesis.


The patient, a 37-year-old woman, was admitted to our hospital on July 7, 2018, after 5 days of sudden headaches with nausea and vomiting. On July 3, 2018, with no obvious cause for symptoms, her family sent her to the local hospital for treatment. On July 3, 2018, head computed tomography (CT) showed subarachnoid hemorrhage (Fig. ). On July 4, 2018, CT angiography revealed no obvious abnormalities. After she underwent conservative treatment at a local hospital for 5 days, she achieved relief from the headaches. To obtain a diagnosis and further treatment, she visited our hospital. Physical examination revealed a positive meningeal irritation sign and a positive Kernig sign, and the 2 transverse fingers displayed positive cervical rigidity; no other obvious abnormalities were found. There was no history of hypertension, heart disease, diabetes, or other conditions. She denied a history of trauma or surgery. Head digital subtraction angiography (DSA) performed at our hospital on July 8, 2018, showed fenestration at the junction of the middle and upper portions of the basilar artery with an aneurysm; spontaneous pseudoaneurysms could not be excluded, the bilateral posterior communication was obviously open, and the posterior cerebral artery was well developed (Fig. A, B).
The patient underwent stent-assisted fenestration and channel occlusion under general anesthesia. An Enterprise stent 4.5 × 22 mm in size was placed in the normal fenestration malformation channel with the 2 ends of the stent completely covering the openings of the lesion channel. Target 360 NANO (1.5 mm × 3 cm) and helical (1.5 mm × 2 cm, 1 mm × 2 cm) spring coils were placed to fill in the fenestration malformation channel. Intraoperative routine heparin saline irrigation and continuous anticoagulation therapy were performed. After stent placement, tirofiban was administered intravenously at 10 mL/kg/h to prevent angiogenesis in the stents. Following the procedure, the stents were found to be well positioned, with no lesion channel or aneurysm visible (Fig. A, B). After embolization, the patient was lucid and exhibited good limb movement. At 24 hours after the operation, the oral administration of 100 mg of aspirin and 75 mg of Plavix once per day was prescribed to continue the anticoagulation treatment and prevent thrombosis in the stents. Five months later, no abnormalities were found by head magnetic resonance imaging (MRI). The stents were well positioned, and no occluded branches or aneurysms were present (Fig. A, B).
Informed written consent was obtained from the patient for publication of this case report and accompanying images.


Our patient, a 45-year-old man residing in Dhofar, was a keen diver with an interest in harvesting Haliotis mariae during the traditional fishing season. Occasionally, he injured his hands with sea urchin spines with the injuries usually resolving within hours to days. He presented to the dermatology clinic two months after his last injury with multiple swellings over the dorsum of both hands. Few swellings were set over joints, but there was no joint pain or impairment of joints mobility. According to the patient, the sites of injury were initially red and painful and became infected a few days later. However, it subsided following administration of oral antibiotic prescribed by the local health center.
His general condition was good. Dermatological examination of dorsum of hands revealed 11 nodular swellings. They were brown, 3–5 mm in diameter, non-tender, firm in consistency, and slightly hyperkeratotic with central hypopigmentation []. Both hand joints examination showed non-painful, normal range of active and passive motions. X-ray examination did not reveal any radiopaque spine fragments. Skin biopsies were taken from two of the granulomas. Hematoxylin and eosin stained sections of one biopsy showed non-specific chronic inflammation with focal suppuration along with foreign body material. The other specimen showed epithelioid sarcoid type granuloma [ and ].
The patient was managed by intralesional steroid (triamcinolone) injections every month, most of the nodules resolved after two months. However, three nodules needed an additional two months to resolve. Subsequent follow-up after five months showed complete recovery.


A 52-year-old female, Para-3, living-3, and not sterilized was evaluated for menorrhagia. Clinically, the uterus was approximately 14–16 weeks' size and anteverted, and no fornicial tenderness noted. The radiological diagnosis revealed a large fibroid measuring 4 cm × 4 cm on the ultrasonogram. She underwent hysterectomy with bilateral salpingo-oophorectomy. Grossly, the uterus with cervix measured 14 cm × 9 cm × 6 cm. Cut section, endometrium measured 0.1 cm in thickness. Myometrium measured 4 cm and showed trabeculated areas. Submucosal fibroid measuring 4 cm in diameter was also noted. Endocervical canal measured 1.5 cm and showed a single nabothian cyst. The left side ovary measured 2.5 cm × 2 cm × 0.5 cm, appeared unremarkable on cut section. The left fallopian tube measured 4 cm in length with patent lumen. The right side ovary measured 2.5 cm × 1.5 cm × 0.5 cm. On cut section, a tiny brown area measuring 0.1 cm in diameter was noted grossly []. The right fallopian tube measured 4 cm in length. External surface showed focal congestion over an area of 0.1 cm, and on cutting, lumen appeared unremarkable.
Microscopically, sections from cervix showed features of papillary endocervicitis with a single nabothian cyst; endomyometrium showed secretory endometrium, with submucosal leiomyoma and adenomyosis. The left adnexa showed unremarkable histology. The right ovary showed lobular proliferation of closely packed capillaries with scant intervening stroma []. Right fallopian tube wall also demonstrated similar morphology with proliferation of closely packed capillaries []. A Walthard cell nest was also noted. Histological diagnosis was confirmed with immunohistochemical marker CD 31 and CD 34 []. These vascular endothelial markers highlighted the capillaries in the lesion of the ovary and the tube.


One of the subjects was a 54-year-old male who has suffered from tinnitus for 4.5 years at that time. He was doing manual labor at a construction site which was a very loud noisy environment. He had slight hearing loss of 30 dB in the left ear and 20 dB in the right ear at a high frequency of 8 kHz. The mean right and left hearing thresholds in the frequency range from 250 to 8,000 Hz were 14.3 (±4.5) and 15.7 (±6.7) dB, respectively ( in the online-only Data Supplement). He complained that the tinnitus intensity was always loud, usually worsening on work days with a better tinnitus status on his days off.


The other patient was a 26-year-old female who complained regarding her chronic hyperacusis. The onset was during middle school; thus, her hyperacusis symptoms had been ongoing for almost ten years at the time of her clinical. She had physical symptoms of hyperacusis in the ipsilateral right ear. When the middle ear muscles were contracted and an ear spasm developed due to an unexpected loud noise, a rustling sound was heard in her ear []. She felt only unpleasant at the moment when there was a physical symptom in her ear, especially on the right side. Her hearing was within normal range in right and left sides. Her mean hearing in the frequency range from 250-8,000 Hz was 5.7 (±3.5) and 4.3 (±1.9) dB in the right and left ears, respectively ( in the online-only Data Supplement).


A 60-year-old postmenopausal female presented with complaints of bleeding per vagina and lower abdominal pain for 4 months. She had four children, and there was no history of any complications during her pregnancy. There was no history of use of any contraception or exogenous hormone intake.
Her past medical and surgical history was unremarkable. No significant findings were noted in her family. Ultrasonography (USG) of the pelvis was done in another hospital which revealed a hypoechoic left adnexal mass measuring 65 mm × 40 mm × 30 mm. Based on these USG findings, the diagnosis of cystic lesion of the left ovary was made by the concerned radiologist, and unfortunately, the radiological image of the same was unavailable. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was done, and the specimen was sent to our department for histopathological examination.
We received a gross specimen of uterocervix with attached right adnexae, uterocervix measuring 8.5 cm × 5 cm × 2.5 cm in size. The cut section of uterocervix was unremarkable. The left ovarian mass was received separately and measured 6.5 cm × 4 cm × 3 cm. The cut surface of the left ovary showed solid, yellow mass replacing the whole ovary without any peripheral ovarian stroma []. Other side ovary was grossly normal.
Sections from the left ovarian mass showed well-circumscribed tumor tissue arranged in lobules, nests, and cords. Individual tumor cells were large, round-to-polygonal showing mild anisonucleosis with round-to-oval centrally placed nuclei with prominent nucleoli and abundant amount of eosinophilic cytoplasm. Occasional mitotic figures were also seen. There was no atypia or necrosis noted in the tumor. Histological features were consistent with steroid cell tumor-NOS type []. The cervix showed features of chronic cervicitis with squamous metaplasia and nabothian cyst, whereas the endometrium showed atrophic changes. The other side ovary and tube were unremarkable.


A 26-year-old, gravida three, para two, female with a history of one lower segment cesarean section and one normal delivery was incidentally detected to have placenta percreta on prenatal ultrasound imaging at 32 weeks of gestation. On further investigation, MRI abdomen and pelvis were suggestive of a complete placenta previa with percreta with invasion of dome of the bladder [Figures and ].
The patient was managed conservatively till 36 weeks. On the day of planned elective cesarean section, prophylactic bilateral internal iliac balloon catheters were placed through the right femoral artery. The patient was then shifted for surgery. Intraoperatively, placenta was found to be densely adherent to the uterine wall, and decision was taken to leave it in situ. A healthy baby was delivered, weighing 2250 g, and the patient was shifted to the postnatal ward. On the 3rd day after cesarean section, 80 mg of intravenous methotrexate was administered to the patient and was planned to be given weekly, up to a maximum of four doses. Beta-hCG levels were monitored, with an initial figure of 560 mIU/ml. On postoperative day 7, the patient had an episode of gross hematuria with passage of clots. Cystoscopy revealed diffuse oozing on the posterior wall of the bladder. 200cc of clots were evacuated, and bleeding points were cauterized. Postoperatively, the patient was stable and urine was clear. She was given the second dose of intravenous methotrexate, and her beta-hCG levels were now 386 mIU/ml. Two days after the first episode, the patient developed another episode of gross hematuria with passage of clots. On repeat cystoscopy, the bladder clots were organized, and 300cc clots were removed by a suprapubic cystostomy. The patient was kept in the surgical intensive care unit and monitored postoperatively. Five days later, she had a third episode of gross hematuria. A repeat MRI abdomen with pelvis was done which was suggestive of 12 cm × 10 cm × 6 cm retained placenta, invading into the bladder, with clots in the bladder lumen [].
The patient was shifted for exploration, wherein the bladder clots were evacuated through an anterior cystotomy, and bilateral internal iliac vessels were ligated. However, there was persistent hemorrhage. A subtotal hysterectomy was performed with partial cystectomy of the focal infiltration of placenta in bladder. The bladder was repaired primarily, and a wide bore perurethral catheter, a suprapubic catheter, and bilateral DJ stents were kept. The patient was extubated the next day. She required six blood transfusions, four bags of fresh frozen plasma, and one bag of platelets. The suprapubic catheter and DJ stents were removed after 4 weeks on confirming a normal cystogram, and the patient was discharged.


A 72-year-old man with previous myocardial infarction and impaired left ventricular (LV) function underwent implantation of an implantable cardioverter-defibrillator (ICD) device (AnalyST Accel™ CD1219-36, Abbott®) for primary prevention of sudden cardiac death in 2006.
During the follow-up period, he received an appropriate shock for a monomorphic VT at a rate of 200 beats per minute (bpm). He initiated therapy with amiodarone 200 mg once a day. In May 2018, he was observed for symptoms of heart failure (HF) and a slow VT at a rate of 145 beats per minute (bpm) was detected and converted to sinus rhythm with burst of anti-tachycardia pacing (ATP). The patient was proposed for VT ablation and the ICD was reprogrammed with three tachycardia zones (Table ).
In July 2018, the patient was admitted to the hospital for acute HF. He described the sensation of a shock two weeks before. The tracing of the device interrogation of the episode (Figure ) showed a trigeminal pattern binned as ventricular sensing (VS)—VS—VF that continued for 14 seconds and VF was detected (denoted by the “Trigger” annotation). Device assumes VF as cycle length was 316 ms; and after 12 intervals, a 36 joule (J) shock therapy was delivered (“HV” annotation) with conversion to ventricular pacing.
He underwent catheter ablation guided by substrate mapping (EnSite NavX, Abbott). Radiofrequency applications were performed at the borders of the posterior scar, in the channels within the scar and at sites with local electrograms showing diastolic potentials. The patient was discharged with amiodarone 200 mg once a day and bisoprolol 5 mg once a day and tachycardia zones were reprogrammed: VF at a HR ≥ 206 bpm (CL 290 ms), VT-2 at HR between 160 and 206 bpm (CL 375–290 ms) and a slow VT-1 zone as a monitoring zone between 148 and 160 bpm (CL 405–375 ms). Tachycardia was diagnosed once 16, 12 and 20 recorded intervals were within VF, VT-2 and VT-1, respectively. After 6 months of follow-up, the patient remains in NHYA class II and no VT/VF were detected or ICD therapies were performed.


A 65-year-old male patient with crescendo angina underwent a coronary angiogram (CAG) in December 2016 through the right radial route. He had a critical right coronary artery (RCA) lesion, and he underwent a staged PCI to RCA.
He had mild pain in the forearm; neither a hematoma nor a bruit could be felt over the forearm, and the distal radial pulse was palpable. We proceeded with PCI to RCA through right radial route initially; however, as the Terumo wire could not cross forearm, we converted to the femoral route and completed PCI. He was discharged after 48 h.
He came back after a week with swelling, redness, bruit, and severe pain over the right forearm. USG showed a large 6 cm × 8 cm pseudoaneurysm of radial artery extending into intramuscular plane. He underwent USG-guided differential compression of the pseudoaneurysm with complete thrombosis and resolution of the aneurysm [Figures –].


A 74-year-old hypertensive, diabetic and hypothyroid female patient with coronary artery disease underwent PCI to RCA in June 2006. She presented to us with acute coronary syndrome, severe LV dysfunction and congestive heart failure. Coronary angiography (right radial approach) after stabilization showed critical RCA disease proximal to previous stent and critical left circumflex artery (LCX) disease (proximal and involving two major obtuse marginals) with minimal left anterior descending (LAD) disease. She underwent ad hoc PCI to RCA. PCI to LCX was planned at a later date. She presented 1 week after the index procedure with swelling at the right wrist. USG Doppler showed a large pseudoaneurysm of radial artery. USG-guided compression was performed as described with successful thrombosis and resolution of pseudoaneurysm [Figures –, Videos and ].


A 69-year-old female presented with acute anterior wall myocardial infarction (AWMI), thrombolysed with reteplase at another facility. There was a history of recurrent primary ventricular tachycardia requiring electrical cardioversion. She underwent CAG though right radial route. She had swelling 3 days postcatheterization in the forearm. USG revealed a well-defined hypoechoic lesion of size 3.8 cm × 1.8 cm × 1.5 cm arising from the origin of right radial artery extending superiorly over brachial artery with a thick wall and inflammation around. As the pseudoaneurysm was originating from proximal radial artery and extending directly over the brachial artery with large hematoma in the forearm, we ruled out USG-guided compression. Thrombin injection was also not considered in view of proximity to brachial artery. She underwent surgery with successful repair of pseudoaneurysm.


A 69-year-old female presented with acute coronary syndrome, non-ST-elevation myocardial infarction, mild LV dysfunction. CAG showed triple vessel disease (TVD) with critical LAD, moderate RCA, and LCX disease. She underwent PCI to LAD. Post-PCI, she developed a painful swelling at the right wrist. USG revealed an 11 mm × 7 mm distal pseudoaneurysm at wrist level, successfully treated with USG-guided compression.
The sheath sizes, antiplatelet and anticoagulation protocol in our patients, mode of hemostasis achieved, duration between index case intervention and recognition of Rap, and treatment modality for RAp are summarized in .


A 61-year-old woman with Kartagener syndrome attended our pulmonology clinic for a routine check-up with complaints of watery diarrhoea for 3 months and progressive bilateral lower leg swelling for 2 months. A month earlier, she had undergone gastrointestinal endoscopy without biopsy at a nearby clinic, but no abnormalities were detected. She did not take any medications and denied arthralgia, abdominal pain, rash and appetite loss. She had mild bronchiectasis due to Kartagener syndrome and had been evaluated annually for 3 years without symptoms.
On physical examination, she was alert and oriented. Her blood pressure was 105/66 mmHg, pulse rate 68 beats/minute, respiratory rate 16 breaths/minute, oxygen saturation 100% on room air and temperature 36.5°C. Abdominal examination revealed hyperactive bowel sounds and no pain on palpation. Laboratory findings showed severe inflammation and hypoproteinemia (white blood cell count, 6600/μL; neutrophils, 72.9%; C-reactive protein, 9.97 mg/dL; erythrocyte sedimentation rate, 60 mm/h; total protein, 5.7 g/μL; albumin, 1.5 g/μL). Her haemoglobin was 14.1 mg/μL. Urinalysis showed no proteinuria (including Bence Jones protein). Rheumatoid factor and anti-cyclic citrullinated peptide antibody were negative. The serum kappa/lambda free light chain ratio was 1.039 (normal). Serum and urine immunofixation electrophoresis showed no monoclonal band. A faecal sample showed occult blood and white blood cells. The Clostridium difficile toxin assay and stool culture were non-contributory. Echocardiography showed no abnormality.
Gastrointestinal endoscopy and colonoscopy were performed again to obtain diagnostic samples. Duodenum and large intestine biopsies showed remarkable amyloid deposition (Fig. ), later confirmed as AA amyloidosis by immunohistological reactivity with anti-AA antibody. Bone marrow aspirate and biopsy did not show monoclonal plasmacytosis. Alpha-1 anti-trypsin clearance, which reflects the value of enteral protein loss, was 300 mL/day, and 99mTc-diethylenetriaminepentaacetic acid human serum albumin scintigraphy showed protein leakage in the gastric body. Thus, we diagnosed protein-losing gastroenteropathy caused by AA amyloidosis. Repeat positron emission tomography and computed tomography (CT) with contrast showed a pelvic mass distant from the ovaries and intestines with a standardised uptake value of 22.9.
Upon hospitalisation, we initiated a low-fat, high-protein diet and administered supplemental parenteral nutrition including albumin through a central venous catheter. On hospital day 12, octreotide was started at 100 μg/day to suppress her diarrhoea. We planned to improve her nutritional status and then perform elective surgery. Steroid therapy was suspended to avoid the risk of postoperative wound dehiscence. Despite multidisciplinary treatment, her hypoalbuminaemia worsened (albumin, 1.0 g/μL). Laparoscopic pelvic mass resection was then performed for diagnosis on hospital day 30. A huge rectal mesentery mass and all mesenteric lymph nodes were removed.
The tumour was a well-demarcated mass measuring 60 × 50 mm (Fig. ). Microscopically, the mass on mesentery comprised a solid sheet of anaplastic cells with prominent necrosis (Fig. ). There was a compressed lymphoid structure at the periphery of the tumour, suggestive of lymph node metastases. On immunohistochemical analysis, the tumour was positive for pan-cytokeratin (AE1/AE3) and partially positive for hepatocyte paraffin 1 (Hep-Par1). However, it was negative for cytokeratin (CK) 7, CK20, calretinin, chromogranin A, synaptophysin, Melan-A, p40, S-100, cluster of differentiation (CD) 30, anaplastic lymphoma kinase and CD45. Considering the tumour location, morphological structure and immunohistochemical phenotype, we concluded that it was an undifferentiated carcinoma of unknown primary origin with multiple lymph node metastases.
After diagnosis, the follow-up plan was monthly observation with CT and PET-CT because all tumours were removed during surgery. Diarrhoea improved gradually. On hospital day 53, her albumin recovered to 2.5 g/μL; the central venous catheter was then removed. On hospital day 77, she was discharged home in good condition with monthly appointments. Her most recent CT and laboratory evaluations performed 6 months postoperatively showed no signs of carcinoma recurrence.


A 61-year-old elderly male was referred by a local ophthalmologist to the retina clinic of a tertiary eye hospital with a history of sudden drop in vision in the left eye (LE) for 6 days. On passing through the patient’s documents, the local ophthalmologist had noted the presence of preretinal heme obscuring the ONH. He was recently diagnosed with non-insulin-dependent diabetes mellitus for 1 year. His blood pressure on presentation was recorded at 180/100 mm Hg, and he was started on anti-hypertensive medications. On examination, his best-corrected visual acuity (BCVA) was counting fingers close to face in both eyes (BE). Intraocular pressure (IOP) measurement using the Goldmann Applanation tonometer was 14 and 16 mm Hg in the right eye (RE) and LE, respectively. Gonioscopy revealed open angles in BE. Anterior segment examination of BE was normal. A dense nuclear cataract with post-subcapsular cataract was noted in the RE. Fundus examination of the RE revealed a hazy media with a cup:disc ratio (CDR) of 0.8:1. Other finer details of the fundus were not appreciated clearly due to dense cataract. LE was pseudophakic with a posterior chamber intraocular lens. LE fundus examination showed a clear media with CDR of 0.8:1, dilated retinal vessels and AV crossing changes, and cotton-wool spots suggestive of hypertensive retinopathy. Multiple layered preretinal and subretinal blood was surrounding the optic disc and macula (). Fluorescein angiography (FA) was done to identify the source of heme in the retina. FA revealed blocked retinal and choroidal fluorescence due to the presence of the overlying preretinal and subretinal haemorrhage in the LE. A small focus of light-bulb hyperfluorescent lesion at the centre of the ONH corresponding to the macroaneurysm was noted in the progressive phases of the FA (). RE showed an old inferotemporal vascular occlusion with areas of capillary non-perfusion along the inferotemporal quadrant and involving the macula as well. A diagnosis of ONH RAM with fresh premacular and submacular haemorrhage in a hypertensive patient was made in the LE. In view of the dense cataract in the RE and a co-existent retinal pathology in the LE, the patient was advised simultaneous cataract surgery in the RE and intravitreal 0.4 cc of 100% perfluoropropane (C3F8) gas injection with prone positioning in the LE after lowering the IOP with anterior chamber paracentesis. One week post treatment, his BCVA in BE was counting fingers at 3 m. LE fundus examination showed a resolved preretinal haemorrhage and altered subretinal haemorrhage with a residual gas bubble. At 3-week follow-up, his LE BCVA improved to 6/18, N6. LE fundus examination showed resolving retinal and subretinal haemorrhage (). Optical coherence tomography (OCT) of the LE through the macula showed a normal foveal contour and a thin layer of subretinal blood with shadowing nasal to the fovea. High-definition OCT images (Zeiss Cirrus HD OCT-500) of the left ONH identified a bulbous lesion in continuation with the retinal vessel sitting within the ONH. This lesion corresponded to the hyperfluorescent lesion seen on the ONH head on FA. We believe this lesion could be the possible ONH RAM (). RE BCVA was counting fingers at 3 m. FA revealed an inferotemporal retinal vein occlusion with macular ischaemia in the RE. Fundus examination at 2 months post treatment revealed a small residual altered subretinal bleed in the LE. His BCVA in the LE improved to 6/12, N8 (). Written consent was obtained from the patient for the usage of data and images for this report. Institutional review board approval was taken for this particular case report (SCEH/17/005).


An adolescent male aged 15 years and three months was diagnosed in another hospital
with cSLE based on the presence of arthritis, proteinuria (1.2 g/day), lymphopenia,
thrombocytopenia and 1:640 positivity of antinuclear antibodies (speckled pattern),
as well as anti-double stranded DNA antibodies (ELISA, > 200 IU/mL [positive ≥ 20
IU/mL]) and low complement levels (C3- 43 mg/dL [normal range 90-180 mg/dL]), C4-
4.5 mg/dL [normal range 10-40 mg/dL]).
Routine non live immunizations (tetanus, hepatitis B, seasonal influenza,
pneumococcus and Haemophilus ) had been previously administered. At
16 years and three months, he was hospitalized at our University Hospital with
fever, myalgia, arthritis, vomiting, dyspnea and clinical signs of sepsis. There
were no meningeal signs. On the same day, he developed a severe and acute headache,
followed by generalized tonic-clonic seizures that required orotracheal intubation.
The chest X-ray showed perihilar infiltrate on the right side. Laboratory exams
revealed: hemoglobin 13.1 g/dL (normal range 14-18 g/dL), hematocrit 37%, white
blood cell count 10,100/mm3 (1% metamielocytes, 15% bands, 68% segmented
neutrophils, 6% lymphocytes and 10% monocyte), platelets 70,000/mm3,
proteinuria (3.2 g/24 h), urinalysis (leukocytes 6,000 high-power field and
erythrocytes 432,000 high-power field), urea 56 mg/dL (normal range 10-42 mg/dL),
creatinine 3.59 mg/dL (normal range 0.5-0.9 mg/dL), C3- 25 mg/dL (normal range
90-180 mg/dL), C4- 2.1 mg/dL (normal range 10-40 mg/dL), and C-reactive protein 368
mg/dL (normal range <5 mg/dL). No abnormalities were observed on the cranial
tomography and the echocardiography. A lumbar puncture was performed, and the
cerebrospinal fluid (CSF) analysis showed the following: white cell count
36/mm3 (88% neutrophils, 8% lymphocytes), glucose 16 mg/dL (normal
range 40-80 mg/dL) and protein 456 mg/dL (normal range 15-40 mg/dL). The initial
clinical diagnoses were bronchopneumonia and meningitis, and the treatment included
intravenous hydration, antibiotics (vancomycin, amikacin, meropenem and micafungin),
vasoactive and inotropic drugs (norepinephrine, dobutamine and milrinone),
mechanical ventilation and continuous renal replacement therapy.
Immunological tests revealed positive results for anti-dsDNA antibodies and IgG
(58.79 GPL/mL) and IgM (31.45 MPL/mL) anticardiolipin antibodies. On the third day
of hospitalization, peripheral blood culture identified Neisseria
meningitidis serogroup Y with intermediate sensitivity to penicillin.
The CSF culture was negative. Ten days after admission, the SLEDAI-2K was 21 and pulse therapy was administered without meaningful response. His
oxygenation index and ventricular function progressively worsened. At this point, a
second echocardiograph showed left ventricle ejection fraction of 49% (normal ≥ 55%,
by the Teichholz method) and mild enlargement of the left ventricle and both atrium
chambers. Despite all therapeutic and supportive measures, he died after 29 days of
hospitalization. Serology tests for hepatitis (A, B and C), HIV, Epstein Barr virus,
cytomegalovirus and toxoplasmosis were all negative.
The autopsy was performed after written consent was obtained from his next of kin. It
revealed bilateral and extensive pneumonia, mild pleuritis and pericarditis, active
diffuse proliferative nephritis and foci of chronic cerebral abscess in the left
temporal cortical area. Remarkably, the heart was heavy, weighting 532 g (reference
range 200-350 g) and stiff, and the cut surface had golden-yellowish rough areas
scattered in both ventricles ( ).
Under microscopy, these yellowish and rough areas were necrotic cardiomyocytes with
dystrophic calcification in their cytoplasm that were associated with interstitial
myocardial edema and fibrosis, without myocardial vasculitis ( , , , ).
Von Kossa staining highlighted the calcifications in black ( ). The pericardium, coronary vessels and
atrioventricular valves were preserved.
Myocardial analysis by electron microscopy showed severe damage to cardiomyocyte
fibers and mitochondria, with intense myocytolysis ( ), edema, cristolysis and calcium accumulation in mitochondria
( ). In more intense lesions,
calcification takes over the cellular content ( , ). Interstitial and
endothelial edema were other findings.


A previously healthy 29-year-old male presented to the emergency department (ED) with crushing chest pain. He reported one week of severe, episodic pain occurring at rest, associated with radiation to the scapula, diaphoresis, and nausea. He also reported palpitations beginning two weeks prior, which prompted an outside hospital ED visit. He was diagnosed with hyperthyroidism at that time based on low thyroid-stimulating hormone (TSH) and high free T4. He denied other symptoms of hyperthyroidism including diarrhea, anxiety, or weight loss. He was started on propranolol for symptomatic treatment and referred to endocrinology. After one week of therapy, the palpitations resolved but his chest pain ensued, prompting presentation to our ED.
Upon arrival, troponin was elevated at 6 ng/mL (reference level < 0.02 ng/mL). He was admitted and received aspirin, clopidogrel, and enoxaparin for suspected acute coronary syndrome. Over the next 4 hours, the patient's chest pain subsided. His troponin peaked at 10.57 ng/mL. Electrocardiography demonstrated sinus tachycardia with abnormal ST-segment convexity in V3-V6 and marginal ST-segment depression in III and aVF (Figure ). Repeat thyroid studies demonstrated undetectable TSH (reference level 0.45-4.5 mIU/L) and elevated thyroid hormones including free T4 of 3.5 ng/dL, total T4 of 17.5 µg/dL, and free T3 of 12.1 pg/dL, consistent with hyperthyroidism (reference levels 0.7-1.5 ng/dL, 4.9-11.0 µg/dL, and 2.3-4.2 pg/mL, respectively).
Given the patient's young age and lack of significant cardiovascular risk factors, cardiac magnetic resonance imaging (CMR) was performed, demonstrating diffuse left ventricular edema and subepicardial late gadolinium enhancement in the mid-to-distal anterior and anterolateral walls (Figure ). There was mild hypokinesis of the corresponding areas with a global left ventricular ejection fraction of 54%. Similar mild regional wall motion defect was noted on limited echocardiogram. These findings were consistent with a clinical picture of acute myocarditis. Because these findings did not support a diagnosis of acute coronary syndrome, cardiac catheterization was not pursued.
The concurrent presentation of acute myocarditis and acute hyperthyroidism raised our suspicion for a viral etiology. While the patient did not recall a preceding illness, his occupation as a dialysis nurse was considered a risk factor for exposure. Given that viral infection is the most commonly identified cause of myocarditis, subclinical hyperthyroidism secondary to a virus was the suspected underlying etiology. In consultation with endocrinology, the patient was started on a beta-blocker to control tachycardia and a nonsteroidal anti-inflammatory medication, as is standard of care for viral thyroiditis. The decision was made to switch from propranolol to metoprolol as the two medications have equivalent efficacy in the treatment of thyroid disease,, while metoprolol additionally has well-established efficacy in the treatment of heart failure. Given his stability, he was discharged with an arrangement for a follow-up visit.


A 45-year-old woman was examined for a tumor in her left breast. The patient had no significant and relevant medical or family history. Upon physical examination, an elastic hard mass of around 5 cm along with accompanying skin flare and ulceration was palpated in the upper outer quadrant of the left breast (Fig. ). Multiple lymph nodes in the left axilla were also palpable. Mammary ultrasonography revealed a clearly marginated hypoechoic tumor of 55.0 × 46.9 × 37.0 mm in size in the upper outer quadrant of the left breast (Fig. ). A vacuum-assisted biopsy (VAB) was performed in the same site and histopathological diagnosis of PMC was made (Fig. ). The immunohistochemical staining revealed a strongly positive diffused expression of estrogen receptor (ER) and progesterone receptor (PgR), negative expression of human epidermal growth factor receptor 2 (HER2), and high expression of Ki-67. Computed tomography (CT) exhibited no distant metastases, although axillary lymph node metastases were suspected. Contrast-enhanced magnetic resonance imaging (MRI) showed a T1W1 low-intensity signal and a T2W1 high-intensity signal at the primary focus, ring enhancement of the tumor margin, and stranding enhancement inside the tumor (Fig. ). Bone scintigraphy also revealed no bone metastasis. A preoperative diagnosis of left breast cancer (PMC), cT4bN1M0, stage IIIB, luminal B-like was made.
We performed a simple mastectomy with axillary lymph node dissection. A 55.0 × 48.1 × 37.1 mm tumor with the gelatinous cut surface was excised (Fig. ). Histopathological examination of the excised specimen revealed mucin lake formation in the tumor containing clusters of atypical cells (Fig. ). The atypical cells showed swollen, irregular nuclei and a papillary growth pattern that lead to the diagnosis of MUMPC. The expression of ER and PgR were diffused and strongly positive, HER2 expression was negative, and Ki-67 expression was high. Based on these findings, the final diagnosis of left breast cancer (MUMPC), pT3N0M0, stage IIB, luminal B-like was made. Adjuvant chemotherapy was initiated after the surgery (adriamycin and cyclophosphamide [AC] followed by weekly paclitaxel) [, ] and an endocrine therapy was planned (tamoxifen 20 mg/day) []. No metastatic recurrence has occurred until the time of drafting this case report, 3 months after the surgery.


A 45-year-old Japanese man with epigastric pain and itchy skin with rash of his arm, chest, and back reportedly ate vinegar-marinated raw mackerel sushi at a ramen restaurant 11 h prior to the development of the epigastric pain. His vital signs were normal. Physical examination showed moderate abdominal tenderness without rebound or guarding in the epigastrium.
On the basis of his clinical history and physical examination, we suspected gastric anisakiasis and performed an emergency endoscopic examination. We used a GIF-H290Z (Olympus Medical Systems) endoscopic apparatus and detected two white larvae penetrating into the thick mucosa of the cardiac part of the stomach. However, this identification was particularly challenging with white light imaging (WLI) because the surrounding mucosa was edematous and erythematous (Fig. ). Using narrow-band imaging (NBI) technology (structure enhancement function B8), the larvae were emphasized and contrasted clearly against the mucosal layers. We identified two larvae measuring approximately 15 mm in length. We could see the movement of the tails of the larvae during the procedure (Fig. ).
For inhibiting gastrointestinal spasms and weakening the larval movements, we sprayed L-menthol (MINCLEA, Nihon Seiyaku, Tokyo, Japan) onto both larvae on site. After the spray, the larval movements got weaker and they changed their shape from circles to straight lines in approximately 10 s (Fig. ). We were then able to easily catch the head portions of the larvae using endoscopic forceps (Fig. ). After the removal of the larvae, we verified the absence of remnants of the larvae in the stomach (Fig. ). After the procedure, the patient's symptoms completely and immediately resolved.
Morphological examination revealed the two larvae as being in the third-stage of A. simplex (Fig. ). For verifying the species, we performed molecular analyses of the removed specimens by sequencing and confirmed them as A. simplex sensu stricto.


A 91-year-old physically inactive man (Minimental State Examination 26 pts, height 165.5 cm, body mass 58 kg, BMI 21.2 kg/m2) was recruited from the Frailty in Brazilian Older People Study (FIBRA-RJ). In his admission, the patient reported excessive exhaustion, muscle atrophy in upper and lower limbs, muscle weakness in lower limbs, and history of multiple falls. Family history did not reveal any disease related to these symptoms. The patient had been diagnosed with systemic arterial hypertension and, in 2011, underwent angioplasty for placement of two coronary stents. He had preserved ventricular systolic function and left ventricular diastolic function with relaxation alteration pattern (E/E’ ratio: 8, reflecting normal filling pressures in the left atrium). No clinical abnormalities were found in carotid and vertebral ecocolor Doppler.
In 2014, he was classified as sarcopenic, with appendicular skeletal muscle mass (ASM) index of 7.10 kg/m2 measured by dual-energy X-ray absorptiometry (DXA). In the pre-participation clinical evaluation, the patient performed a pharmacological myocardial perfusion scintigraphy and did not exhibit abnormalities suggestive of ischemia. During the strength training intervention, the patient was under daily prescription of Losartan Potassium 50 mg, Carvedilol 6.25 mg, Warfarin 5 mg, Finasteride 5 mg, Spirolactone 25 mg, and Simvastatin 20 mg.


The patient was a 78-year-old Caucasian woman, with a medical history including type 2 diabetes mellitus, hypertension and gout, who presented to the emergency department with disoriented speech, a temperature of 40°C (104°F), hypertension and tachycardic. There was an initial concern for heat stroke when she was admitted to the hospital considering the high temperature and humidity outside. She was admitted to the general medical floor where she rapidly deteriorated and developed respiratory distress with oxygen saturation declining to 86%. She was placed on non-invasive positive pressure ventilation (NIPPV) and transferred to the critical care unit (CCU). On CCU arrival, the patient’s vital signs showed a temperature of 39°C (102.3°F) with her blood pressure spiking to 204/74 mm Hg. She remained in sinus tachycardia at a rate of 124 beats/min and respiratory rate of 23 while on NIPPV. Physical exam findings revealed worsening mental status, severe nuchal rigidity and positive Brudzinski’s sign (forced flexion of neck causes reflex hip flexion) and Kernig’s sign (flexing at the hip and knee at 90° and subsequent knee extension is painful and patient resists).
On initial CCU history and chart review, it was found that she had no recent history of trauma or illness. She was taking amlodipine, atorvastatin, benazepril, calcium carbonate, glipizide, sitagliptin/metformin, insulin glargine and lisinopril at home. Her initial lab values showed multiple abnormalities, including hypokalaemia with a potassium of 3.3 mmol/L, glucose of 11.4 mmol/L mg/dL, Blood Urea Nitrogen (BUN) of 13.57 mmol/L, serum creatinine of 214.86 μmol/L, magnesium 0.64 mmol/L, phosphorus of 0.484 mmol/L, platelets at 86×109/L and a white blood cell (WBC) at 12.44×109/L.


A 42-year-old male patient presented with difficulty in walking since 15 days, pain and tingling sensation in the right leg since 6 months. The symptoms aggravated in night and were relieved after taking rest. No bowel or bladder complaints were reported. There was no previous history of hospitalization for any medical or surgical illness. The neurological evaluation showed decreased motor power of right lower limb with no sensory deficits.
An isodense, heterogeneous, intradural, extramedullary, enhancing mass lesion extending from lower margin of D12 to upper margin of L2 vertebra, at the level of conus medullaris extending up to the beginning of cauda equina was noted [Figure and ]. No evidence of extraspinal invasion was seen. As the lesion was extramedullary and showed extension into the right neural foramina [], radiological impression was kept as a neurogenic tumor-Schwannoma/ependymoma/meningioma/neurofibroma. The clinical diagnosis was also kept the same.
Tumor was identified to be intradural, extramedullary with no extension in surrounding extraspinal, paravertebral soft tissue. Laminectomy and excision of tumor followed by duroplasty was done. Excised tumor was submitted for histopathology.
Multiple gray white tissue bits aggregating to 3 cm × 2.5 cm × 2 cm were received. Histopathological examination showed a capsulated tumor [] comprising of three distinct types of cell populations.
Predominant cells were tumor cells (neuroendocrine cells) which were small, round to polygonal, with pale eosinophilic cytoplasm, round to oval nuclei, stippled chromatin, forming solid nests, and trabeculae [].
Ganglion cells having irregular contour, abundant cytoplasm, large vesicular nuclei with prominent nucleoli; were seen scattered and intermingled with neuroendocrine cells [].
Spindle (interstitial) cells, representing Schwann cells, were present along with branching vascular channels forming fine delicate network around the predominant cell type-giving rise to zellballen pattern [Figure and ].
No evidence of pleomorphism, hyperchromasia, mitotic activity, and necrosis was noted.
Thus, the provisional histopathological diagnosis of “GP” was given.
The tumor cells showed diffuse immunohistochemical expression of chromogranin, synaptophysin and neuron-specific enolase (NSE) supporting neuroendocrine differentiation. Ganglion cells expressed NSE, along with chromogranin and synaptophysin. Spindle cells showed S-100 positivity. The findings proved the microscopic diagnosis.
Figures and show immunohistochemical staining for chromogranin [], synaptophysin [], and neuron-specific enolase NSE [] highlighting neuroendocrine and ganglion cell components. : Immunohistochemical staining for S-100 is positive in spindle/interstitial cells 400×, IHC.
The postoperative period was uneventful. Suture removal was done on 10th day. The patient was instructed to come every month for follow-up and third postoperative month for radiology (magnetic resonance imaging [MRI]). In spite of this, he came only once for check-up, i.e., 1 month postoperative, when he was clinically fine. After this, the patient was lost to follow-up.


A 62-year-old man with a no family history of aneurysms has been admitted to our hospital by screening program. In his life history, he has no hypertension, dislipidemia, and has a smoking habit only. A three-dimensional computed tomography (3DCT) with angiography was performed at our institution revealed a small aneurysm at the proximal end of fenestrated right A1 segment [Figures – and ]. The patient underwent surgical clipping of the aneurysm using pterional craniotomy. During the operation, we have used as an adjunct intraoperative controlling modalities – indocyanine green videoangiography (ICG-VA) and endoscopic visualization to precisely detect the aneurismal anatomy. Surgical exploration revealed the aneurysm just proximal to the right fenestrated A1 segment [] which is also confirmed with ICG-VA and by endoscopy [Figure and ]. The aneurysm was clipped successfully using two clips, size of 6 mm first straight and then another fenestrated clip across the aneurysmal neck []. Postclipping ICG-VA and endoscopic control showed complete obliteration of the aneurysm and preserved normal patency of both two channels of fenestration []. Postoperative period passed without any complications and the patient was discharged home neurologically intact. The patient is doing well for the 2 years of follow-up period. Postoperative follow-up 3DCT was performed 1 month later, which reveals normal position of clip regular position of the vessels [].


A 63-year-old man with a no family history of aneurysms has been admitted to our hospital by screening program. In his history, he has diabetes mellitus, gout and underwent operation on removing mediastinal tumor 12 years ago.
A 3DCT with angiography were performed at our institution revealed a small aneurysm at the proximal end of fenestrated right A1 segment [Figure and ]. The patient underwent operation, with the pterional craniotomy approach. During the operation, we have encountered another small aneurysm on bifurcation point of A1 into two channels. The major aneurysm was projecting laterally, to the right side []. Intraoperative view also confirmed with ICG-VA and by endoscopy [Figure and ] The aneurysms were clipped successfully, size of 4 mm Sujita II curved clip for the minor aneurysm, another Sujita I straight clip size of 6 mm for the bigger one []. Postclipping ICG-VA and endoscopic control confirmed obliteration of the aneurysms, preserving normal patency of both two channels of fenestration []. Postoperative period passed uneventful and the patient was discharged home neurologically intact. The patient is doing well until the last follow-up period. Postoperative follow-up 3DCT was performed 1 month later, which shows adequate lumen of fenestrated A1 [].


A 54-year-old male presented with the complaints of headache for 1 month followed by progressively decreasing speech output and was in altered sensorium at presentation. The tone was increased in the right upper and lower limb with weakness of the right upper and lower limb. He was evaluated with computed tomography (CT) of the brain plain and contrast [Figure and ] which showed left lateral intraventricular lesion with septal attachment with enlargement of the left lateral ventricle. The lesion was hyperdense with Hounsfield units suggestive of blood, and there was no contrast enhancement.
The patient underwent left frontal craniotomy transcortical transventricular approach and gross total resection of the septal lesion followed by an external ventricular drain (EVD) placement. Tumor was firm, reddish with blood clots and had an attachment to the septum. The surrounding tissue was gliotic and had a yellowish discoloration suggestive of remote bleed. Histopathological examination showed features of a neurocytoma with increased tumoral vascularity, large areas of hemorrhage, and early organization of hematoma. The periphery showed aggregates of numerous vessels resembling a vascular malformation. Vessels at the periphery were calcified.
Postoperative CT brain [] showed gross total excision of the lesion with resolving intraventricular bleed and EVD tip in situ. EVD was removed on postoperative day 3. The patient improved postsurgery and was discharged on postoperative day 4. At 2 months, the patient had improved to normal neurological status and on latest follow-up of 3 years, the patient maintained his normal neurological status.


During 2015 a 54-year-old woman underwent kidney transplantation in Spain. She was a white native Spaniard and denied any risk factors for HTLV-1 infection. To date, HTLV testing of blood and tissues in Spain is only recommended for ‘donors that came from or live in highly endemic regions, or have either sex partners or parents from those regions’. However, many transplantation centers do HTLV screening in all donor-recipients when possible. In this case, results of the donor HTLV testing are received after surgery, informing of reactivity for HTLV antibodies using a commercial enzyme immunoassay. No organs other than the two kidneys were transplanted from the same donor.
Positivity for HTLV-1 in the first kidney recipient was confirmed thereafter using immunoblot. Soon after this became known she was informed and antiretroviral therapy with zidovudine, lamivudine plus raltegravir was introduced within the first week following transplantation. Antiretrovirals were used for at least 18 months. In a retrospective interview of the deceased donor’s relatives, it became apparent that he most likely had acquired HTLV-1 from a Brazilian heterosexual partner.
Eight months later the recipient developed difficulty walking, along with unsteady gait, followed by progressive lower extremity weakness, all suggestive of HAM/TSP. Her HTLV-1 proviral load had been high in sequential longitudinal samples collected soon after transplantation, in the range of 320–350 HTLV-1 DNA copies per 10,000 peripheral blood mononuclear cells/ml. At the time of presentation of the initial neurological symptoms, the proviral load in the cerebrospinal fluid was also very high (2340 HTLV-1 DNA copies per 10,000 mononuclear cells/ml).


A 65-year-old male was the second kidney transplant recipient from the same infected donor. Like the other recipient, he was a white native Spaniard who denied any risk factor for HTLV-1 infection. He also became infected with HTLV-1 after transplantation but to date, 3 years later, has not developed any disease. He received antiretroviral drugs, including zidovudine, lamivudine plus raltegravir during the first 2 months following transplantation. It should be highlighted that immunosuppressive therapy (mycophenolate and tacrolimus) dosing had to be reduced from the second month due to hematological toxicity. Finally, rejection of the allograft forced its removal 8 months following transplantation. The patient is no longer undergoing immunosuppressant treatment and is currently undergoing hemodialysis. His proviral load has always been low, in the range of 25 HTLV-1 DNA copies per 10,000 peripheral blood mononuclear cells/ml.


A 62-year-old female presented to our department with a 6-month history of red eye and progressive right-eye ptosis with right-side headache for 2 months. Two years earlier, she underwent right-eye ECCE and ACIOL implantation due to intraoperative zonular dialysis. Postoperatively, the best corrected visual acuity (BCVA) was 20/25. The patient did not report any traumatic history. She had no underlying diseases (eg, diabetes mellitus, allergy, or autoimmune disease). The patient noticed blurred vision and redness in the right eye approximately 6 months before the current presentation. She received treatment twice with intravitreal injections of bevacizumab for unspecified CME in the right eye at a local clinic, without the improvement of symptoms.
At our clinic, the ophthalmic examination revealed a BCVA of 20/630 and 20/20 in the right and left eye, respectively. The intraocular pressure was 8 and 15 mm Hg, respectively. The slit lamp examination showed right-side diffuse limbal neovascularization and serious bulbar conjunctiva injection. The pupil was oval and distorted to the upper side. A peripheral iridectomy was detected at the 6 o’clock position, and a vertically aligned angle-supported Kelman Multiflex ACIOL was found in the anterior chamber. The ACIOL had deviated upward, with upper haptic protruding from the previous ECCE scleral tunnel wound. The upper haptic was exposed without coverage of the intact conjunctiva (Fig. ). We performed a Seidel test, which was positive. Fundus examination and optical coherence tomography (OCT) revealed CME with mild epiretinal membrane (ERM) (Fig. ). The white-to-white distance was 13.4 mm. The examination of the left eye was unremarkable.
Considering the risk of endophthalmitis, the patient agreed to undergo extraction of the ACIOL. During operation, the ACIOL was easily extracted by loosening the fragile tissues surrounding the perforated wound after conjunctival peritomy. The sclera and conjunctiva wounds were erosive and dehiscent. Afterwards, the perforated wounds were sutured. The upper part of the intact conjunctiva was advanced toward the limbus to cover and protect the sutured scleral wound. Subsequently, we performed pars plana vitrectomy, ERM peeling, and scleral-fixated posterior chamber IOL (PCIOL) implantation using an Alcon CZ70BD (Alcon, Fort Worth) poly methyl methacrylate lens.
Three months after surgery, the BCVA in the right eye improved to 20/80 and the right-side headache and hyperemia of the conjunctiva had significantly subsided. Moreover, the cornea and anterior chamber were clear. The iris was oval-shaped and shifted upwardly. The scleral-fixated PCIOL remained centrally located (Fig. ). Fundus examination and repeated OCT revealed improved CME with decreased central retinal thickness (Fig. ).
Written informed consent was provided by the patient for the publication of this case report.


A 41-year-old male farmer presented with a diffuse swelling in the right foot of 1-year duration. He also had multiple cystic swellings of varying sizes in the right ankle, left leg, left foot, and right forearm for 6 months. There was a history of discharge of whitish granular material from the swelling in the right foot on and off. There was no history of trauma before the onset of swelling. The patient was a known diabetic with poor glycemic control. He was having frequent attacks of joint pain and back pain with swelling involving multiple joints for last 7-year duration and was prescribed systemic steroid for joint pain by the family physician. After that, the patient started taking systemic steroid on his own whenever he gets joint pain.
On examination, there was a diffuse swelling in the right foot with multiple small cystic lesions and discharging sinuses. There was discharge of seropurulent material []. He also had cystic swellings of varying sizes in the right ankle, left leg, and right forearm [Figure and ]. Our patient also showed effusion and stiffness in the joint whenever he had severe joint pain. The differential diagnosis of mycetoma and phaeohyphomycosis with dissemination were considered. Rheumatoid arthritis and reactive arthritis were suspected as the etiology for joint pain.
The patient was investigated for the cystic swellings and joint pain. Hematological investigations were within normal limits except elevated blood sugar values. ELISA for HIV infection was negative and his chest X-ray was normal. X-ray of the right foot, ankle, and other joints did not show any bony involvement. Fine-needle aspiration was done from the cystic lesions, with the aspirate being purulent material [], and the cytology smear showed enormous cylindrical fungal hyphae []. In the KOH mount of the aspirate as well as in the discharge from the foot lesion, branching, segmented hyaline hyphae were seen []. Fungal culture in Sabouraud's dextrose peptone agar showed whitish colonies initially, which later became grayish white and black []. On the reverse, the colonies were brownish black and grayish white in color [].
In lactophenol cotton blue mount of the fungal culture, septate, cylindrical hyphae branching at acute angle with conidiophores bearing oval conidia were identified [Figure and ]. The cultural characteristics of the fungi were consistent with S. apiospermum. Skin biopsy from the lesion also revealed fungal hyphae in the necrotic inflammatory background, and there were no grains [Figure and ]. He was found to be positive for rheumatoid factor and anticyclic citrullinated peptide with elevated C-reactive protein and was diagnosed to have rheumatoid arthritis along with fungal infection. Based on the clinical findings and positive investigations, the diagnosis of disseminated scedosporiosis was made, and the patient was started on voriconazole 200 mg twice daily. He was referred for surgical excision of the lesions and was lost to follow-up.


A 74 years old man, was admitted to the emergency department for syncopal episode, with detection of hypoglycaemia resistant to medical treatment.
His past medical history included hypertension, benign prostatic hypertrophy and glaucoma.
Physical examination revealed a morbidly obese (body mass index = 35), painless hepatomegaly without weight loss.
Investigations reported a marked hypoglycaemia, with capillary blood glucose measurements ranged from 30 to 70 mg/dL treated with continuous glucose infusion and a rich carbohydrate diet.
Laboratory tests revealed low levels of insulin: 0.3 mcrU/mL (range 5-25 mcrU/mL), low levels of C-peptide: < 0.10 ng/dL (range 0.9-4 ng/dL), low levels of GH: <0.02 ng/dL (range 0.02-1.23 ng/dL), normal level of cortisol: 8.9 mcg/dL (range 3.7-19.4 mcg/dL), normal level of insulin-like growth factor I (IGFI): 84 ng/mL (range 20-300 ng/mL) and normal level of insulin-like growth factor II (IGFII): 575.70 ng/mL (range 373-1000 ng/mL) in the patient’s serum. The IGFII/IGFI ratios was 7:1. Liver functions, viral panel, tumor markers (alpha-fetoprotein, carcinoembryonic antigen and cancer antigen 19-9) were within the normal limits.
A total body computed tomography (CT) was performed and revealed a solid mass measuring 15 cm of the left liver. The intravenous contrast demonstrate an inhomogeneous enhancement for necrosis within the tumour as well as a thick capsule that enhances during the portal phase (Figure ). Radiological findings on abdominal magnetic resonance (MR) were similar to ones described for abdominal TC with evidence of a voluminous liver mass measuring 15 cm × 13 cm (Figure ). There was no evidence of metastatic disease


A 7-year-old boy presented with breathlessness and recurrent respiratory tract infection for 3 months. His physical examination revealed tachycardia (heart rate – 122 beats per min), tachypnea (respiratory rate – 36 breaths per min), and Grade II/VI early systolic murmur at the left upper sternal border. Echo revealed patent ductus arteriosus (PDA), with a left-to-right shunt. Coarctation of the aorta was also suspected on echocardiography, and therefore, cardiac computed tomographic (CT) angiography was planned. MDCT angiogram showed ARCAPA with IAA type A and PDA [], dominant left coronary system with single left coronary artery arising from the left anterior coronary sinus []. The pulmonary artery was markedly dilated and connected to descending aorta via large PDA, giving the appearance of a low aortic arch []. The patient was referred to a higher center for cardiothoracic surgery evaluation and unfortunately lost to follow-up.


In 2016, a 79-year-old woman was diagnosed with stage IIIB MCC, and underwent surgery and radiation therapy at the Department of Plastic Surgery at Hokkaido University Hospital in Sapporo, Japan. Two years after the first treatment, she was found to have retroperitoneal metastasis, and ICI monotherapy with avelumab was started in February 2018. Before avelumab, her plasma glucose and hemoglobin A1c levels were 110 mg/dL and 6.1%, respectively. She received avelumab monotherapy (523 mg every 2 weeks) for 5 months (10 times in total) without any symptoms or any laboratory findings of hyperglycemia.
At a regular visit in July 2018, elevated plasma glucose level (483 mg/dL) and hemoglobin A1c level (7.5%) were detected without any hyperglycemia symptoms or any signs of antecedent infection. The patient was referred and admitted to the Internal Medicine II, Hokkaido University Hospital on the same day of the visit. Her height, bodyweight and body mass index were 147 cm, 48.7 kg and 22.5 kg/m2, respectively. Venous blood gas analysis showed no metabolic acidosis, but blood ketone bodies were increased (Table ). A glucagon loading test, carried out 1 week after the admission, and 24-h urinary C-peptide immunoreactivity showed severe insulin deficiency. Neither antiglutamic acid decarboxylase antibodies nor anti-islet antigen 2 antibodies were positive (Table ). No metastasis signature in the pancreas was detected with computed tomography. Based on her clinical course and laboratory findings, we diagnosed the patient with fulminant type 1 diabetes mellitus induced by avelumab. Human leukocyte antigen (HLA) analysis showed she had DRB1 *09:01:02, DRB1 *14:54:01, DQA1 *01:04, DQA1 *03:02, DQB1 *05:02:01 and DQB1 *03:03:02, being partially concordant with type 1 diabetes susceptible haplotype. Post-avelumab, positron emission tomography–computed tomography showed the disappearance of the retroperitoneal metastasis. Although avelumab administration was suspended because of the ICI-induced fulminant type 1 diabetes mellitus, it will be resumed with intensive insulin therapy if any sign of MCC recurrence appears. The patient's hemoglobin A1c levels were between 7.4 and 8.2% after discharge with intensive insulin therapy.
We obtained informed consent and received a consent form from this patient.


A 15-year-old boy presented to the emergency department with acute bilious vomiting and epigastric pain. The patient appeared to suffer from considerable pain which occurred while participating in a basketball game, and gradually worsened after taking a meal and more than 2 L of water during the sports game. The patient reported that he received several forceful blows to his abdomen from other players. In addition, for the last one and a half years, the patient had early satiety and postprandial epigastric pain which was relieved when he was in a knee-chest position. One year prior to this admission, the patient had been brought to a different hospital for similar abdominal symptoms that had resolved without treatment within several hours.
His initial vital signs were normal. The patient had a strong, muscular and athletic build, a height of 185 cm and a weight of 66.0 kg. He reported a growth in height of 9 cm and no weight change for the past year. Palpation of his abdomen revealed generalised tenderness, but no guarding, and no abdominal masses were detected. Blood tests, including blood count (haemoglobin 154 g/L, white cell count 7.60×109/L and platelet count 22.9×109/L), chemistries (sodium 142 mmol/L, potassium 4.6 mmol/L, chloride 105 mmol/L, blood urea nitrogen 6.57 mmol/L and serum creatinine 59.3 μmol/L), liver function tests (albumin 56 g/L, bilirubin 21.2 μmol/L, aspartate aminotransferase 28 IU/L and alanine aminotransferase 18 IU/L) and inflammatory marker (C reactive protein 0.1 mg/L), were normal, with the exception of a slight elevation of amylase (163 U/L). The urinalysis was within normal limits. The abdominal ultrasound (US) showed no abnormalities other than a dilated stomach.
Despite the patient’s abdominal findings, his severe abdominal pain and history of forceful blows to the abdomen suggested the possibility of abdominal injuries, including intestinal perforation and abdominal haemorrhage. As a result, a contrasted CT of the abdomen and pelvis was performed. This CT was initially interpreted by a doctor on night duty as normal apart from a massively distended stomach (). After the CT scan, the patient lay in prone and knee-chest position for about an hour and then his symptoms resolved spontaneously. Therefore, gastric decompression with a nasogastric tube was not performed. The patient only underwent observation with intravenous hydration. No vomiting was found during the observation period. The next morning, the patient made a swift recovery. He had no abdominal symptoms, including postprandial pain. The follow-up abdominal US showed the volume of the stomach significantly decreased as normal and no other abnormalities were found. These suggested that there was a transient intestinal obstruction that was thought not to require emergency interventions. The patient was discharged, and a follow-up visit was arranged.
After his discharge, the CT review by an experienced radiologist revealed, however, a dilated proximal duodenum and an abrupt calibre change in the third portion of the duodenum (). The aortomesenteric angle and distance were 14° (normal range: 38°–65°) and 4 mm (normal range: 10–28 mm), respectively (). In addition, the abdominal fat was significantly low (). Furthermore, the third part of the duodenum passed anterior to the aorta in the retroperitoneal space. The position of the SMA and superior mesenteric vein was normal (). These suggested that there was no intestinal malrotation or volvulus in this patient. Based on these radiological findings and his clinical history, the patient was diagnosed with SMA syndrome.


Patient 1 was a 16-year-old male high school student who experienced three seizures that always occurred in the afternoon at school because of a poor night of sleep before intervention. At his second seizure, he was diagnosed with focal cortical dysplasia-related epilepsy on the basis of 24-hour, abnormal, dynamic electroencephalography (EEG) () and cranial magnetic resonance imaging (MRI) (). After the patient and his family provided signed informed consent, 0.5 Hz rTMS was applied over the epileptic focus, which was approximately localized 7.5 cm vertically up the outer ear channel based on his MRI, with a 50% resting motor threshold. The patient could not tolerate more than 50% intensity of stimulation at the site. A commercially available magnetic stimulator with a 70-mm figure-eight coil (CCY-IV; YIRUIDE Co. Ltd., Wuhan, China) () and low-frequency rTMS procedure was used for both patients with the following parameters: 0.5 Hz, 10 s of stimulation, 1-s interval, and a total of 1200 pulses for each day (from Monday to Saturday). During treatment, the patient experienced one seizure because of pressure from his final exam (). However, his classmates reported that his convulsion was mild and the episode lasted for a much shorter time than observed previously. The patient received 10 treatment courses (2 weeks each) and a subsequent 16-week follow-up. Treatment was performed for 36 weeks. No obvious adverse events were observed.


Patient 2 was a 21-year-old female university student who had a history of febrile convulsions at the age of 2 years. Before rTMS, she had four seizures that always occurred between 12:00 AM and 1:00 AM with similar symptoms. Unfortunately, an enhanced brain MRI and several 24-hour dynamic EEGs showed no obvious abnormalities. Therefore, she was initially diagnosed with sleep-related epilepsy. After she provided signed informed consent, rTMS treatment was performed (). In the absence of a defined epileptic focus, bilateral hand M1 regions were chosen as the stimulus sites because they are representative of large cortical motor areas and are the common motor output pathways. rTMS at 0.5 Hz was delivered at 80% resting motor threshold at 600 pulses for each site. During the interval as indicated in , the patient experienced a seizure at approximately midnight following a meal with a large amount of crab meat and spicy food at home. Observers indicated that she only gasped for breath with mild limb convulsion for a shorter time than observed previously. The patient received the same treatment schedule as did patient 2. During follow-up, no seizures or major adverse events occurred.


We present a case of a 25-year-old male who presented with complaint of clawing of his left great toe. The patient had sustained a left-sided distal tibia fracture treated with open fixation of the tibia fracture 3 years ago. The implant was removed 18 months after the procedure due to the patient’s preference. After 6 months, the patient initially noticed clawing of his left great toe when he climbed stairs barefoot. He also complained that he had started to find it difficult to sit cross-legged, squat, as well as ride his bicycle due to the tightness he felt in his toes with certain ankle movements on examination, the patient walked with a normal bipedal unassisted gait. He had well-healed surgical scars and a plantigrade foot. Active dorsiflexion of the ankle joint produced an exaggerated flexion of the great toe. With the ankle held in dorsiflexion, it was not possible for the patient to actively or passively fully extend any of the toes (Figs. and ). The diagnosis of checkrein deformity of the left great toe was made and attributed to the previous fracture of the distal tibia resulting in scarring or ischemic injury to the toe flexors. The possible patterns of injury could have been an isolated injury to FHL muscle or tendon causing fixed flexion of the great toe. The patient elected for surgical intervention and surgical exploration of FHL tendon at the level of the midfoot was planned. Exploration at the midfoot would give easier access to both tendons free from scar tissue making correction easier. The patient was suitably consented and the exploration was performed under spinal anesthetic with an inflated tourniquet at 250 mmHg. The tendon of FHL was identified (). Subsequent z-plasty of FHL tendon gave a full correction of the great toe flexion contracture (). The patient made an uncomplicated post-operative recovery and after final review, 3 months post-surgery had maintained the full range of movement in all toes (). Weight-bearing photograph is also shown before surgery () and after surgery ().


We present a 20-year-old female who presented with sudden onset epigastric abdominal pain associated with intractable nausea and non-bloody vomiting that began 5 days ago. The pain was sharp in character and radiated to the back. Upon presentation, vitals were stable and physical exam was remarkable for severe epigastric tenderness. Labs were notable for an elevated lipase of 1295, AST 27 and ALT 56. Triglycerides, IgG4, PTH and ionized calcium were within normal limits. The patient did eventually admit to marijuana use after a urine drug screen was obtained and noted to be positive for tetrahydrocannabinol (THC). She admitted to smoking marijuana daily for the past 14 months. Abdominal ultrasound was obtained and demonstrated a non dilated common bile duct. MRI/MRCP demonstrated post-cholecystectomy changes, no choledocholithiasis, and mild peripancreatic stranding at the head of the pancreas. Of note, the patient was hospitalized with a similar presentation 2 months ago at which time she underwent a laparoscopic cholecystectomy. She also underwent an endoscopy 3 days prior to admission which demonstrated mild gastritis that was H. pylori negative.


A 67-year-old male patient, previously diagnosed with hypertension, diabetes mellitus, dyslipidemia and gout, was brought to the hospital with the history of acute interscapular pain followed by mental confusion and syncope. He denied dysphagia, or any respiratory symptoms, but lately noticed frequent choking. He was a heavy smoker until the age of 60. He had never followed a regular treatment, and was hospitalized 16 years ago for a hypertensive crisis when an acute myocardial infarction was suspected. Recently, he was taking losartan, amlodipine, simvastatin, and gliclazide. The initial physical examination revealed an agitated patient, unconscious, with isochoric pupils, and normal and symmetric muscular strength. He was afebrile, hydrated but pale. His blood pressure was 230/146 mmHg (symmetrical in both upper limbs), pulse rate was 124 beats per minute and regular, respiratory rate was 20 respiratory movements per minute, room air oximetry was 88%, capillary blood glucose was 194 mg/dL. The remaining physical examination was unremarkable.
With the diagnosis of hypertensive encephalopathy, the patient was treated with sodium nitroprusside, esmolol, morphine, and oxygen supplementation by Venturi mask. Gradual improvement of the level of consciousness and psychomotor agitation was observed. The initial laboratory tests are shown in .
The electrocardiogram showed sinus tachycardia with atrial extrasystoles and left ventricular hypertrophy. The chest radiography showed cardiomegaly with mediastinal enlargement (). A brain CT scan was normal.
Due to the persistence of thoracic pain (which was also referred in the anterior area of the chest) and the image of mediastinal enlargement, the patient was submitted to a thoracic angiotomography that revealed a tortuous aorta with diffuse atheromatosis characterized by parietal thickening and calcified plaques. Dilatation of the aortic arch was evident, with a maximum diameter of 5.3 cm. The presence of a spontaneous hyper density was observed along the upper posterior wall, displacing the intima and intimal calcification, which suggested intramural hematoma ().
An aberrant right subclavian artery emerging from the posterior portion of the aortic arch passing behind and displacing the esophagus anteriorly and to the right is shown in , presenting in its proximal segment a dilatation with 4.1 cm diameter characterizing a diverticulum aneurysm of Kommerell (, and ).
The transesophageal echocardiography, performed at the bedside under sedation and topical anesthesia, revealed preserved ventricular systolic function, no segmental dysfunction of ventricular walls mobility, and thickening of the aortic and mitral valve rings with mild to moderate insufficiency. Aortic diameters were measured: aortic valve sinuses 41 mm, tubular sinus junction 39 mm, ascending aorta 53 mm, and aortic arch 49 mm.
The descending aorta showed a marked intimal thickening extending to the transition of the aortic arch, probably corresponding to a hematoma or thrombus. A mobile filament image in the descending aorta, suggestive of thrombus, was also evident.
The adopted treatment was conservative once the intramural thrombus remained steady and because of the unfeasibility for percutaneous treatment by the presence of the dilated ascendant aorta associated with the aberrant right subclavian artery.
The patient was discharged to outpatient follow up after 16 days of hospitalization.


A 72-year-old man was referred to our hospital for his bilateral hydronephrosis. His serum PSA level was 178 ng/mL, and bone scintigraphy showed multiple bone metastases. A prostate needle biopsy revealed a Gleason Score of 4 + 4 = 8. NSE, LDH, and ALP levels were also elevated. In July 2018, combined androgen blockade including leuprorelin and bicalutamide was introduced. Despite some decrease in the PSA level, the PSA nadir was not very low and tended to elevate again, so docetaxel was introduced for six courses. Despite docetaxel treatment, however, the PSA and NSE levels were not markedly decreased, so enzalutamide was introduced. After six weeks of enzalutamide installation, the patient showed Common Terminology Criteria for Adverse Events (CTCAE) grade 1 dysgeusia, which later increased to grade 2, so we changed the medicine intake time from the morning to the night just before sleep without dose reduction. Four weeks after changing the timing, his dysgeusia had improved (Fig. ).


A 63-year-old man was referred to our hospital for a further examination of his elevated PSA level (919 ng/mL). A prostate needle biopsy revealed a Gleason Score of 5 + 5 = 9. Bone scintigraphy and computed tomography (CT) revealed multiple bone metastases and axillary lymph node metastasis. In November 2017, he started combined androgen blockade including leuproreline acetate and bicalutamide (80 mg/body). His serum PSA level had decreased to 1.16 in March 2018 but gradually increased afterward, so leuprprelin was changed to degarelix, and bicalutamide was stopped. Bone scintigraphy revealed residual bone metastatic lesions, so combination therapy with Ra-223 and enzalutamide (160 mg/body) was introduced. His serum PSA level gradually decreased, but CTCAE grade 2 dysgeusia developed in March 2019. We speculated that the dysgeusia had been induced by enzalutamide. Because his dysgeusia was persistent and enzalutamide showed efficacy in reducing his PSA level, the timing of enzalutamide intake was changed from the morning to the night just before sleep without dose reduction. One month after changing the timing, his dysgeusia had improved (Fig. ).


A 57 year old male with a past medical history of hypertension presented with 3 months of worsening right sided nasal congestion and facial swelling. A few days prior to presentation he developed right eye lacrimation and blurry vision. A maxillofacial CT scan revealed a 4.5 × 2.8 cm lobulated, rim enhancing hyperdense mass centered in the right nasal cavity with extension into the right choana and maxillary sinus with mass effect causing leftward bowing of the nasal septum. He was promptly evaluated by otolaryngology for biopsy.
Microscopic examination revealed respiratory mucosa with underlying proliferation of large lymphoid cells with centroblastic morphology. The cells exhibited high nuclear:cytoplasmic ratio, vesicular nuclei, one to three peripherally placed nucleoli, and frequent mitoses (Fig. ).
IHC stains showed large lymphoid cells to be positive for CD20, PAX-5, CD5 (weak), CD10, BCL2 (strong and diffuse), BCL6, MUM-1 and C-MYC, while negative for CD30, ALK-1, HHV8, EBV (ISH-EBER), and BCL1. CD3 and CD5 stains were positive in the scattered reactive T-cells. Ki-67 proliferation index >95%.
FISH analysis did not reveal a MYC or BCL-2 translocation; it did reveal an abnormal MYC-IGH and BCL2-IGH hybridization pattern in 96% and 97% of nuclei, respectively. FISH was also negative for rearrangements of MYC, BCL2, and BCL6. There were 3–4 copies of MYC in 71% of nuclei. BCL2 showed amplification in 86% of nuclei, and 3–8 copies of BCL6 were seen in 81% of nuclei. These findings were consistent with DLBCL-NOS. Given the high expression of MYC and BCL2, the tumor was further classified as a DEL.
The overall morphologic, immunophenotypic and molecular characteristics of the tumor cells were consistent with DLBCL, not otherwise specified (NOS), and favored a germinal center B-cell subtype. The high proliferation index of Ki-67 (Fig. ) with coexpression of C-MYC (Fig. ) and BCL2 (Fig. ) on the tumor cells (DEL) suggested a resistant lymphoma.
Initial positron emission tomography-computed tomography (PET-CT) scan revealed marked diffuse FDG activity involving the right nasal cavity extending into the adjacent right frontal sinuses with SUV of 28.7 (Fig. ). PET-CT also revealed focal FDG activity in the right submandibular gland with SUV of 5.4. CT scan of the head, chest, abdomen, and pelvis was negative for any metastasis. A bone marrow biopsy did not reveal evidence of lymphoma on IHC or flow cytometry. Cerebrospinal Fluid (CSF) analysis did not reveal the presence of abnormal cells. The findings of additional lymph node involvement were consistent with DEL, stage IIB.
The patient underwent 4 alternating cycles of R-CODOX-M/R-IVAC, as follows:
R-CODOX-M (cycle 1 and 3):
Rituximab 375 mg/m2 on day 1
Cyclophosphamide 800 mg/m2 day 1–4
Doxorubicin 40 mg/m2 day 1
Vincristine 1 mg/m2 IV day 1–2
Methotrexate 300 mg/m2 IV mg on day 8 with leucovorin rescue
Granulocyte colony-stimulating factor beginning 24 h after completion of IV chemotherapy and continuing until absolute neutrophil count >1,000/microL
R-IVAC (cycle 2 and 4):
Rituximab 375 mg/m2 IV on day 1
Ifosfamide 1,500 mg/m2 IV day 1–5 with mesna
Etoposide 60 mg/m2 IV day 1–5
Cytarabine 2 g/m2 IV on days 1 and 2 (total of four doses)
Intrathecal Methotrexate 12 mg on day 5
Granulocyte colony-stimulating factor beginning 24 h after completion of IV chemotherapy and continuing until absolute neutrophil count >1,000/microL
After the first cycle of R-IVAC, the patient experienced febrile neutropenia, grade 3, requiring readmission to the hospital and IV antibiotics. During the same admission the patient was found to have anemia, grade 3, and thrombocytopenia, grade 3, requiring transfusion of packed red blood cells and platelets. During cycle 2 of R-CODOX-M the patient developed acute kidney injury that improved with IV fluids; his renal function returned to baseline by cycle 2 of IVAC and he did not experience additional acute kidney injury. After cycle 2 of R-CODOX-M the patient also developed proctocolitis, grade 2, requiring treatment with antibiotics, as well as transaminitis with AST peaking at 200, ALT at 451, and ALP at 450, with all enzymes gradually returning to normal values. From the time of diagnosis through completion of chemotherapy, the patient experienced a 40 lb weight loss.
After completion of chemotherapy course, the patient had repeat PET-CT imaging which revealed SUV activity in the original right nasal cavity mass of 6.1 (Fig. ), with resolution of SUV activity in the right submandibular gland. Given the persistent metabolic activity of the nasal cavity mass he was treated with radiotherapy consolidation with 36 Grey. PET/CT after radiation showed no residual uptake in the right nasal cavity (Fig. ), indicating a complete response.


A 70-year-old woman presented to the emergency room with symptoms of total aphasia and right unilateral spatial neglect. She had a history of bronchial asthma, dyslipidemia, and type 2 diabetes. Dyslipidemia and type 2 diabetes were noted from the age of 62. The onset of symptoms was 46 min prior to presentation to the emergency room. Her neurological findings confirmed mixed aphasia (she could not obey simple commands), neglect of the right half of her body, and left conjugated deviation. No obvious paralysis or sensory disturbances were noted. Deep tendon reflex was normal, and no pathological reflex was observed. Her NIH Stroke Scale (NIHSS) was 7. Her baseline MRI revealed no obvious high signal in DWI (Fig. ). Fluid-attenuated inversion recovery (FLAIR) confirmed FLAIR vessel hyperintensities in the left middle cerebral artery (MCA) (Fig. ). Magnetic resonance angiography (MRA) showed partial obstruction in the distal part of the left MCA (Fig. ), which may have been an artifact. To make a definitive diagnosis, perfusion-weighted imaging (PWI) was additionally performed to assess the tissue at risk. Our automated software showed 12 mL of Tmax >6 s of hypoperfusion in the left MCA region, which matched the lesion side. Because the ischemic core calculated by an apparent diffusion coefficient (ADC) <620/10−3 mm2/s was not present, this case was diagnosed as DWI-negative DWI-PWI-mismatched AIS (Fig. ). Carotid vascular ultrasound showed no stenosis in the bilateral common carotid artery or internal carotid artery. We diagnosed her as AIS, and intravenous rt-PA therapy was administered 22 min after entering the MRI suite. She responded well to the reperfusion therapy, and her NIHSS was 0 after leaving the MRI suite. No further deterioration of neurological signs was observed during her admission. A FLAIR scan on the 9th day showed an ischemic change in the same lesion as the perfusion abnormality. This area was also identical to the obstructed left MCA seen on MRA. Transthoracic echocardiography did not reveal any apparent cardiac dysfunction or intracardiac thrombus. Nonvalvular atrial fibrillation was observed on day 2 of admission, and we diagnosed her stroke subtype as a cardiogenic cerebral embolism. Direct oral anticoagulant (dabigatran 300 mg/day) was prescribed at the time she was discharged to her home.


An 88-year-old man visited the emergency room with complaints of minor unilateral spatial neglect. He had a history of hypertension and type 2 diabetes mellitus from the age of 87. The onset of symptoms was 70 min prior to presentation to the emergency room. His neurological findings confirmed minor unilateral spatial neglect, right conjugate deviation of the eyes, and dysarthria. No obvious paralysis or sensory disturbances were found. Deep tendon reflex was normal, and no pathological reflex was observed. His NIHSS was 4. His baseline MRI revealed no obvious high signal in DWI (Fig. ). FLAIR confirmed FLAIR vessel hyperintensities in the right MCA (Fig. ). MRA showed partial obstruction in the distal part of the right MCA. PWI was additionally performed and showed 60 mL of Tmax >6 s of hypoperfusion in the right MCA region, which matched the lesion side. Because the ischemic core was not obvious, this case was also diagnosed as DWI-negative DWI-PWI-mismatched AIS (Fig. ). Carotid vascular ultrasound showed no stenosis in the bilateral common carotid artery or internal carotid artery. He was diagnosed as AIS, and intravenous rt-PA therapy was administered 20 min after his arrival (90 min after onset). He responded well to the reperfusion therapy, and his NIHSS was 0 after 24 h. No further deterioration of neurological signs was observed during his admission. Transthoracic echocardiography did not reveal any apparent cardiac dysfunction or intracardiac thrombus. CT angiography (CTA) showed 75% stenosis in the right M1 branch (Fig. ), and his stroke subtype was diagnosed as an atherothrombotic brain infarction. Oral antiplatelet therapy (clopidogrel 75 mg/day) was prescribed at discharge.


A 44-year-old woman (height: 5′4′′ [1.6 m]; weight: 185 lbs [83.9 kg]; body mass index: 31.75) presented to the Emergency Department with complaints of severe low back pain and bilateral lower extremity pain and weakness. She stated that the symptoms initiated one day earlier with muscle cramping of the bilateral posterior thighs and had gradually progressed to an inability to walk or lift her legs. The patient initially attributed her symptoms to dehydration as she had worked several 12-h shifts in a row as a registered nurse. She reported some urinary hesitancy although denied urinary or bowel incontinence, any sensory deficits of the lower extremities, or severe headaches. The patient mentioned several stressors, including marriage in the next week to a man to whom she had been previously married, working overtime, minimal sleeping at night, and long commute to work. Past medical history was significant for anxiety, depression, and migraines. The patient denied a history of sexually transmitted diseases. Two years earlier, the patient was hospitalized for 24 h following complaints of right hemiparesis and chest pain. She was diagnosed with conversion disorder due to a psychological stressor.
The physical examination revealed 1/5 power in the lower extremities bilaterally, with distal strength greater than proximal. Sensation, deep tendon reflexes, and muscle tone of the lower extremities were normal. There was an absence of atrophy or hypertrophy of the muscles, rigidity or spasticity, and tremors or abnormal movements. Serial cervical, thoracic, and lumbar MRI scans with and without gadolinium contrast demonstrated no significant stenosis, neural compression, or other etiologies of her symptoms. The brain MRI with and without gadolinium contrast was normal. The initial impression was conversion disorder due to the patient's multiple work and familial stressors. She was hospitalized and treated with methylprednisolone and hydromorphone.
A lumbar puncture and a CSF pathogen panel were performed, the latter utilizing a multiplex nested PCR followed by a melting analysis to detect nucleic acid sequences. The sensitivity was greater than 95%, and the specificity was greater than 99.6%. The panel detected HSV-2 and was negative for crypococcal antigen. The following findings were also observed in the CSF: glucose 90 mg/dL (40–70 mg/dL), protein 55 mg/dL (12–60 mg/dL), WBC 434/μL (0–5/μL), RBC 9/μL (0–2/μL), lymphocytes 94% (40–80%), and monocytes 6% (15–45%). The culture CSF/Gram stain revealed rare WBCs and no organisms, and the HIV-1 and -2 antibody/antigen combination was nonreactive. Borrelia burgdorferi antibodies (Lyme disease), syphilis total antibodies, hepatitis B core antibodies and surface antigens, Chlamydia trachomatis, and Neisseria gonorrhoeae were all negative. The patient was diagnosed with radiculomyelitis due to HSV-2 marked by back pain and weakness of the lower extremities with evidence of lymphocytic pleocytosis. She initiated treatment with acyclovir 650 mg infused every 8 h for 3 weeks and was subsequently prescribed valacyclovir.
The patient had attained no improvement of her lower extremity pain when evaluated by a neurologist 5 and 8 months following the start of her symptoms. Additionally, CSF findings demonstrated a WBC count 6/μL, protein 33 mg/dL, and glucose 57 mg/dL 5 months after her initial lumbar puncture, respectively. There were greater than 4 oligoclonal bands detected in the CSF with no corresponding bands detected in the serum, and the IgG index was 0.9 [0.3–0.7]. These positive findings indicated an inflammatory process. The aquaporin 4 (AQP4) receptor antibody was negative. The patient was evaluated by a neurologist 17 months after her symptoms initiated, at which time she had significant improvement in the range of motion strength in her lower extremities. Her minimal residual pain was controlled with gabapentin, baclofen, and alprazolam.