A 73-year-old white male was seen for blurred vision attributed to neovascular age-related maculopathy that did not respond to intravitreal bevacizumab and triamcinolone. He reported good vision from birth and began wearing +7 diopter hyperopic correction lenses as a young adult. He had no past ocular trauma, inflammation, or known ocular disease other than narrow angles, for which he had remotely received laser iridotomy. He had medically controlled hypertension and hyperlipidemia. He denied headache or niacin use, and otherwise had a negative review of systems. Several months earlier, he had developed blurred vision attributed to macular edema () and cataract. He underwent uneventful cataract surgery in both eyes; however, his blurred vision persisted in the right eye, and the macular edema increased bilaterally. Treatment with intravitreal bevacizumab twice and with intravitreal triamcinolone did not improve the condition.
At the time of the initial examination with one of the authors (SEP), the patient’s visual acuity was 20/32 OD (oculus dexter, right eye) and 20/20 OS (oculus sinister, left eye). OD, there was mild distortion on the Amsler grid. The intraocular pressure was 12 mmHg OD and 15 mmHg OS. The anterior segments were normal; they had patent peripheral iridotomies and horizontal corneal diameters of 12 mm. The posterior chamber lens implants were in proper position, and there was no anterior segment inflammation. In the Schlemm’s canal, there was no blood that could be seen by gonioscopy. The vitreous was free of cells. The discs were free of pit, tilt, and coloboma (). No peripapillary choroidal excavation was present. Macular edema extended from the disc into the macula and was greater in the right eye than in the left. No dome maculopathy was present. None of the following conditions were detected by ophthalmoscopy or B-scan echography: peripheral retinoschisis; retinal detachment; leopard-spot pigmentation; choroidal detachment; or posterior scleritis. Seen by the use of B-scan echography, the choroidal–scleral thickness was consistent with hyperopia. The axial lengths were 22.3 mm OD and 22.42 mm OS. Fluorescein angiography showed leakage from neither the retinal vascular system nor the retinal pigment epithelium (RPE), although there was staining of the temporal peripapillary crescent (). Indocyanine green angiography revealed late hypercyanescence in the posterior pole; this suggests late hyperpermeability. There was hypofluorescence of the temporal peripapillary crescents (). Enhanced-depth imaging optical coherence tomography (OCT) for both maculae demonstrated thick choroids (496 μm OD and 537 μm OS) and outer retinal edema extending from the disc into the macula with subretinal fluid OD ( and ). In each eye, the thickness of the choroid was greatest posteriorly. There was no evidence of shifting fluid. A neurological examination revealed an isolated left fourth cranial nerve palsy, but all of other examined factors, including cerebrospinal fluid pressure and composition, were normal.
Attempting to avoid surgical intervention and frustrated with the lack of response to intravitreal triamcinolone and bevacizumab, the patient was started on a trial of acetazolamide, 500 mg extended-release sequels orally twice a day; subretinal fluid and edema were promptly resolved in both eyes (). Initial improvement in effusion was seen after 1 week on acetazolamide; central retinal thickness, seen by the use of OCT, decreased from 768/301 (OD/OS) to 718/296. Four weeks later, central retinal thickness was 233/278 with minimal subretinal fluid OD and no edema oculus uterque (OU). After another 4 weeks, there was neither residual fluid nor edema in either eye. The visual acuity improved from 20/40 OD and 20/25 OS prior to treatment to 20/20 OU after treatment. However, the patient developed malaise and confusion, and he requested that the medication be discontinued. After 9 weeks of treatment, acetazolamide was tapered to 125 mg daily. His vision was unchanged, but macular edema began to recur at the temporal crescent of the disc OD. Therefore, a course of 2% dorzolamide hydrochloride ophthalmic solution was started OU three times a day; this resulted in a reduction of retinal edema within 1 month and no change in vision. At this point, the acetazolamide was stopped. Over the next 5 months on dorzolamide monotherapy, a small area of cystic edema at the temporal crescent remained unchanged, and the patient’s vision remained stable. At this point, he requested that the dorzolamide be stopped. Within a month, the edema recurred and threatened the macula of the right eye greater than the left (). This change prompted restarting topical dorzolamide; the edema promptly retreated to the temporal crescent of the disc. The choroid remained thickened without definitive change throughout the course of treatment and follow up, which exceeded 1 year.


A 19-year-old male reported with a complaint of restricted tongue movement and difficulty in speech. Clinical examination showed the presence of Kotlow's Class II ankyloglossia [].


A 21-year-old man reported with a complaint of difficulty in speech and deglutition. History of speech therapy 6 months back with no benefit from it. He also had difficulty in licking, sucking or whistling. The ear, nose, throat and other physical examination was normal. Intra oral examination revealed Kotlow's Class IV ankyloglossia []. Any attempt to protrude the tongue resulted in clefting at the tip of the tongue and retrusion of tongue blanched the tissue lingual to anterior teeth.
Both the patients were neither be able to protrude their tongue beyond the vermillion border of lower and upper lip nor able to utter letter sound “l,” “t,” “d,” “n,” “s,” and “th” clearly. In both the cases, aetiology of ankyloglossia was unknown, and there was no association with any syndrome. There was no frenum related recession on the lingual aspect of mandibular central incisors. After assessing, an informed consent was attained, and lingual frenectomy was planned.


A 59-year-old female presented with a 17 year history of intense pruritus with progressive appearance of multiple hyperkeratotic papules over the lower extremities and abdomen. There was severe pruritus with an imperative need to scratch. The past history was not significant for any pre-existing cutaneous or systemic disorder. She gave a history of use of topical corticosteroids with salicylic acid combination off and on with no relief. Upon dermatological examination, there were bilaterally symmetrical hyperkeratotic, hyperpigmented, dome shaped papules over the anterior and posterior aspects of the legs, lateral aspects of the thighs and the abdomen []. Laboratory investigations including the complete blood count, erythrocyte sedimentation rate, renal, hepatic, lipid and thyroid profiles, urine analysis and IgE levels were normal. The clinical diagnosis of LA was confirmed on histopathology by the presence of globular eosinophilic deposits throughout the papillary dermis that stained positive with Congo red [].
The patient was started on oral acitretin 25 mg once daily with topical 0.05% halobetasol propionate with 3% salicylic acid lotion at bedtime. A month later, there was 90% reduction in itching and the lesions of LA started to flatten out. On completion of two months of daily dosing, acitretin was decreased to 25 mg every alternate day and the topical steroid-salicylic combination was stopped. There was complete flattening of the lesions with the disappearance of itching []. The patient was further maintained on oral acitretin 25 mg twice a week for two months, after which acitretin was stopped. The lesions of LA healed with colloid milia formation and the patient had terra firma-forme dermatosis on the posterior aspect of the legs post-treatment []. The patient did not experience any subjective adverse effects related to acitretin. Hepatic and lipid profiles repeated every two months were found to be normal. The results were maintained at eight months follow-up.


A 4-year-old female patient with a nasal anomaly was admitted to our outpatient clinic. She was born 2300 g prematurely on her 37th gestational week from a 34-year-old mother with a history of five pregnancies, two still births and two abortions. On her physical examination, she had a left-sided supernumerary nostril and cliteromegaly []. Her laboratory studies revealed low levels of androstenedione and her history showed that she had medical therapy for 1 year for her congenital adrenal hyperplasia. No additional anomaly except patent foramen ovale was detected in her work-ups. Paranasal sinus computed tomography study revealed that her left-sided accessory nostril opened to the left nasal cavity. The patient was operated, the opening of the supernumerary nostril to the nasal cavity was obliterated and the widened nostril was narrowed by excisions from the alar ground and lateral side []. A revision toward the nostril asymmetry is planned 1 year after the operation.


A 45-year-old female presented at the Oral and Maxillofacial Surgery Department of a public hospital in the city of Recife, Brazil, complaining of an increase in volume on the right side of her face and being unable to open her mouth adequately for more than a year. She reported not having suffered facial trauma or infections in the region. The extraoral examination revealed an increase in volume of the right zygomatic arch and palpation revealed hard tissue mass [], which was not evident in the intraoral inspection. The patient had limited mouth opening (8 mm), with an inability to perform protrusive or lateral movements. No pain symptoms were reported.
The tomogram revealed altered anatomy of the coronoid process, with a radiopaque mushroom shaped characteristic developing in an upward and lateral direction, invading the infratemporal space and causing deformity of the zygomatic arch. The upper region of the tumor had a growth on the zygomatic arch exceeding the limit of the infratemporal cavity and causing limited mouth opening with the inability to perform excursive movements. The lateral growth had caused deformity and fracture of the right zygomatic arch. The fracture line was evident in the three-dimensional reconstruction [].
On the basis of the clinical and imaging features, a preliminary diagnosis of pseudo-ankylosis of the right coronoid process was established. The patient was hospitalized and underwent a coronoidectomy under general anesthesia. Osteotomy of the coronoid process was performed using an intraoral incision. The removal of the fibrous tissue between the tumor and zygomatic arch and excision of the tumor were performed using a coronal incision [].
The specimen measured about 3 cm at its largest diameter and had a shape similar to a mushroom []. The microscopic analysis revealed sclerotic, lamellar and dense bone similar to the cortical bone, allowing the diagnosis of osteoma of the mandibular coronoid process [].
Postoperative recovery was satisfactory. No occlusal changes were observed and mouth opening had increased to 25 mm. Signs of injury to the temporal branch of the facial nerve were also observed. The patient was referred to a physiotherapist for the recovery of the full range of mandibular movements and the stimulation of nerve function. After 1 year of clinical and X-ray follow-up, the patient had experienced recovery of her normal facial contour, full recovery of nerve function, the return of excursive mandibular movements and maximum mouth opening had increased to 37 mm [Figures and ]. Moreover, there were no signs of recurrence.


The patient was a 44-year-old Japanese man who had been suffering from swelling of the left parotid region. The left neck also began to show swelling. He was admitted to our hospital 9 years later. On MRI scan, a 40 × 42 × 70 mm irregularly shaped mass with low intensity on T1 and T2 resonance imaging was seen in the left parotid gland (Figure A). Total parotidectomy was performed under the clinical diagnosis of parotid gland carcinoma (cT4aN2bM0). After the operation, radiotherapy (60 Gy) was performed but, after 8 months, multiple bone metastases were found.


The patient was a 66-year-old Japanese man who had been suffering from swelling of the right neck region. He was admitted to our hospital 3 months later and, clinically, this cervical lesion was diagnosed as metastatic carcinoma of unknown origin. MRI indicated an approximately 50 × 50 × 50 mm mass with low intensity on T1 imaging and partially high intensity on T2 imaging in the right submandibular regions and multiple lymph node metastases. At the first clinical examination, a 30 × 30 mm metastatic nodule was found in the right upper lobe of the lung on CT, and upper digestive endoscopic examination indicated early gastric cancer, which consisted of an erosive lesion. Right neck dissection was performed under the clinical diagnosis of metastatic tumor of unknown origin (cTxN2bM1). He has been followed up for 6 months with lung metastasis. Gastric cancer has been followed up until the present without any treatment.


A 35-year-old male was admitted to our center after sustaining a head injury during a road traffic accident (RTA). At presentation, his vitals were stable, Glasgow Coma Scale (GCS) was E2M4V2. Computed tomography (CT) scan (Cranium) was suggestive of multiple hemorrhagic contusions. Patient was managed conservatively, he was doing well and recovering but on 8th day of admission he developed episodes of fever, tachycardia and hypertension along with dystonic posturing, each episode lasted for a few minutes and there were several such episodes per day, details of such episodes are summarized in . Patient was diagnosed to be suffering from PSH after ruling out other conditions mimicking PSH as described above. He was treated using propranolol 40 mg thrice a day, clonidine 0.1 mg thrice a day. The frequency of these episodes reduced markedly on this treatment after several days, and these episodes stopped after 1 week.


A 62-year-old female who was admitted with complaints of sudden onset unconsciousness and weakness of the right side of the body. At presentation, her BP was 220/130, GCS was E3M5V1, right hemiparesis was present and right plantar was extensor. Noncontrast CT scan of the head was suggestive of left thalamic bleed. Patient was given injection labetalol 20 mg intravenous to control the BP; she was managed conservatively using cerebral decongestants and physiotherapy. Her condition showed improvement and on 9th day of admission she was fully conscious, although weakness of her right upper and lower limb persisted. She was about to discharge, when she developed episodes of fever, tachycardia, diaphoresis and hypertension along with dystonic posturing. These episodes were diagnosed as PSH after exclusion of other closely related conditions. A repeat CT scan of the brain was repeated, which was not suggestive of any other abnormality apart from resolving thalamic hematoma. Her episodes also responded well to propranolol and clonidine. Details of her PSH episodes are described in .


A 17-year-old male who was admitted with fever, altered sensorium and seizures. He was diagnosed to be having Japanese encephalitis (JE) as his cerebrospinal fluid (CSF) was positive for JE antibodies and magnetic resonance imaging (MRI) brain was showing bilateral thalamic hyperintensities. He was managed conservatively and was showing gradual improvement when on 18th day of admission he started to develop episodes of PSH. He was treated using propranolol and clonidine, but his episodes did not stopped completely so gabapentin 600 mg/day was added, after few days of adding gabapentin his episodes stopped.


A 42-year-old male was diagnosed as a case of acute ischemic stroke with infarct in right middle cerebral artery territory, he presented to us with left hemiparesis. He was being managed using aspirin 150 mg/day and atorvastatin 20 mg/day and other conservative measures. His hospital course was uneventful till day 10 when he started to develop episodes of PSH. He also responded well to propranolol and clonidine.


A 37-year-old male, who sustained head injury following RTA; he was admitted with GCS of E1M4V1. He developed episodes of PSH on 12th day of admission. He responded poorly to propranolol and clonidine, but on adding gabapentin 600 mg/day his episodes subsided within a few days.


A 27-year-old male presented to us with fever, headache and altered sensorium. He was diagnosed as a case of tuberculous meningitis on the basis of CSF findings, and MRI brain revealed meningeal enhancement, basal exudates and communicating hydrocephalous. He was being treated with antitubercular drugs and steroids; he developed PSH episodes on 11th day of admission, his episodes responded to morphine 15 mg thrice daily, clonidine 0.1 mg thrice daily and propranolol 80 mg twice daily.


A 15-year-old girl presented to a dentist with the chief compliant of a gradually progressive parageusia without halitosis, which started 9 months ago. Initial examination of the mouth did not reveal any mucosal lesions or dental cavities. On physical examination of the head and neck, she had a coarse face, mild acne, and sporadic macular lesions on the posterior neck. Similar lesions were found in the abdominal area.
During the initial evaluation, panoramic x-ray revealed multiple bilateral unilocular cysts in the mandible (), and her chest X-ray showed bilateral bifid ribs (). Non-enhanced computed tomography (CT) scan demonstrated bilateral odontogenic cysts of the mandible and maxilla ( and ), along with calcification of anterior part of the falx cerebri (). The patient underwent a biopsy of the left maxillary cyst under local analgesia and histopathologic evaluation revealed odontogenic keratocyst (OKC), nowadays better known as keratocystic odontogenic tumor (KCOT). She was eventually diagnosed with Gorlin-Goltz syndrome based on histopathologic and genetic analysis findings.


A previously healthy 29-year-old woman was referred to our institute due to paraparesis after a fall. Her visit to our institute was prompted by the development of motor weakness and voiding difficulty on the day of the visit. Physical examination revealed a weight of 118 kg, height of 162 cm, and body mass index of 44.9 kg/m2, indicative of morbid obesity for this patient. There were no associated systemic or muscle contusions, internal organ damage, or swelling of the extremities. However, we had observed marked rigidity in both arms and legs. Preoperative laboratory test findings, including full blood count, electrolytes, creatinine, liver function test, and urine analysis were all unremarkable. However, a neurological examination revealed reduced patellar and Achilles tendon reflexes in both legs, motor weakness (Grade III/Grade III), and hypoesthesia below the T4 dermatome. Magnetic resonance imaging (MRI) and computed tomography scanning of the thoracic spine had revealed ossification of the posterior longitudinal ligament and severe stenosis at the T3, T4, and T5 levels ().
General anesthesia was induced using propofol, fentanyl, and rocuronium for surgery. After endotracheal intubation, the patient was placed in the prone position on the operating table cautiously, and the neck was kept in line with the spine. The head was maintained in a hyperflexion position using Mayfield head pins to obtain a better surgical view. Decompressive laminectomy at T4, T5 and interbody fusion at the T3-4 level by the posterior approach was performed (). The T3 posterior segment was removed by using drill and rongeur. Then dural sac and both T4 nerve roots were exposed. Due to the narrowing of disc space, interbody fusion using autologous block bone was performed. The surgery was uneventful and had lasted 297 minutes. After then, she was transferred to the recovery room and was alert, oriented, and hemodynamically stable. After extubation, she complained of pain in the anterior aspect of both arms and thighs. On physical examination, we noted the presence of mildly swollen and erythematous regions in her arms and thighs over the deltoid, pectoralis major, and rectus femoris muscles. Neurological examination revealed that there was no neurological change of her motor weakness (Grade III/Grade III). With the diagnosis of unsuitable positioning, she was treated with adequate hydration and analgesics for pain control. However, the color of her urine changed from clear yellow to dark brown, and myoglobinuria was developed 3 days after surgery with a high serum creatine kinase (CK) level of 38,732 IU/L (normal, 55-215 IU/L). Acute renal failure developed 5 days after surgery despite the initiation of intensive fluid replacement and diuresis. The CK level was peaked at 49, 735 IU/L and serum myoglobin exceeded 24mh/dL (normal <2.5mg/dL) on 5 day after surgery. She was referred to department of nephrology for intensive supportive treatment. Although the patient received intensive supportive treatment, she became anuric 7 days after admission and required hemodialysis. We could not find any evidence of muscle breakdown, but her condition had deteriorated rapidly, and she eventually had a cardiorespiratory arrest with dilated nonreactive pupils. Unfortunately, she died 12 days after spine surgery.


A 68-year-old male with a 30-year history of AS presented with a 3-month history of aggravation of a progressive urinary incontinence and pain in the left buttock associated with paresthesia of the right buttock which radiated down the back of the leg to the level of the sole. He also presented a three-year history of insidious onset, slowly progressing, urinary incontinence and numbness along the heel and sole of the left foot. On physical examination, the left foot showed a grade IV weakness with an atrophy of the sole. Mild hypesthesia and paresthesia was found in his left posterior calf and sole along the S1 dermatome. The left ankle jerk was diminished. No mechanical allodynia was noticed. The patient had experienced intermittent low back pain in the past, but was not currently asymptomatic regarding this for many years.
On admission, laboratory values were as follows : erythrocyte sedimentation rate 22 (0-15) mm/hr/C-reactive protein 0.23 (range 0.03-0.47) mg/L and negative Anti-Nuclear Ab 2.5. Electromyography and nerve conduction study showed bilateral lumbosacral radiculopathies, clinically equivalent to a cauda equina syndrome, with a more severe involvement of the left side. An urodynamic study showed an evidence of a neurogenic bladder with detrusor hypoactivity and low compliance of the bladder.
Plain X-rays of the lumbosacral spine and subsequent 3D computed tomography (CT) showed the typical 'bamboo spine' of AS with fusion of both sacroiliac joints, squaring of the lumbar vertebral bodies, syndesmophyte formation along the lumbar spine, and also multiple erosions of the roof of an enlarged spinal canal (). A CT scan showed the spinal canal enlarged by laminar erosions (). The magnetic resonance imaging (MRI) demonstrated a widened thecal sac from L4 to S1, with an extensive scalloping of the laminae and a spinous process caused by multiple thecal diverticula (). Axial T2-weighted images showed a clumping of the nerve roots of the cauda equina on the side of the ecstatic thecal sac suggesting an adhesion of nerve roots to the ararchnoid membrane. The conus medullaris terminated at the level of L1 body, however, the filum terminale seemed to be adherent to the posterior wall of the thecal sac at L1 level (). No abnormal enhancement or mass lesion such as herniated lumbar disc was found.
Considering the chronic progressive course of a CES and the radiologic findings of adherence of filum terminale to the posterior wall of the dural ectasia, dethetering of the filum terminale was performed after getting informed consent from the patient and his relatives.
The dura was opened through a laminectomy of L2 under general anesthesia. The filum terminale was identified under microscopic vision and tested with a low current bipolar stimulation (). After identifying the filum, it was coagulated and sharply divided, and the dura and overlying wound were closed in layers. No adverse effect of the untethering procedure was observed and the postoperative course was uneventful as well. After 6 months of follow up, the urinary incontinence was not improved. The left buttock pain was improved; however, the dysesthetic pain and paresthesia in his left posterior calf and sole were not improved.


A 29-year-old woman with a complaint of diminished vision in her left eye since 7 months ago was referred to our clinic. She had a history of left eye redness which did not respond to topical medications. In past medical history, she had a seizure attack 4 years before and had received appropriate treatment.
Examination in the right eye (OD) was normal. In the left eye (OS), visual acuity was one meter finger count, anterior segment examination was normal, intraocular pressure was 14 mmHg, and relative afferent pupillary defect was 2+. In the posterior segment examination, there was a dome-shaped partially pigmented mass with vitreous hemorrhage obscuring tumor features.
Orbital magnetic resonance imaging (MRI) revealed an enhancing nodular mass lesion in the posterior aspect of the left globe protruding into the vitreous and associated with exudative or nearby hemorrhagic retinal detachment [Figures , and ]. B scan of left eye revealed a dome-shaped solid mass with high internal reflectivity 16.5 × 12.5 × 11 mm in size with point calcifications [Figures and ].
Intraocular tumor, possibly retinoblastoma or aggressive astrocytoma, was considered as a provisional diagnosis and biopsy was suggested. Fine-needle aspiration biopsy (FNAB) was performed but the specimen lacked enough cells for definitive diagnosis. Vitrectomy (23 G) assisted biopsy was carried out. The sample was obtained from the vitreous after removing the vitreous hemorrhage and also from the tumor by high aspiration and low cut level.
With a histologic diagnosis of choroidal melanoma, enucleation was proposed but was refused by the patient. After one month, the patient presented with severe left eye pain, visual acuity was no light perception, anterior segment showed circumcilliary congestion, neovascularisation of the iris and angle with intraocular pressure of 60 mmHg. Due to severe pain and loss of vision, at the time, the left eye was enucleated.
In the pathology report, macroscopically, a blood filled globe cavity and a tumor with hypo and hyperpigmented areas and foci of hemorrhage 20 mm in diameter and 5 mm in thickness was shown []. The microscopic section was composed of atypical proliferation of cellular elements with small round hyperchromatic nuclei and scant cytoplasm, arranged as closely packed cells forming a nodule occupying half of the posterior chamber. The neoplastic cells were differentiated in some areas accompanied by Flexner-Wintersteiner rosette formation [Figure and ]. No calcification was reported. For a definite diagnosis immunohistochemistry (IHC) study was recommended. IHC stains were positive for neuron-specific enolase (NSE), retinoblastoma protein and synaptophysin and negative for human melanoma black 45 (HMB45) and leukocyte common antigen in the tumor cells and hence the final diagnosis was retinoblastoma.


Emergency medical service (EMS) brought in a 4-year-old girl, who was involved in a car accident, to the emergency room (ER). The mechanism of trauma was a car accident; she was ejected from the car after it flipped. Her past medical history was noncontributory. Before arriving to ER, she had had a seizure for three minutes controlled by administration of 2 mg diazepam by EMS paramedics. Upon admission, she had temporoparietal skin abrasion and subgaleal hematoma; the child was unconscious. Her Glasgow coma scale (GCS) score was eight, (her response to pain stimulation was eyes opening, grunting, and withdrawing the limbs). The pupils were miotic with symmetrical and had a weak response to light. Neurological examination findings were symmetrical. We immediately intubated her. Early vital sign were as follows: pulse rate (PR), 150 beat/min; respiratory rate (RR), 18 breath/min; blood pressure (BP), 100/70 mm Hg; axillary temperature (AT), 36.4℃; and arterial oxygen saturation (SpO2), 98%. The primary brain computed tomography (CT) showed SDH (). Early laboratory tests results were within normal limits. Neck, pelvic and chest radiography findings were unremarkable. Serial FAST (focus abdominal sonography for trauma) revealed no significant finding. Doppler ultrasonography revealed the carotid artery thrombosis. We admitted her to the ICU. One day after admission, her GCS score decreased to six; the attending neurosurgeon requested a control brain CT that showed hemicerebral infarction (). Another brain CT was performed on the fifth day of admission. The next day, her condition deteriorated, her GCS score decreased to three, and her pupils became bilaterally dilated and unresponsive to light. She was pronounced dead. The latest laboratory test results showed normal values except for hypernatremia (153 mmol/L).


An 86-year-old male patient with a past history of muscle-invasive bladder cancer diagnosed in 2000 was treated with external beam radiotherapy and transurethral resection of the bladder tumour. He was then followed up with 6-monthly surveillance flexible cystoscopies. A recurrence of his bladder tumour in November 2007 staged as carcinoma in situ and was treated with standard six instillations of intravesical BCG. The final BCG therapy was given in May 2008.
His renal function deteriorated during the course of urology follow-up to a creatinine of 310 μmol/L and an estimated glomerular filtration rate of 17 mL/min/1.73 m2 and a renal ultrasound showed moderate left hydronephrosis and severe chronic hydronephrosis of atrophic right. Left hydronephrosis was related to recurrence of bladder cancer and was not evident on previous imaging. He had bilateral ureteric stents inserted and was referred to the renal team. His renal function continued to decline and he was started on continuous ambulatory PD (CAPD) in July 2009.
In December 2010, he presented acutely with abdominal pain, fever and cloudy dialysis bags. Microscopy of his CAPD fluid showed 400 × 106/L of polymorphs with no lymphocytes and no growth on culture. He was treated for PD peritonitis with standard intraperitoneal vancomycin and gentamicin. Clinically, he made a recovery and was discharged home only to present again 1 month later with similar symptoms. On this occasion his CAPD fluid showed 130 × 106/L polymorphs, 28 × 106/L lymphocytes and again no growth on standard culture. He recovered after receiving a further course of intraperitoneal vancomycin and gentamicin. He re-presented a month later with septic shock and cloudy bags. Standard blood, urine and PD fluid cultures were all negative. Due to the recurrent nature of his peritonitis, his CAPD fluid was sent for acid- and alkali-fast bacilli, which was positive. His Tenckhoff PD catheter was therefore removed and peritoneal tuberculosis was confirmed by extended culture of CAPD fluid. The isolate was later identified as BCG strain by genetic analysis (using Hain GenoQuick® technology) at the regional public health laboratory (Birmingham, UK). A cytology of PD fluid was not performed. Computed tomography scan of the chest, abdomen and pelvis did not reveal any evidence of disseminated tuberculosis or lymphadenopathy.
His renal replacement therapy was changed to in-centre haemodialysis and he was started on anti-tuberculosis medications. He was commenced on Rifater (rifampicin 120 mg, isoniazid 50 mg and pyrazinamide 300 mg) five tablets and ethambutol 300 mg daily for 6 weeks before switching to Rifinah (rifampicin 300 mg and isoniazid 150 mg) two tablets daily prescribed for a further 11 months. He received pyridoxine throughout his treatment. He had no further episodes of peritonitis during his treatment but died from withdrawal of haemodialysis 4.5 months after presentation with tuberculous (TB) peritonitis.


A 68-year-old male came to our hospital because of dyspnea. He had symptoms like dyspnea and weight loss for 4 months before he came to our hospital. He was admitted to another university hospital and diagnosed with MPM through thoracoscopic biopsy. So, pathological examination was not performed on the patient in our hospital. In the other hospital, chemotherapy had been already performed on him but the symptoms did not improve. To control dyspnea, pleurodesis was carried out following two times of chest tube insertion. Instead, increased effusion on the right sided pleura aggravated dyspnea. Eventually he had to come to our hospital with aggravated dyspnea. He had taken medications for 3 years to treat hypertension. His smoking history was 30 pack years. His job had nothing to do with asbestos. At the time of admission in our hospital, his initial vital signs are as follows: blood pressure, 150/80 mm Hg; respiratory rate, 28 breaths per minute; pulse rate, 86 beats per minute; and body temperature, 36.5℃. Chest auscultation revealed decrease in breathing sound over the entire right lung. The chest radiography taken at the time of admission showed about 11-cm mass with multiple pleural nodules and pleural effusion in the right hemithorax (). Chest computed tomography (CT) scans revealed about 11.5×16-cm mass with pleural effusion and disseminated pleura metastasis in the right hemithorax. Direct invasion to paratracheal lymph node, right paraaortic nodal station, and pleura in the back of the right lower lobe was observed (). Bone scan revealed bone metastasis on the ninth right rib and the fourth lumbar spine.
The patient with severe dyspnea did not respond to oxygen supply. He had already received chemotherapy in the other hospital but his symptom aggravated. He was disappointed with the treatment and he refused to take any more chemotherapy. So we advised him to try photodynamic therapy (PDT) for treatment and he took the advice.
To perform PDT, photosensitizer used for the patient is photogem (Lomonosov Institute of Fine Chemical, Moscow, Russia), and 2.5 mg/kg was injected through intravenous 48 hours before the procedure. To prevent complications due to skin photosensitive reaction, the patient was advised to avoid direct light for 48 hours. After that, under general anesthesia the patient had thoracoscope inserted after posterolateral thoracotomy. As a light delivery device, flexible cylindrical fiber was used, and diode laser system (Biolitec, Jena, Germany) was employed based on 632 nm wavelength, with 15 J/cm2 light doses being applied (). Following PDT, light shield was carried out to avoid sun light. After PDT, dyspnea improved and complications were not observed. Once his symptom and general condition got better, he changed his mind and decided receive chemotherapy.
Pemetrexed and cisplatin combination chemotherapy was performed. Pemetrexed 500 mg/m2 with cisplatin 60 mg/m2 were administered once every 3 weeks. The patient was discharged with better condition of dyspnea (). After six cycles of pemetrexed/cisplatin combination chemotherapy, the tumor volume had not shown significant interval change. But, he remained relatively stable for 6 months after multimodality therapy. At 7 months of the multimodality treatment, dyspnea was aggravated and progression of disease was shown on the chest CT. So the PDT single treatment was carried out in the same way and the symptom improved. Although significant decreased in the mass volume was not seen, his symptom improved and he remained stable without further treatment during the 9 months after the second PDT. Ten months after the second PDT, dyspnea became worse again, and progression of disease was shown on the chest CT. The third PDT was intended but failed to be performed due to the patient's financial reason. Instead, the patient took combination chemotherapy. The patient was treated with gemcitabine 1,000 mg/m2 on days 1 and 8, and cisplatin 60 mg/m2 on day 1. This chemotherapy regimen was repeated once every 3 weeks. However dramatic response was not shown. He died 27 months after initial diagnosis with MPM.


Case 1: female, 27 years old, married, housewife, diploma
It was about 3 years ago that I visited the home care due to lethargy, thirst and frequent urination. The doctor told me to go to the city for blood test. I traveled to the Damghan to go to the Social Security Clinic and did blood test. After getting the result and presenting it to the doctor, I realized that my blood sugar is around 260 and I am suffering from type 2diabetes. The doctor told me that I should inject insulin to control the disease before the breakfast. I also use herbal medicines as supplement. Every 3 month I visit the Social Security Clinic located in Damghan to measure the level of my blood sugar. High cost of medicines, tests and commuting are among my problems for which I always should borrow some money from my brother. I believe to the Imams and at the martyrdom night of Imam Ali pray and distribute dates as charity.


Case 2: male, 63 years old, married, unemployed, illiterate
5 years ago, I always was lethargic and tired. Thirst, severe asthenia and frequent urination were my problems. A medicine was prescribed by doctor of home care but it couldn’t improve my bodily condition. A friend of mine recommended me to go to a clinic in Damghan. The doctor advised me to do blood test. After testing, it was evidenced that my blood sugar (hyperglycemia) is high and I suffer from type 2 diabetes. I am inhibited to eat bread, rice and candy. Last year, my finger was cute in Rezaee hospital of Damghan due to infection. I currently take insulin injections and use opium for decreasing pain and hyperglycemia (blood sugar). I have made a vow to go to the imam Reza’s shrine if I get recovered.


Case 3: female, 48 years old, housewife, illiterate
I missed my husband because of an accident 6 years ago. Since then I was depressed. After that I felt some tingling in my body fingers and also some pain in my stomach and head. The village doctor told me that I must do blood test. I traveled to the city according to my brother’s advice. After blood test, I realized that I am suffering from high blood sugar. The doctor prescribes me the related medicines which are hard to provide. Nowadays, I’m using pills for controlling my disease. The pills are also expensive. In my opinion there is no benefit for herbal medicines, so I use none of them.


Ms. V, a 10-year-old girl was referred to psychiatric Department with complaints of bleeding from eyes for last 3 months. Bleeding was spontaneous, transient, without any history of trauma, subsided as soon as the site was wiped. There were around 5–6 instances of intermittent bleeding within the same day.
Ms. V has been brought up by her maternal grandparents living in a rural village, who pampered her by yielding to all of her demands. When the mother visited her, she tried to discipline her adamant behavior and tried to be strict with her demands. After one such instance, Ms. V had bleeding from eyes. As she continued to have bleeding episodes, she was hospitalized. During the admission in hospital, Ms. V was found to be irritable with frequent crying spells. Social withdrawal was also noted. Establishing rapport was difficult as she was guarded and remained silent to most of the questions asked on the 1st day. On subsequent days, Ms. V started to open up gradually revealing the stressors. She expressed that: “Whenever she was not happy, she had bleeding.” Clinically depressed mood and preoccupation about the symptoms were evident in Ms. V. On Childhood apperception test, conflicts with mother came into light. She has average levels of Intelligence with IQ of 93. Computed tomography brain was normal. All Hematological investigations (Peripheral blood smear examination, complete blood counts, bleeding time, clotting time, prothrombin time, activated partial prothrombin time, platelet counts, Factor XIII assay) were found to be normal.[] Ophthalmic examination, Otoscopy, and rhinoscopy were also normal. She was started on Sertraline 25 mg at bedtime. In the hospital stay, Ms. V expressed concerns about the photographs taken by the mother during these bleeding episodes, to be shown to the doctors. She was apprehensive whether the same might be depicted in media. Meanwhile bleeding from eyes (being a rare symptom) sought the attention of multiple medical personnel in the ward. Ms. V started to become anxious whenever her symptoms were discussed by the medical team and other patients. During further episodes of bleeding, she became concerned and fearful whether the bleeding from eyes may represent a serious and life-threatening illness.[]
When her mother was discussing the prognosis of the illness with her physician, Ms. V started to suddenly exhibit giddiness, jerky movements of the head and upper limbs. She also exhibited childish talk and started clinging onto her mother. Few minutes later, she claimed that she could not identify her mother. On examination, there was no focal neurological deficit or altered consciousness.[] Mental status examination revealed Ms. V to be agitated and irritable. She was found to be staring at the space most of the time during the interview. Initiating conversation and establishing rapport were difficult. She was anxious, occasionally communicating (through gestures) and most of the time weeping. Intramuscular Lorazepam was administered. The dose of sertraline was increased to 50 mg/day. Clonazepam 0.5 mg/day in 2 divided doses and propranolol 30 mg/day in 3 divided doses were added to the prescription. After 2 days, bleeding episodes and other symptoms gradually reduced.
Ms. V was 2nd born of nonconsanguineous marriage belonging to low socioeconomic strata. She has an elder male sibling with moderate intellectual disability and a younger female sibling who is healthy. As the mother had difficulty raising three children simultaneously, Ms. V had been brought up by the maternal grandparents in their village. School refusal was present throughout the class I. She cried every day to go to school. The grandparents had always been yielding to all the demands of Ms. V perpetuating her adamant behavior. Mother who lives in Chennai visited Ms. V every fortnightly or monthly. During her periods of short stay with Ms. V, mother tried to control and discipline her.
After completion of primary schooling, she was admitted in a boarding school in the nearby town. Only after considerable persuasion, Ms. V consented to join the hostel. The first 2 days of stay in the hostel was unpleasant for her. She was scolded by another senior student for not complying with her instructions. When Ms. V had complained about the presence of some insects in the hostel food, she was asked to ignore the insects and continue eating the same. She threw away the unhygienic food. Following these significant distressing experiences,[] Ms. V started to have bleeding episodes due to which she was sent to the home.


A 33-year-old woman with a 3-month history of vision loss in right eye and left hemiplegia due to right ICA occlusion and MCA infarction. The best corrected visual acuity was 20/20 in the left eye and there was no light perception in the right eye. Ocular motility, intra-ocular pressure, anterior segments were normal in the both eyes. Her fundus examinations were normal except optic atrophy in the right eye. Visual field test was not performed because of cooperation difficulties. Magnetic resonance imaging (MRI) revealed an infarction of the right MCA (). Computed tomographic angiography showed right ICA occlusion (). Optical coherence tomography (OCT) demonstrated 6 clock hours of RNFL thinning in the right eye (). Average RNFL thickness of the right and left eyes were 53µm, 96 µm respectively.


A 62-year-old male came to our outpatient department complaining of a slowly enlarging painful swelling over the left index finger of 1 year duration. The pain was intermittent and radiated to the left wrist. There was no history of diabetes mellitus or any other cutaneous lesion. Examination revealed a sharply demarcated nodular plaque with thick adherent scale over the skin on the distal phalanx of left index finger. The lesion had a warty, keratotic surface []. The nail showed complete dystrophy. X-ray of the involved hand revealed soft tissue swelling in index finger with partial resorption of tip of distal finger phalanx.
A punch biopsy performed from the lesion was sent for histological examination that demonstrated an acanthotic epidermis with reticulate proliferation of malphigium layer extending into the dermis in the form of branching anastomosing strands []. These strands, 2-4 cell layers in thickness, were seen extending from multiple points in the epidermis. Also, identified were small ductules lined by cuboidal cells within the epithelial strands. These ductules are indicators of eccrine differentiation. The dermis entrapped between the epithelial strands is mildly cellular fibrovascular in appearance [].
The tumor cells reacted with PAS stain.
The lesion was completely excised by the general surgeon and sent for histopathological examination which confirmed the above findings.


A 20-year-old male presented to skin out patient department of tertiary care center with history of (h/o) elevated lesion and ulceration over nose and adjoining parts of cheeks since 1½ year with no nasal symptoms. It started with a single papule over left cheek which gradually enlarged and progressed in due time to involve nasal bridge, extending to right side of nose. He developed swelling over right side of neck 3 months back. Patient was treated with oral and topical antibiotics by general physicians showing no improvement. Patient had h/o weight loss and decreased appetite but no h/o cough and fever. No h/o trauma at that site. No past h/o of pulmonary TB present. Patient's mother died of pulmonary TB 2 years back. General and systemic examinations were normal. Cutaneous examination showed well-defined erythematous, scaly plaque of around 8 cm × 4 cm in size, involving nasal bridge, both naso-labial folds and cheeks. Two erythematous papules were present over left cheek separately. Single ulcer of 3 cm × 3 cm in size at the center of nose, with pus and sero-sanguineous discharge was seen []. Right sided submandibular and jugular nodes were enlarged and tender. Nasal septum, lips and oral mucosa were normal. Anterior rhinoscopy and nasal endoscopy findings were normal.
Laboratory findings were normal except erythrocyte sedimentation rate which was 60 mm/1st hour. X-ray chest was normal. Veneral disease research laboratory and ELISA for HIV were negative. Mantoux test was positive with 23 mm × 18 mm diameter induration and erythema. Ziehl-Neelsen staining didn’t reveal any acid fast bacilli. Deep biopsy taken showed features of LV on H and E, staining. Epidermal atrophy and epitheloid cell granuloma composed of langhans type of giant cells, epitheloid cells and lymphocytes along with intense inflammatory infiltrate was seen in dermis [].
Patient was treated with category-1 ATT under revised national TB control programme. Lesions healed after 6 months of therapy but an atrophic scar was left over nose at the site of ulceration [].


A 60-year-old female presented with one year history of three asymptomatic slowly growing lesions on her right lower leg. It started as a single raised lesion near medial aspect of right ankle, which slowly increased in size. Three months later patient developed two more similar slow growing lesions nearby. On examination, patient had three linearly arranged erythematous polypoidal fleshy nodules of size ranging from 1 × 1 to 3 × 2 cm, with the larger lesion showing ulcerations and crusting on the surface []. Lesions were firm and nonindurated. There was no pallor or lymph node enlargement. Systemic examination did not show any abnormality.
A differential diagnosis of sporotrichosis, chromoblastomycosis, B cell lymphoma and squamous cell carcinoma were considered and patient was investigated. Routine blood investigations, chest X-ray and ultrasound abdomen were within normal limits. Excision biopsy of a nodule was taken and sent for fungal culture and histopathological examination. Fungal culture showed no growth. Histopathology showed a subepithelial lesion extending up to subcutis composed of sheets and nests of cells in zellballen pattern [] suggestive of paraganglioma. Cells had clear to eosinophilic cytoplasm and vesicular nuclei with prominent nucleoli []. No epidermal involvement or pagetoid spread was seen. Atypia, increased mitotic activity or necrosis were absent, thus ruling out malignant tumors-like melanoma. Immunohistochemistry staining showed densely positive S100 and melan-A and faintly positive cytokeratin [] confirming the diagnosis of PDMT.


A 68-year-old man was diagnosed with achalasia associated with multiple esophageal diverticula. He had symptoms of dysphagia and regurgitation of food for 2 years. Thus, barium esophagography, esophageal manometry, and esophagogastroduodenography (EGD) were performed. Barium esophagography showed three right-sided esophageal diverticula along the mid-to-distal esophagus with achalasia (). Initial esophageal manometry showed aperistalsis in the esophageal body, and each swallow resulted in simultaneous contractions (). The average peak pressure of the esophageal body was 56 mm Hg as determined by conventional manometry. Therefore, the patient in this case was considered to have vigorous achalasia, defined by an average esophageal body pressure ≥37 mmHg, rather than the classic form of achalasia associated with lower pressures. His symptoms improved after botulinum toxin injection. However, dysphagia, regurgitation of food, and epigastric discomfort recurred 5 years later. A follow-up EGD showed a slight increase in the size of the previous diverticula, mild erythematous mucosal changes, and multifocal lugol-voided lesions (). The biopsy result showed a moderate degree of epithelial dysplasia. Because the lesions were located in a large and tortuous diverticulum, performing endoscopic submucosal dissection (ESD) was considered too difficult and dangerous. Photodynamic therapy (PDT) was therefore considered a better option and was administered. Two months after PDT, EGD was repeated to evaluate the response to PDT (). Mucosal nodularity was observed at a nontreated diverticulum, which on biopsy was revealed to be squamous cell carcinoma in situ (). The patient underwent an Ivor-Lewis esophagectomy with regional lymph node dissection for recurrent multifocal esophageal neoplasia, and the final pathologic staging was TisN0M0 (stage 0). The patient showed no evidence of disease at the 3-year follow-up.


A 22-year-old male presented with history of a slowly progressive painless swelling in the right parieto-occipital region since the last eight years. There was history of preceding trauma. On examination there was a large, 14 × 12 cms, non tender, compressible swelling with ill defined margins []. The swelling involved the skin and no bony abnormality could be felt underneath. There was no bruit over the mass. There were no café-au-lat spots over the body. FNAC done at another hospital revealed blood only. A computed tomography revealed a hypodense lesion, without enhancement after contrast administration []. According to the clinical and CT scan findings, a provisional diagnosis of subcutaneous venous hemangioma was made. At surgery, running sutures were passed around the swelling and a grayish pink soft vascular tissue was separated from the pericranium and galea. At places, the lesion was found to be infiltrating the skin. Redundant skin was excised. Post operatively, the skin necrosed at places and was treated with antibiotics and dressings. Histopathology showed elongated cells arranged in haphazard fashion. Wagner Meissner bodies and pigment laden macrophages were seen []. Interspersed adnexal structures were present. Fibro-adipose tissue with collagen, and spindle cells bearing wavy nuclei were also seen. Some nerve bundles were found in the tumor. The final histopathological diagnosis was subcutaneous diffuse neurofibroma.


An 80-year-old woman, with a remote history of breast carcinoma (15 years before), underwent laparotomic hysterectomy with bilateral oophorectomy, pelvic lymphoadenectomy, and omentectomy for an endometrial carcinoma (clear-cell type, pT2b, N0, M0, G3, R0, V0, L0; FIGO IIb) at the end of 2008. Six weeks later, she received external fractionated radiotherapy (1.8 Gy fraction for a total dose of 50.4 Gy) on the pelvic area. Three years later, she developed a rapid expansive painless right temporo-parietal mass, non-pulsating, covered by normal cutis. A computed tomography (CT) scan of the head [] revealed an osteolytic lesion of the calvaria in the temporo-parietal area, suspicious for a metastasis. In January 2012, the patient underwent macroscopic radical resection of the tumor of the skull, invading the superior part of the temporalis muscle, with craniectomy and immediate acrylic cranioplasty. Underlying dura was removed and duraplasty with heterologous material was accomplished. Histological investigation documented a metastasis from carcinoma with clear-cell appearance in accordance with an endometrial origin []. Subcutaneous tissue was not infiltrated as well as the galea and dura (histological confirmation). Postoperative course was uneventful. Given the radical resection, confirmed by a CT scan of the head [], no adjuvant treatment was prescribed at that time. Bone scintigraphy revealed no other suspicious lesions of the skeleton. At 4 months follow-up, the patient is alive and neither clinical nor radiological signs of local recurrence are present.


A 40-year-old non-smoker female patient in good general health conditions attended a private dental clinic with two Branemark implants (unknown trademark) in function for about 2 years. The implants were placed in the posterior left side of the mandible, corresponding to the mandibular left second premolar (3.75 X 10 mm) and first molar (3.75 X 7.5mm), joined by the structure of a metal-ceramic fixed partial denture. The UCLA abutment screws were fractured and the crowns were dislodged. The crows were cemented to the abutments and exhibited visible misfit, mainly on the distal implant. There was no radiographic evidence of marginal bone loss. After several unsuccessful attempts to remove the fractured screw fragments, it was decided to remove the implants. The fractured components were further analyzed with a scanning electron microscope (FEI Philips XL-30, Eindhoven, The Netherlands) to assess the structural failures.
Failure analysis by SEM demonstrated the occurrence of two moments of rupture in the first fractured implant (mesial), corresponding to the mandibular left second premolar, with evidence of ductile fracture due to shear strength, as demonstrated by the first line, followed by compression ( to ).
In the second implant, the fracture was characterized by a strong torsional component, with fewer compressive evidences and characteristics of final fracture due to the presence of "dimples", also called alveolar-like structures. There were no signs of failure in the material or screw fabrication ( and ).


A 39-year-old man visited our Korean Internal Medicine Department complaining of a severe headache over his left parietal region, left-sided tongue weakness, loss of taste and sensation on the left side of his inside tongue, a slight alteration in speech, vague swallowing difficulty, and indigestion for 5 days. There was no past history except Bell's palsy one year earlier and gastritis six months before. The medical history showed no trauma, other illnesses, family history of disease, medications, smoking, drinking, and so on. He had not caught a common cold, nor had be received any vaccination or specific treatment before his first visit.
On neurological examination, his tongue deviated to the left on protrusion but no atrophy or fasciculations were observed. Mild right-sided deviation of the uvula and a decreased gag reflex on the left side of the throat were observed. The other pathological reflexes were normal. Magnetic resonance imaging (MRI), with and without gadolinium, and computed tomography (CT), with and without enhancement, of the brain, brain stem, upper neck, and skull base were normal. Laboratory tests and electrocardiography were also normal. The syndrome differentiation of the patient was categorized as the syndrome of ascendant hyperactivity of liver yang with heart fire on the grounds that overwork and much stress had given a rise to a flushed face, bitter taste, thin dry tongue coating in the mouth, palpitation, and string-like pulse
From among the types of eight principle pharmacopuncture (EPP) a 0.2-mL dosage of Hwangyeonhaedoktang pharmacopuncture was administered at CV23 and CV17, respectively, at a perpendicular angle and a 0.5-mm depth by using the tapping method [].
0.25 mm × 3.0 mm stainless needles (Dongbang Acupuncture Inc., Korea), was applied at P06, Li05, TE05, and G37 on the right side of the body at depths of 0.5~1 cm, a total of 4 needles, by using neutral supplementation and draining (平補平瀉) for 20 minutes.
Zhuapiandutongbang(左偏頭痛方)was administered at 30 minutes to 1 hour after mealtime three times a day for 5 days. Herbal compounds, a twentyfold dosage of the total amount in Table , were decocted in 6000 mL of water for two to three hours. The extracted liquid was packed in 15 pouches, 120 mL per pack. The composition of zhuapiandutongbang is shown in Table .
The severity of headache was decreased 20% on the first day of treatment, 50% on the third day, 70% on the 4th day, and 100% on the 10th day. Herbal medicine was stopped on the 6th day of treatment because of cost problems. The left-sided deviation and the weakness of tongue were improved 30% on the 4th day and 100% on the 10 days of treatment (Figs - ). The swallowing difficulty was recovered 10% on the 3rd day, 50% on the 4th day, and 100% on the 10th day of treatment. The mild dysarthria on the left side of throat was restored 30% on the 3rd day, 80% on the 4th day, and 100% on the 10th day of treatment. The taste and the sensation of the tongue were regained 20% on the 3rd day, 50% on the 4th day, and 100% on the 10th day of treatment.


A 16-year-old girl visited our clinic with PNFs in March 2010. She had been diagnosed with PNFs in NF1 10 years earlier and had surgery to remove the benign tumors three times at a general hospital in Seoul during her first 4 years of treatment at that hospital. Although she had undergone surgery, the re-growths of the PNFs occurred rapidly, so her family wanted to consider the use of alternative medicines. She had multiple subcutaneous fibromatoses (30 cm × 20 cm) in the right hip joint and pelvic region, subcutaneous nodules (1 cm × 5 cm) in the left hip joint, and limited range of motion in the right hip joint (Fig ). The L-spine and pelvic X-rays showed only low-grade scoliosis, no bones had been affected by fibromatosis (Fig ). She also showed symptoms of general weakness, coldness of the hands and the feet, and skin rashes.
The patient and her family signed an informed consent form, and this case was approved by Institutional Review Board of the Korean Medicine Hospital of Sangji University.
SBV was administrated intracutaneously at the borders of the PNFs in the pelvic regions in a dose of 5 mL every two weeks. We used SBV produced by the Korean Pharmacopuncture Institute and 1-mL syringes (26 G × 13 mm, Sungshim Medical Co., Bucheon, Korea) were used for SBV. Also MGP (Korean Pharmacopuncture Institute, Seoul, Korea) was injected intravenously in a dose of 20 mL every two weeks using syringes (23 G × 25 mm, 20 mL, CPL Co., Ansan, Korea). We performed no treatments other than the treatment using SBV and MGP.
From March 2010 to April 2014, her tumors almost stopped growing and showed only a few changes. Also, the range of motion in her right hip joint and her general condition improved. magnetic resonance imaging (MRI) in April 2014 to check if bones or organs had been affected showed that the fibromatosis remained subcutaneous and that no bones or organs had been affected (Fig ).


A 38-year-old woman was admitted due to claudication of the legs that had been sustained for 2 years. Eight months ago, she was diagnosed as having Takayasu arteritis during the work ups for chronic headache in primary clinic. Immunosuppressive medication with methotrexate was followed due to glucocorticoid resistance. Two months ago, embolic infarction of the kidney and the spleen resulted in anticoagulation therapy with warfarin. Her past medical history included hypertension requiring medication 10 years ago and a surgical history of appendectomy 9 years ago. On admission, physical examination revealed that the pulse over the right femoral area was absent. Asymmetrical systolic blood pressure of 142 mmHg in the arms and 88 in the right legs and 94 mmHg in the left leg was found. Thus, ankle-brachial index was 0.62 in right and 0.66 in left. The findings obtained from a central nervous system examination were unremarkable. The patient’s C-reactive protein level and erythrocyte sedimentation rate were within normal range. Three-dimensional computed tomography (CT) revealed multifocal severe luminal narrowing of descending thoracic and abdominal aorta (). In addition, significant stenosis of the right carotid artery, brachiocephalic trunk, proximal celiac trunk ostium and total occlusion at left subclavian artery ostium with collateral development were observed. Whole body positron emission tomography-CT revealed no hypermetabolic lesions in the arterial system indicating that the vasculitis was not active. Cardiac evaluations including echocardiography and coronary angiography revealed normal cardiac function (left ventricular ejection fraction, 60%) without any stenotic lesions in coronary arteries. In order to relieve ischemic symptoms of the lower extremities, the patient underwent axillo-femoral bypass surgery in the right side using a 10 mm-ringed polytetrafluoroethylene graft (). After the surgery, the difference in arterial blood pressure between the upper and lower extremities disappeared, and the symptom of claudication was relieved. Anticoagulation therapy was started on the first postoperative day and continued once daily.
Two years later, she was re-admitted with recurred claudication in the lower extremities. Systolic blood pressure was 136 mmHg and 74 mmHg in the arms and in the legs, respectively. CT angiography revealed complete occlusion of the axillo-femoral bypass graft. At this point, extra-anatomic ascending aorta-to-infra-renal aorta bypass surgery was planned to restitute reliable blood supply in the lower extremities.
At operation, a median upper hemisternotomy down to the level of 4th intercostal space through a limited skin incision and an additional small midline laparotomy (6 cm) around the umbilicus level were made. Through the sternotomy incision, the ascending aorta was exposed through pericardial incision and tenting, and the infra-renal abdominal aorta was exposed through the laparotomy facilitated by Alexis wound retractor (Applied Medical, Rancho Santa Margarita, CA, USA). In order to create a route for the passage of a prosthetic graft, a small incision (3–4 cm) was made in the mid-anterior portion of the diaphragm, the lesser omentum was cut, and a hole was made in the transverse mesocolon. An 18 mm-Gelweave graft (Vascutek Terumo, Glasgow, Scotland) was chosen for the bypass conduit. After systemic heparinization, graft was anastomosed proximally to the mid ascending aorta in an end-to-side fashion after partial aortic clamping. Then the graft was passed through the route made previously, and was anastomosed to the infrarenal abdominal aorta in the same fashion ().
Postoperatively, vital signs were stable and the arterial blood pressure in the upper and lower extremities equalized. The patient was transferred from intensive care unit to general word at postoperative day 1 and was discharged without complication at 8 days. During her 5 months of follow-up, the patient remained completely free of limb claudication with excellent exertional tolerance. Postoperative CT showed excellent graft configuration without architectural distortion (). Then the patient was given warfarin for 2 months with continued immunosuppressive medication.


A 19-year-old male was found unresponsive following a 40-foot fall while under the influence of alcohol and psilocybin mushrooms. On arrival to the hospital, he was hemodynamically stable with patent airway. He had a Glascow Coma Scale of 8 (M5V1E2), a soft abdomen and no obvious signs of trauma other than a gross deformity of his right ankle. Plain radiographic imaging of the chest was normal whereas pelvic and extremity X-rays revealed fractures to the pubic rami, sacrum, right tibia and right fibula. Axial imaging revealed bilateral occult pneumothoraces with bilateral pulmonary contusions, low-grade splenic and liver lacerations, a right superior renal hematoma and numerous vertebral body and transverse process fractures. Additionally, there was a trace amount of pneumodediastinum and pneumopericardium [].
The patient was admitted to the Surgical Intensive Care Unit. His ankle was reduced and externally fixed 2 days after admission. Because of persistent tachycardia that did not respond to fluid resuscitation, computed tomography (CT) angiography of his chest, abdomen and pelvis was obtained that was negative for pulmonary embolus or signs of active hemorrhage. The following week, his tachycardia improved to a resting heart rate in the 100-110s. He was discharged to inpatient rehabilitation on hospital Day 8 in stable condition.
Four days following arrival to rehabilitation, he began experiencing left shoulder and chest pain exacerbated by lying supine and alleviated when leaning forward. He was pale with a resting heart rate of 106 and a blood pressure of 98/48. Physical examination revealed a pericardial friction rub. His ECG was normal with no ST changes. CT scan of the chest revealed increased pneumopericardium []. Transthoracic echocardiogram (TTE) showed a small pericardial effusion with no evidence of tamponade.
Treatment was initiated for pericarditis with intravenous ketorolac followed by a 2-week course of ibuprofen and a 3-month course of colchicine. The patient's chest pain and pericardial friction rub resolved and he was discharged to rehabilitation 4 days later.
Three days later, he awoke with diaphoresis, chest pain, left flank and back pain and shortness of breath and was again transferred to the emergency department. On arrival, he was tachycardic to the 120s and hypotensive with a blood pressure of 88/56. Physical exam revealed muffled heart sounds and increased jugular venous distension. TTE showed a 2 cm collection of fluid in the pericardial space and right atrial collapse during diastole. ECG revealed T-wave inversions consistent with a diagnosis of pericardial tamponade with cardiogenic shock. He was transferred to the cardiac catheterization laboratory where he underwent pericardiocentesis and drainage of 550 cc of sanginous fluid. His symptoms and vitals quickly normalized.
The pericardial drain was removed after 48 h when a repeat TTE showed resolution of effusion. He underwent open reduction and internal fixation of his right leg with the orthopedic team. On hospital Day 13, he was discharged back to rehabilitation on a 6-month course of 0.6 mg colchicine BID and 50 mg indomethacin TID. He recovered well at rehab with no further events and was discharged to home 8 days later.


A 28-year-old female patient presented with a hypoechogenic hepatic nodule
incidentally found at routine ultrasonography (US). Further investigation with
magnetic resonance imaging (MRI) identified other predominantly peripheral hepatic
nodules varying in size, with low signal intensity on T1-weighted sequences and
intermediate signal intensity on T2-weighted sequences, and a hypovascular,
"bull-eye" enhancement pattern ().
Additionally, other hepatic nodules ()
as well as pulmonary nodules and lytic lesions in the left iliac bone marrow were
characterized at computed tomography (CT) (). Tumor markers, colonoscopy and endoscopy were negative for neoplastic
lesions. The diagnosis of epithelioid hemangioendothelioma was confirmed by
US-guided biopsy whose biopsy slide is illustrated on .


A 52-year-old male patient had been diagnosed with liver cirrhosis due to HBV infection in 1980 and had been without regular follow-up due to lack of desire on his part. In 2008, he was diagnosed with three nodules of HCC in S4 and S5, the largest being 35 mm in diameter (fig. ). Two months after these findings, the patient had rupture of the HCC nodule in S5 and received urgent TAE for hemostasis (fig. ). He underwent LDLT 3 months after the episode of ruptured HCC (fig. ), with a Child-Turcotte-Pugh (CTP) class of C (10 points), a model of end-stage liver disease (MELD) score of 10 points, and also had HCC within the Milan criteria (fig. ). The tumor marker levels before transplantation showed an increase in the PIVKA II level up to 423 mAU/ml, with a normal AFP level of 7.5 ng/dl. The patient was also treated following the general clinical and immunosuppressive protocol.
Peritoneal dissemination was diagnosed in this patient 32 months after LDLT (fig. ). He started to receive adjuvant therapy with sorafenib 400 mg without evident benefit, then after 1 month of this treatment was changed for to tegafur and uracil 400 mg, but after another 1 month this was also stopped due to lack of efficacy. After 6 months, the patient developed strong intra-abdominal pain, probably connected with the dissemination of the peritoneal tumor, and received palliative therapy. He died due to the progression of disease 70 months after transplantation. Maintenance immunosuppressive therapy was under tacrolimus with a trough level of 3–5 ng/μl without any signs of rejection. This patient was the only one who had ruptured HCC before LDLT in our series.


A 62-year-old male patient with liver cirrhosis due to HCV infection since 1993 was found to have HCC in 2003 and underwent caudate lobe resection. After surgery, he received antiviral therapy, with a sustained virological response to antiviral therapy by interferon plus ribavirin. Four months after the liver resection the patient was found to have HCC recurrence in the liver, and during the next 3 years, he consequentially received several TAE/TACE procedures for HCC in S2, S4 and S7, percutaneous ethanol injection for HCC in S7 and RFA for HCC in S2, with good clinical and roentgenological outcomes.
Thirty-eight months after the initial finding of HCC, the patient underwent LDLT from his daughter, using a left lobe graft, and his treatment followed the general clinical protocol of the center, including immunosuppressive therapy. A few days before transplantation, the patient's prognostic criteria presented with CTP class C (10 points), a MELD score of 10 points and HCC within the Milan criteria. The tumor marker levels were normal: AFP 1.9 ng/dl and PIVKA II 24 mAU/ml. Histopathological study of the explanted liver showed no evidence of malignancy.
However, with elevation of AFP 19 months after LT, a single nodule of peritoneal tumor in the pelvic cavity was observed under PET/CT. Within 1 month the patient underwent peritoneal tumor resection, and histopathological investigation of the tumor revealed moderately differentiated HCC. After 3 months, the patient was accepted for adjuvant therapy with sorafenib 400 mg, but at the same time, he was observed to have recurrence of the pelvic tumor, with invasion in the rectum, and it was noted that he also had liver recurrence. TACE was performed for both the pelvis and liver tumors, and thereafter the patient was supported with palliative treatment. After about 1 month of this treatment, the patient asked to be discharged so he could go home, where he died within 3 months. Overall, the patient survived 46 months after LT. Maintenance immunosuppressive therapy was under cyclosporine with the trough level of 50–100 ng/μl without any signs of rejection. This case was partly reported in our previous publication [].


A 75-year-old male was referred to our unit with unsteadiness, falls, and slurred speech. His past medical history included a hormone escaped prostate adenocarcinoma with known bony metastases to the ribs and pelvis as well as intrabdominal lymph node involvement. He was originally managed with single androgen suppression therapy (LHRH analog) and external beam radiotherapy for the primary disease but not for the bony metastases. At the time of presentation no calvarian or intracranial disease was present. His Eastern Cooperative Oncology Group (ECOG) performance status was 2.
Computed tomography (CT) head performed at the referring hospital revealed bilateral chronic subdural collections, larger on left side, with 18 mm midline shift to the right. These had the typical radiological appearance of liquefied hematomas [].
On admission his Glasgow Coma Scale (GCS) was 15/15 with no lateralizing neurology except mild expressive dysphasia. He was not taking any antiplatelet or anticoagulant medication and his hematological profile revealed unimpaired coagulation and normal biochemical profile.
The patient underwent evacuation of his left sided subdural collection (the prominently symptomatic side) via two burr holes under general anesthetic. The operating surgeon described the procedure uneventful, the subdural collection as “typical light brown color,” under high pressure. A Jacques catheter was left in the subdural space for 48 h, which is standard practice in our unit. Postoperatively the patient improved only marginally and the fifth postoperative day, it was clear that he was again expressively dysphasic. Repeat CT of his head revealed re-accumulation of the subdural collection, with moderate mass effect []. He returned to theatre where again the left sided subdural collection was re-opened, found to be under high pressure, re-drained and send for cytology. In addition a specimen of dura was biopsied.
Histopathology revealed connective tissue containing occasional small, cohesive epithelial cell lobules. The cells had pale cytoplasm and round or oval nuclei with medium-sized nucleoli. Immunohistochemistry revealed positive staining for AE1/AE3 (cytokeratin cocktail), prostate specific antigen and prostatic specific acid phosphatase, but not for CK7, CK20, and TTF-1. All these findings were consistent with a metastatic prostate adenocarcinoma with similar histopathological features with the original biopsy. Cytology did not reveal any malignant cells.
The patient had a slow and fluctuating neurological recovery. Further CT of his head revealed re-accumulation of the subdural fluid []. He was discussed in the regional neuro-oncology multidisciplinary meeting (MDM) and it was decided to be refereed for whole brain radiotherapy (WBR). Four weeks later, and while still inpatient, he received 20 Gy in 4 fractions of 5 Gy, delivered in five consecutive days. Despite WBR, the patients ECOG performance status deteriorated from 2 to 3. He was discussed again in the neuro-oncology MDT meeting and the decision was made for best supportive treatment and palliative care. After a total inpatient stay of 5 weeks, he was discharged to a hospice for palliative care management where he succumbed to his disease one month later.


A 35-year-old lady presented with a rapidly growing mass (6 cm × 5 cm) near right angle of mandible. Clinically, the mass was firm, angry-looking, with surface ulcerations. There was no fixity to deeper structures or any lymphadenopathy. Clinically, it appeared as malignant parotid tumor []. Routine investigations were within normal limits.
Fine-needle aspiration cytology smears showed epithelioid cells arranged discretely with high N:C ratio, prominent single to multiple eosinophilic nucleoli and pseudoinclusions. Occasional binucleated and multinucleated tumor giant cells were seen. Few cells had eccentric nuclei [ and ]. Features were suggestive of an undifferentiated malignant neoplasm. Epithelioid melanoma (amelanotic), poorly differentiated carcinoma, high grade NHL, epithelioid sarcoma were kept in differential diagnoses. Immunocytochemistry (ICC) was advised.
On ICC, some tumor cells were positive for Melan-A/MART-1 [] while most were positive for S-100; but pancytokeratin, leukocyte common antigen, and desmin were negative. Based on these findings, cytodiagnosis of epithelioid variant of malignant melanoma (amelanotic) was rendered.
Subsequently, surgical resection of the mass was done and sent for histopathology. Grossly, the mass was globular, measuring 5.3 cm × 4.4 cm and attached to the skin. Adjoining parotid gland was free. Cut surface was whitish solid, with spotty areas of hemorrhage. Microscopic examination showed a tumor arising from the epidermis and was composed of the epithelioid type of malignant cells devoid of melanin pigment in sheets and alveolar pattern at some places. These cells had high N:C ratio and large single or more eosinophilic nucleoli. A few binucleated and occasional multinucleated cells were also noted [ and ]. The mitotic count was high. The tumor exhibited junctional activity. Focal areas of necrosis and chronic inflammatory cell infiltrate were seen. Immunohistochemistry (IHC) revealed Melan-A and S-100 positivity in tumor cells. A final diagnosis of amelanotic melanoma (epithelioid), Clark's level V was established [ and ]. This corroborated with previous ICC findings.


In February 2011, this 26-year-old Ethiopian male awoke in the morning with bilateral paralysis of his extremities. When he presented to the emergency department 4 h later he exhibited partial recovery. He had no associated swallowing or breathing difficulty, weakness of the facial muscles, sphincter disturbances, pain, sensory symptoms, or change in mental state. Two months earlier, he had undergone a similar episode, beginning after midnight, which resolved spontaneously within 3 h. He had been experiencing heart palpitations for several months, along with heat intolerance and failure to gain weight despite a good appetite. He had no other known prior medical or surgical illnesses. He denied alcohol abuse or illicit drug use and was not on any medications. No similar weakness or other remarkable diseases in the family was reported.
At presentation, his blood pressure was 120/80 mmHg and his heart rate was 114 beats/min. He had no thyromegaly, and his skin was warm and dry. He was fully oriented and cooperative. No gross cranial nerve deficits were noted. He demonstrated flaccid symmetrical proximal and distal weakness of the arms and legs (power: legs 3/5, arms 4/5). Knee and ankle deep tendon reflexes were depressed bilaterally (grade 1/4). Sensation was intact.
Blood tests showed serum K+, 2.7 mmol/L (normal range: 3.6–5.5); Mg2+, 1.8 mg/dL (normal range: 1.9–2.5); and creatine kinase-total, 223 IU/L (normal range: 38–174). Serum phosphate was not determined, but results of the remainder of the electrolyte panel, complete blood count, and blood chemistry were normal. Thyroid-stimulating hormone (TSH) was less than 0.0005 IU/mL (normal range: 0.27–4.2), free triiodothyronine (T3) was 17.72 pg/dL (normal range: 2.02–4.43), and free thyroxine (T4) was 5.33 ng/dL (normal range: 0.93–1.71). The ECG showed sinus tachycardia at 120 beats/min, U waves fused with P waves, and inferior T-wave inversions (Fig.). Results of an electromyography and a nerve conduction study conducted the following day were within normal limits. Because of logistical constraints, a thyroid radioiodine uptake scan could not be done until ≈6 weeks later, during which time the patient had been receiving antithyroid medication. The results were reported normal.


A 20-year-old male patient presented to our outpatient services with 1-month history of low backache with radiation to both lower limbs and progressive paraparesis. Patient had developed urinary and fecal incontinence from last 15 days. History revealed that he was operated for right insular glioma in March, 2010. Gross total excision of the tumor was carried out at that time via a right fronto-temporal craniotomy and trans-sylvian, trans-insular approach. Histo-pathological examination was suggestive of anaplastic astrocytoma World Health Organizations (WHO) Grade III. Patient received postoperative radiotherapy and chemotherapy including Temozolomide. Patient was functionally preserved since then.
Neurological examination revealed a paraparesis with a power of 2/5 (Medical Research Council grading). Patient had sensory impairment to all modalities of sensation below L2 level. Gadolinium – enhanced magnetic resonance imaging (MRI) of the spine revealed dual, well defined, intra-dural extramedullary drop metastases at D11-L2 and S1-S2 levels. They were hypointense on T1weighted, hyperintense on T2-weighted images with intense, heterogenous postcontrast enhancement []. MRI brain with contrast did not reveal any evidence of residual/recurrent tumor.
In view of the diffuse nature of the disease, it was decided to surgically decompress the symptomatic lesion at D11-L2 level. D10-L2 laminoplasty and tumor decompression was carried out. Intra-operative findings revealed that the tumor was intra-dural, extramedullary with infiltration of the cord tissue. It was heterogeneous, moderately vascular and suckable. However, there was no definite plane between the tumor and the cord tissue. Maximum safe decompression was carried out. Patient had an uneventful postoperative course. Power in both lower limbs improved to 3/5 at the time of discharge.
Histo-pathological examination (H and E, ×100) [] showed a highly cellular astrocytic tumor with frequent mitosis, necrosis and hyperchromatic pleomorphic tumor cells. Endothelial cell proliferation and necrosis were also noted. Immunohistochemical staining showed positivity for P53 and positivity for glial fibrillary acidic protein. Tumor cells were negative for epidermal growth factor receptor, and MIB 1 labeling index was 55% in highest mitotic areas. Based on these findings, diagnosis of GBM (WHO Grade IV) was rendered. Patient was referred to the radiotherapy department for further management.


A 65-year-old man presented to the emergency department complaining of feeling unwell, chest pain during exercise, nausea and vomiting for a few days ago. He had recurrent episodes of apnea, palpitation and dizziness since the night before. A review of his past medical history revealed that he was a previous intravenous heroin user who was attending a community methadone substitution program. He was prescribed syrup methadone 240 mg daily. He was not known to have cardiac, respiratory, hepatic or neurological disease. Physical examination was remarkable for normal mental status, blood pressure of 140/90 mmHg, pulse of 50 beats/min in sinus rhythm, O2 saturation: 90%, no cardiac murmurs, and no signs of heart failure. Laboratory studies included potassium of 2 mmol/l and magnesium of 1.4 mmol/l, white blood cell: 12,000/ mm3, hemoglobin: 15 g/dl, platelet: 222,000/mm3, blood sugar: 122 mg/dl. We though his hypokalemia was due to prolonged vomiting. His electrocardiogram (ECG) demonstrated sinus bradycardia and a QTc of 550 ms ().
The patient was admitted to the cardiology service for ECG monitoring. Shortly thereafter, the patient experienced an episode of TdP and ventricular fibrillation ().
Cardiopulmonary resuscitation, defibrillation and Lidocaine administration resulted in a successful return of spontaneous circulation. Lidocaine was an antiarrhythmic agent of choice because it does not prolong QTc anymore in contrast to other antiarrhythmic agents. Potassium and magnesium were administered intravenously to correct hypokalemia and hypomagnesemia. Due to the recurrent episode of TdP temporary pacemaker was inserted, and it was set at rate of 100-120 beats/min to prevent bradycardia. Temporary pace maker (TPM) was removed after 48 h. Methadone was discontinued and substituted with buprenorphine. Over the following 7 days, QTc interval progressively returned to normal limits. Discharge serum potassium and magnesium were 4.5 mmol/l and 2.5 mg/dl respectively. The patient was discharged with buprenorphine and a follow-up at the cardiology clinic, 2 weeks and 1 month later revealed no further cardiac symptoms with a normal ECG. He did not have any electrolyte abnormality at follow-up.


A 21-year-old male presented with a history of swelling, which was gradually increasing since 2 years, in the left preauricular region, inferior to the lobule of the ear, and associated with dull intermittent nonradiating type of pain with no aggravating and relieving factors. The pain increased in intensity on turning his head to the right side. He did not complain of dyspnea or dysphagia.
On examination, fullness was seen in the parotid and retromandibular regions on the ipsilateral side with raised ear lobe. The swelling measured about 6 cm × 4 cm []. Margins of the swelling were diffused, extending up to 1 cm superior to the ear lobe, 3 cm inferior to the inferior border of the mandible, anteriorly up to the level of the body of the mandible and posteriorly to the mastoid region.
Neck movements were unrestricted. Skin over the swelling was pinchable, with no local rise in temperature, but mildly tender on palpation. Mouth opening was unrestricted. Oropharyngeal examination did not reveal any abnormalities. His general physical examination and medical history did not show the presence of any co-morbid conditions. Routine laboratory tests were within the normal limits. A computed tomography (CT) scan of the parotid region with intravenous contrast was performed, which showed a hypodense fat-attenuated lesion with linear strands measuring 52 mm × 37 mm in the retromandibular region deep to the sternocleidomastoid muscle, inferior to the parotid gland extending to the submandibular region. Medially the carotid vessels, supero-laterally the parotid gland and antero-superiorly the submandibular gland were displaced. Based on the above findings, the lesion was provisionally diagnosed as lipoma in the retromandibular region []. The patient was planned for excision of the lesion under general anesthesia.
A Risdon's incision extending up to the retromandibular region [] was placed and a subplatysmal dissection was performed to approach the mass. Intraoperatively, the extent of the swelling confirmed the CT findings. The dissection was carried out to expose the sternocleidomastoid muscle, which was retracted laterally as the lesion was found beneath the muscle. The inferior aspect of the lesion was found to be in close proximity to the external carotid artery. Hence, careful dissection was performed to separate it from the vessel to prevent any injury to the same []. Because the lesion was well-encapsulated, blunt dissection was performed to free its margin and the mass was excised in toto. Hemostasis was achieved in the field. The excised mass [] was soft, yellow, smooth, shiny and ovoid, measuring about 5 cm × 4 cm, which was sent for histopathological examination. A suction drain was placed and the site was closed in layers. The biopsy report revealed lobular growth of large mature fat cells with clear cytoplasm and thin strands of connective tissue, scanty inflammatory cells and few blood vessels []. The above findings confirmed it as lipoma.
Postoperatively, symmetry of the face could be appreciated [] without any facial nerve palsy and other complications. The patient was discharged 1 week postoperatively. He is on follow-up for the last 2 years with no evidence of recurrence till date.


A 68-year-old male presented for progressive loss of appetite and lethargy for 3 weeks. He also developed persistent low back pain in the last 2 months. The past medical history included hypertension, hyperlipidemia, coronary artery disease with multiple percutaneous coronary interventions (the most recent one was 3 weeks prior to the admission), congestive heart failure with automated implantable cardioverter-defibrillator placement, and psoriasis diagnosed 5 years ago. The patient had a history of smoking one pack of cigarettes per day for 40 years. The vital signs were within normal range; he was lethargic and dehydrated. There were multiple large erythematous plaques of skin in the bilateral inguinal and axillary and right hip areas weeping serous discharge (fig. ).
The laboratory tests showed normal hemoglobin initially, which dropped to 8.5 within 1 month after admission, hypercalcemia, M spike, and elevated β2-microalbumin and IgG levels (table ).
The CT scan of the head demonstrated multiple ‘punched out’ lucencies within the skull (fig. ). Bone survey revealed innumerable lytic lesions throughout the visualized bones: cranial vault (fig. ), proximal shaft of femur bilaterally, hips, and pelvic bones (fig. ). Bone marrow core biopsy showed increased plasma cells up to 10%, confirmed on CD138, with predominance of monoclonal lambda. Skin biopsy revealed epidermal spongiotic dermatitis with acanthosis and parakeratosis, the upper dermis with perivascular lymphocytic exocytosis and rare eosinophils. In addition, the patient had eosinophilia and elevated IgE (table ); atopic eczema was the most likely diagnosis.


The patient was a 60-year-old female in whom a tumor of 15 mm in diameter was detected in the Couinaud's segment (S6) of the liver on the grayscale US in a medical examination. She visited our hospital for further examination and treatment. She had no subjective symptoms, relevant medical or family history, and did not drink alcohol. Physical findings on admission were: blood pressure 136/80 mm Hg, pulse rate 80/min, and body temperature 36.4°C. Blood tests on admission showed Hb 10.4 g/dl (normal 14.0–17.0 g/dl), suggesting a mild anemia. Tumor markers were normal, tests for hepatitis B virus (HBV) and hepatitis C virus (HCV) were negative, and there were no other abnormal findings.
Grayscale US showed a tumor with a snowman-like appearance and a relatively clear boundary in the S6 of the liver, with hypo- and hyperechoic areas in the lateral and medial parts of the lesion, respectively (fig. ). On dynamic CT, the tumor was shown as a single lesion protruding from the S6. The tumor had a pale, low-density area on unenhanced CT, and prolonged enhancement in the equilibrium phases (fig. ). On MRI, the whole lesion gave a low-intensity signal on T1-weighted imaging, but isointensity in the lateral part and high intensity in the medial part were seen on T2-weighted imaging. Similarly, the lateral part showed high intensity and the medial part had a higher intensity on heavy T2-weighted imaging (fig. ). On dynamic MRI, the lateral part was enhanced in the arterial phase, and enhancement persisted in the portal phase. In contrast, the medial part was gradually enhanced in the arterial phase, compared to the portal phase.
CEUS was performed using an Aplio XG (Toshiba Medical Systems, Tokyo, Japan) with a convex probe (PVT-375BT, 3.75 MHz frequency). The mechanical index for the acoustic output was set to 0.2, and a single focus point was set at the lower margin of the lesion. Sonazoid (0.5 ml) was injected into the left cubital vein followed by a flushing with 10 ml of normal saline. The lateral hypoechoic region was homogenously hyperenhanced in the vascular phase (0–40 s) early after injection, and the contrast medium was washed out after about 30 s. The medial hyperechoic region was gradually stained from the margin toward the central region (fig. ). The tumor showed a defect in both hypo- and hyperechoic regions in the post-vascular phase (after 15 min). Similar findings were observed after a second intravenous injection of 0.5 ml of sonazoid using defect reperfusion imaging [] in the postvascular phase. The imaging findings suggested that 2 contiguous tumors were present. Liver hemangioma was suspected for the medial part of the lesion based on the typical contrast findings on MRI and CEUS. In the lateral part, the contrast medium was washed out in the vascular phase on CEUS early after the intravenous injection of sonazoid. Furthermore, the lateral part showed a defect in the postvascular phase, and this defect led to the suspicion of a malignant tumor, including hepatocellular carcinoma (HCC). Thus, surgical resection was performed.
In a macroscopic examination of the resected specimen, the medial part was whitish and the lateral part was yellowish-white. On hematoxylin and eosin (HE) staining, the medial part comprised blood vessels formed by a single layer of flattened endothelial cells and an interstitium formed by thin connective tissue, with the vascular lumen filled with blood. Based on these findings, the medial part of the tumor was diagnosed as hemangioma. In the lateral part, lymphocyte infiltration in a dense arrangement was observed on HE staining, and most lymphocytes contained a moderately sized nucleus, but some cells contained a large nucleus and noticeable nucleolus (fig. ). On immunohistochemical staining, CD30-expressing lymphocytes containing a large nucleus were occasionally noted. B lymphocytes expressing CD20 and CD79α were present (fig. ), but bcl-2 expression in the germinal center was unclear. Lymphocytes expressing CD43 were more common than those expressing CD3. Similar findings were present in the germinal center, with atypical lymphocytes invading the germinal center. Based on these findings, the lateral part of the tumor was diagnosed as marginal zone B-cell lymphoma. The patient was discharged 5 days after surgery.


A 50-year-old man presented with difficulty in swallowing and nasal speech. First he had difficulty swallowing solids, which later progressed to ingestion of liquids.
He mentioned three episodes of nasal regurgitation of liquids. His consort noticed incomplete closing of his eyes during sleep.
The muscles of jaw closure were involved and produced weakness with a prolonged meal time (fatigable chewing). For evaluation of dysphagia, he was referred to an internist for a barium swallow and video fluoroscopy was performed. Benign narrowing of the hypopharynx and pharyngoesophageal junction was detected. He was advised follow-up and was not offered any treatment for stenosis.
The symptom of the patient progressed and he was referred to a neurologist. The neurologist recommended magnetic resonance imaging (MRI) of the brain, an electrophysiological test, and prescribed an acetylcholinesterase inhibitor (AChEi), pyridostigmine 60 mg twice a day. The brain MRI was normal and the electrophysiological findings were reported as a bulbar onset of amyotrophic lateral sclerosis. The acetylcholine receptor antibody titer was normal and in a pharmacological testing with neostigmine no improvement in the bulbar symptom was seen.
After three months, gait disturbance and generalized weakness was added to his symptoms and low dose pyridostigmine was continued due to lack of response.
Several months after the start of the symptoms, he was urgently admitted to the Emergency Department of our University Hospital because of respiratory failure and intubated. In the first neurological examination hyperreflexia was detected and in the light of his previous history, motor neuron disease was suspected and supportive care and neuroprotective drugs were started. The patient was referred to the Internal Medicine Service with an impression of aspiration pneumonia. A few days after admission, a neuromuscular consultation and electrodiagnosis was requested. On the first neuromuscular visit, the patient was intubated and mechanically ventilated. He was extremely cachectic due to several months of dysphagia, and profoundly ill on account of the intercurrent infection. Hyperreflexia in the upper and lower limbs was prominent. Nerve conduction studies (NCS) and electromyography (EMG) of the limbs was normal and no evidence of motor neuron disease (MND) was seen. A repetitive nerve stimulation test revealed a significant decremental response in the proximal and facial muscles, with post activation facilitation and exhaustion (23% decremental response in the trapezius muscle and 32% in the nasalis muscles) [Figures and ]. Short-duration polyphasic motor unit action potentials (MUAPs) were detected in the bulbar and proximal muscles, without any neurogenic finding. The electrical findings were consistent with a neuromuscular junction defect. The patient was transmitted to the Neuromuscular Service and treated with plasma exchange, pyridostigmine, and prednisone.
The patient recovered dramatically and the bulbar symptoms and limb weakness improved rapidly. He was extubated in a few days and discharged with significant improvement in his functional state. In a follow-up visit there was no evidence of respiratory or bulbar weakness. Ptosis and facial weakness were the prominent signs and the Cogan and peek signs were positive. The acetyl choline receptor antibody and anti-MuSK (muscle-specific Kinase) antibody titers were normal. A chest CT scan revealed an anterior mediastinal mass [] and the patient was referred for a thymectomy. Thymoma type B2 with capsular invasion was detected in the pathological specimen.