We described a case of well differentiated papillary mesothelioma (WDPM) of the peritoneum in a 68-year-old man because of its rarity. It was incidentally found during operation for renal cell carcinoma. Grossly, the tumor appeared as multiple small granules or nodules on the entire peritoneal surface, including serosa of the intestine, omentum and mesentery. Microscopically, characteristic features are papillary, tubulopapillary or solid architecture lined by single layer of uniform, cytologically bland, cuboidal or polygonal mesothelial cells. The tumor cells were positive for EMA, cytokeratin and vimentin, but negative for CEA on immunohistochemical staining. Ultrastructural study showed characteristic long slender microvilli on the surface and intercellular junctions.
Male ; Humans

Chronic active Epstein-Barr virus infection(CAEBV) is a nonfamilial syndrome that shows a specific immunodeficiency for the Epstein-Barr virus(EBV). CAEBV is characterized by fever, lymphadenopathy, splenomegaly, hepatitis, interstitial pneumonitis, interstitial nephritis, and uveitis. Cardiovascular complications are rare in EBV infection. Patients with CAEBV show characteristically high titers of anti-viral capsid antigen(VCA) IgG antibody and anti-early antigen(EA) antibody, as well as relatively low titer of anti-EB nuclear antigen(EBNA) antibody. We experienced a case of CAEBV with giant coronary aneurysms, who was a 6-year-old boy. He had 5 episodes of high fever and cervical lymphadenopathy and hepatosplenomegaly. The 6 mm sized bilateral coronary aneurysms were detected by echocardiography at second admission. IgG antibodies to EBV was positive, whereas negative for IgM antibody. Antibodies to EA and EBNA were also positive. The EBV was detected in lymph node tissue by in situ hybridization, and in the peripheral blood and bone marrow by the PCR. Treatment has been done with ganciclovir and interferon (IFN)-alpa for 5 weeks. The EBV-PCR of peripheral blood was converted to negative from 12th day of treatment. At present he has followed well for two years without fever and enlargements of lymphnode, except large coronary aneurysm.
Antibodies ; Bone Marrow ; Capsid ; Child ; Coronary Aneurysm* ; Echocardiography ; Epstein-Barr Virus Infections ; Fever ; Ganciclovir ; Hepatitis ; Herpesvirus 4, Human* ; Humans ; Immunoglobulin G ; Immunoglobulin M ; In Situ Hybridization ; Interferons ; Lung Diseases, Interstitial ; Lymph Nodes ; Lymphatic Diseases ; Male ; Nephritis, Interstitial ; Polymerase Chain Reaction ; Splenomegaly ; Uveitis

Congenital lobar emphysema is a rare disease and a cause of respiratory distress in early infancy. The most common location of pulmonary involvement is the left upper lobe. We diagnosed a case of congenital lobar emphysema in a 5-month-old female infant, who had a complex heart disease using flexible bronchoscopy. In spite of palliative heart surgery, respiratory difficulty and cyanosis did not improve and weaning from artificial ventilation was not possible. Chest X-ray and CT scan showed persistent hyperinflation in the left upper lobe. So, we performed flexible bronchoscopy. The findings showed no cartilage and patent bronchus during inspiration, but complete collapse during expiration due to a check valve effect in the left upper lobar bronchus. After excision of the involved lobe, the patient improved from respiratory distress. The observed airway patency during inspiration, and dynamic airway collapse on expiration suggest that bronchomalacia contributed to lung overinflation in this case.
Bronchi ; Bronchomalacia ; Bronchoscopy ; Cartilage ; Cyanosis ; Emphysema* ; Female ; Heart Diseases ; Humans ; Infant ; Lung ; Rare Diseases ; Thoracic Surgery ; Thorax ; Tomography, X-Ray Computed ; Ventilation ; Weaning

Heterotopic pancreas can be defined as the occurrence of pancreatic tissue at aberrant anatomic sites that lack vascular, neural and anatomic community with the pancreas. A minority of heterotopic pancreas elicit clinical signs or symptoms that can vary according to its location and size, and the involvement of the overlying mucosa. We report here on a case of heterotopic pancreas that was removed by pancreatoduodenectomy under the erroneous diagnosis of distal common bile duct cancer.
Ampulla of Vater* ; Common Bile Duct* ; Diagnosis ; Jaundice ; Mucous Membrane ; Pancreas* ; Pancreaticoduodenectomy

We report a 52-yr-old Korean woman with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) whose diagnosis was confirmed by skin biopsy and the presence of a novel mutation in the NOTCH3 gene. The patient's clinical features were rather unusual in that 1) clinical presentations were only two episodes of stroke and mild dementia unaccompanied by mood disturbances or migraine, and 2) there was no family history. Brain MRI showed T2 hyperintensities in both temporal pole areas in line with the recent suggestion by O'Sullivan et al. that the abnormality could be a radiologic marker of CADASIL. FDG-PET also showed a hypometabolism in the temporal pole areas with an abnormal finding on MRI in addition to the hypometabolism in cortical and subcortical regions. We could learn from this case that CADASIL may be included in the differential diagnoses in patients with vascular dementia associated with a small vessel disease, even in the absence of a family history, especially when there are no known stroke risk factors and when the MRI shows T2 hyperintensity in the temporal pole regions.
Amino Acid Substitution ; Biopsy ; Brain/pathology ; Codon/genetics ; Dementia, Multi-Infarct/diagnosis ; Dementia, Multi-Infarct/genetics* ; Dementia, Multi-Infarct/pathology ; Dementia, Multi-Infarct/radionuclide imaging ; Female ; Human ; Korea ; Magnetic Resonance Imaging ; Middle Aged ; Mutation, Missense* ; Neuropsychological Tests ; Point Mutation* ; Proto-Oncogene Proteins/genetics* ; Skin/pathology ; Tomography, Emission-Computed

Up to 40% of patients with anti-glomerular basement membrane (GBM) disease, which is a rare autoimmune disorder usually manifesting as rapidly progressive glomerulonephritis (RPGN), are positive for circulating anti-neutrophil cytoplasmic antibody (ANCA). Many previous reports showed poor outcomes in these "double-positive" patients. We report a patient with perinuclear (p)-ANCA positive anti-GBM disease who presented with RPGN and required hemodialysis. Plasmapheresis and steroid and cyclophosphamide therapy were initiated following renal biopsy and resulted in normalization of anti-GBM antibody and p-ANCA titers, recovery of renal function, and discontinuation of hemodialysis. This case suggests that aggressive immunosuppression with plasmapheresis in patients who are p-ANCA positive with anti-GBM disease should be considered, even in those with severe renal dysfunction.
Anti-Glomerular Basement Membrane Disease ; Antibodies, Antineutrophil Cytoplasmic ; Autoantibodies ; Basement Membrane ; Biopsy ; Cyclophosphamide ; Glomerulonephritis ; Hemorrhage ; Humans ; Immunosuppression ; Lung Diseases ; Plasmapheresis ; Renal Dialysis

Peripheral nerve tumors are mostly benign and can arise on any nerve trunk or twig. Although peripheral nerve tumors can occur anywhere in the body, including the spinal roots and cauda equina, many cases are subcutaneous in location and present as a soft swelling, sometimes with a purplish discoloration of skin. There are two major catagories, schwannoma(neurilemmoma), and neurofibroma. Schwannomas are usually solitary and grow in the nerve sheath, rendering them relatively easy to dissect free. In contrast, neurofibromas tend to be multiple, grow in the endoneural substance, which renders them difficult to dissect, may undergo malignant changes, and are the hallmark of von Recklinghausens neurofibromatosis. Masses in the anterior part of neck may be initially thought to be thyroid nodule and then other cervical masses should be considered. The diagnosis rests on clinical suspicion and diagnostic support may be obtained by CT scan, magnetic resonance imaging(MRI) and substraction angiography in the literature. After imaging, fine needle aspiration for cytology may be helpful. If they are resected unrecognized and/or without regard to their nerve origin, major and permanent nerve defects can unnecessarily occur. We experienced 2 cases of peripheral nerve tumors of anterior neck simulating a thyroid nodule.
Angiography ; Biopsy, Fine-Needle ; Cauda Equina ; Diagnosis ; Neck* ; Neurilemmoma ; Neurofibroma ; Neurofibromatoses ; Peripheral Nerves* ; Peripheral Nervous System Neoplasms* ; Skin ; Spinal Nerve Roots ; Thyroid Gland* ; Thyroid Nodule* ; Tomography, X-Ray Computed

Although the papillary thyroid carcinoma generally follows an indolent course characterized by slow growth and abscence of distant metastasis, distant metastases to extrathyroid organ may occur in the lung, bone and brain in about 5% of patients. The lung metastases are usually diagnosed by plain X-ray, chest CT and 131I whole body scan. However, we present a case of papillary thyroid carcinoma with microscopic pulmonary metastasis that could not be detected by conventional method, such as chest X-ray, chest CT and 131I whole body scan. A 62-year-old female visited due to traumatic hemopneumothorax. In the pathology of resected tissues from lacerated lung parenchyme, metastatic papillary carcinoma was diagnosed without evidence of abnormality in radiologic examination and we diagnosed and treated an asymptomatic papillary thyroid carcinoma.
Brain ; Carcinoma, Papillary ; Female ; Hemopneumothorax ; Humans ; Lung ; Middle Aged ; Neoplasm Metastasis* ; Pathology ; Thorax ; Thyroid Gland* ; Thyroid Neoplasms* ; Tomography, X-Ray Computed ; Whole Body Imaging

BACKGROUND AND PURPOSE: We investigated predictors of institutionalization in patients with Alzheimer's disease (AD) in South Korea. METHODS: In total, 2,470 patients with AD aged 74.5±7.8 years (mean±standard deviation, 68.1% females) were enrolled from November 2005 to December 2013. The dates of institutionalization were identified from the public Long-Term-Care Insurance program in January 2014. We used a Cox proportional-hazards model to identify predictors for future institutionalization among characteristics at the time of diagnosis in 2,470 AD patients. A similar Cox proportional-hazards model was also used to investigate predictors among variables that reflected longitudinal changes in clinical variables before institutionalization in 816 patients who underwent follow-up testing. RESULTS: A lower Mini Mental State Examination score [hazard ratio (HR)=0.95, 95% confidence interval (CI)=0.92–0.97] and higher scores for the Clinical Dementia Rating and Neuro-Psychiatric Inventory (HR=1.01, 95% CI=1.00–1.01) at baseline were independent predictors of institutionalization. The relationship of patients with their main caregivers, presence of the apolipoprotein E e4 allele, and medication at baseline were not significantly associated with the rate of institutionalization. In models with variables that exhibited longitudinal changes, larger annual change in Clinical Dementia Rating Sum of Boxes score (HR=1.15, 95% CI=1.06–1.23) and higher medication possession ratio of antipsychotics (HR=1.89, 95% CI=1.20–2.97) predicted earlier institutionalization. CONCLUSIONS: This study shows that among Korean patients with AD, lower cognitive ability, higher dementia severity, more-severe behavioral symptoms at baseline, more-rapid decline in dementia severity, and more-frequent use of antipsychotics are independent predictors of earlier institutionalization.
Alleles ; Alzheimer Disease* ; Antipsychotic Agents ; Apolipoproteins ; Behavioral Symptoms ; Caregivers ; Dementia ; Diagnosis ; Follow-Up Studies ; Humans ; Institutionalization* ; Insurance ; Korea*

PURPOSE: Despite the rapid improvement in survival rate from Burkitt lymphoma and mature B-cell lymphoblastic leukemia (B-ALL) in children, a small subset of patients do not respond to first-line chemotherapy or experience relapse (RL). Herein, we report the clinical characteristics and outcomes of these patients. MATERIALS AND METHODS: RL or refractory Burkitt lymphoma and mature B-ALL in 125 patients diagnosed from 1990 to 2009 were retrospectively analyzed. RESULTS: Nineteen patients experienced RL or progressive disease (PD). Among them, 12 patients had PD or RL less than six months after initial treatment and seven had late RL. Seven patients achieved complete response (CR), 11 had PD, and one had no more therapy. Six patients who achieved CR survived without evidence of disease and four of them underwent high-dose chemotherapy (HDC) followed by stem cell transplantation (SCT). However, 11 patients who failed to obtain CR eventually died of their disease. Five-year overall survival (OS) was 31.6+/-10.7%. OS of patients with late RL was superior to that of patients with early RL (57.1+/-18.7%, vs. 16.7+/-10.8%, p=0.014). Achievement of CR after reinduction had significant OS (p < 0.001). OS for patients who were transplanted was superior (p < 0.01). In multivariate analysis, achievement of CR after reinduction chemotherapy showed an association with improved OS (p=0.05). CONCLUSION: Late RL and chemotherapy-sensitive patients have the chance to achieve continuous CR using HDC/SCT, whereas patients who are refractory to retrieval therapy have poor prognosis. Therefore, novel salvage strategy is required for improvement of survival for this small set of patients.
Adolescent* ; B-Lymphocytes* ; Burkitt Lymphoma* ; Child* ; Drug Therapy ; Humans ; Multivariate Analysis ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prognosis ; Recurrence ; Retrospective Studies ; Stem Cell Transplantation ; Survival Rate