The clear cell variant of ependymoma is a rare, recently described, intracranial tumor which is composed of clear neoplastic ependymal cells. Clear cell ependymomas may share characteristic histologic features of oligodendrogliomas or central neurocytomas; striking nuclear uniformity, perinuclear halos, and numerous angulated capillaries. In contrast to oligodendrogliomas, however, clear cell ependymomas are noninfiltrating tumors with sharp boundaries. Perivascular pseudorosette formation is frequent. Oligodendrogliomas are usually nonreactive for GFAP compared to diffuse immunoreactivity of clear cell ependymoma. Central neurocytomas may also be differentiated by their immunoreactivity for synaptophysin. This is a case of clear cell ependymoma in a 40-year-old man. By computed tomography and magnetic resonance imaging scans, a well circumscribed cystic tumor with mural nodule was demonstrated in the right frontal lobe. It was 6cm in diameter and well enhanced. Histologically, it was sharply demarcated from the brain parenchyma. The cystic wall was lined by atypical ependymal cells, which "transformed" to clear cells in the solid area. The cells had uniform nuclei and perinuclear halos. Mitotic figures and necrotic foci were focally present. The cells were immunoreactive for glial fibrillary acidic protein (GFAP), while synaptophysin was negative. Electron microscopy revealed densely packed polyheadral cells with scant organelles and well developed intercellular junctions.
Adult ; Brain ; Capillaries ; Ependymoma* ; Frontal Lobe ; Glial Fibrillary Acidic Protein ; Humans ; Intercellular Junctions ; Magnetic Resonance Imaging ; Microscopy, Electron ; Neurocytoma ; Oligodendroglioma ; Organelles ; Strikes, Employee ; Synaptophysin

No abstract available.
Humans ; Tuberculosis, Pulmonary*

We report an autopsy case of secondary hemochromatosis associated with multiple frequent blood transfusion for the treatment of aplastic anemia. A 23-year-old man had been diagnosed as having aplastic anemia at the age of 13. He received a whole blood transfusion, about 1280 ml, every month during the past 10 years. Recently he developed diabetes mellitus and a congestive heart failure. The autopsy revealed that multiple organs were affected by secondary hemochromatosis, including the liver, heart, pancreas, spleen, bone marrow, stomach, thyroid gland, adrenal glands, and testes. The lungs and liver showed gross and microscopic findings consistent with a congestive heart failure in addition to hemochromatosis. The details are presented. This is a case of rare secondary hemochromatosis occurring in a young man and presenting the classic histopathologic changes indistinguishable from those of primary hemochromatosis.
Adrenal Glands ; Anemia ; Anemia, Aplastic* ; Autopsy* ; Blood Transfusion ; Bone Marrow ; Diabetes Mellitus ; Heart ; Heart Failure ; Hemochromatosis* ; Humans ; Liver ; Lung ; Pancreas ; Spleen ; Stomach ; Testis ; Thyroid Gland ; Young Adult

Mobius syndrome is generally considered to be a static disorder of congenital origin, and is manifested as unilateral or bilateral facial weakness and lateral gaze limitation. In most instances the syndrome occurs sporadically, but rarely familial cases have been reported. We report a family of three members with Mobius syndrome; a 7-year-old girl, a 6-year-old boy, and their 29-year-old mother. Each patient revealed facial diplegia, and unilateral or bilateral lateral rectus palsy. Brain MRI scans showed normal and there were no definite brainstem dysfunctions on electrophysiologic studies.
Abducens Nerve Diseases ; Adult ; Brain ; Brain Stem ; Child ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Mobius Syndrome* ; Mothers

Mobius syndrome is generally considered to be a static disorder of congenital origin, and is manifested as unilateral or bilateral facial weakness and lateral gaze limitation. In most instances the syndrome occurs sporadically, but rarely familial cases have been reported. We report a family of three members with Mobius syndrome; a 7-year-old girl, a 6-year-old boy, and their 29-year-old mother. Each patient revealed facial diplegia, and unilateral or bilateral lateral rectus palsy. Brain MRI scans showed normal and there were no definite brainstem dysfunctions on electrophysiologic studies.
Abducens Nerve Diseases ; Adult ; Brain ; Brain Stem ; Child ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Mobius Syndrome* ; Mothers

Ovarian mature cystic teratoma containing benign prostatic tissue is rare and only 11 cases have been reported in the literature to date. We report a case of mature cystic teratoma of the ovary containing prostatic tissue. A 23-year-old female patient came to our hospital complaining of irregular menstruation for 3 months. Her menarche had occurred when she was 13 years old. The patient showed no evidence of virilization nor of endocrinopathy. A goose-egg-sized mass was palpable in the left lower abdomen on physical examination. Ultrasonography revealed a cystic ovarian mass with internal echogenecity. Microscopic finding was consistent with that of usual mature cystic teratoma except for a 1.5 cm focus of prostatic and bladder tissues. Prostatic tissue demonstrated strong immunoreactivity for prostatic specific antigen (PSA), prostatic alkaline phosphatase (PAP) and cytokeratin 7. Basal cells of the prostate glands were positive for high molecular weight cytokeratin (34betaE12). Although ovarian mature cystic teratoma containing prostatic tissue has been reported as a rare occurrence, a careful examination with immunohistochemical staining may increase the detection of prostatic tissue in mature cystic teratoma of the ovary.
Abdomen ; Adolescent ; Alkaline Phosphatase ; Female ; Humans ; Keratin-7 ; Keratins ; Menarche ; Menstruation ; Molecular Weight ; Ovary ; Physical Examination ; Prostate ; Teratoma* ; Ultrasonography ; Urinary Bladder ; Virilism ; Young Adult

No abstract available.
Hearing Loss, Sensorineural*

After advent of lymphangiographic technique, the causes of chyluria can be evaluated by lymphangiography. Themost common etiology known until today is parasitic origin, expecially filariasis. In Korea, established organismof filariasis is Brugia malayi. And other nonparasitic etiologies such as retroperitoneal malignancy, chronicinflammatory diseases, trauma, pregnancy, aneurysm are very rare. The authors experienced two cases oflymphatico-calyceal fistulas causing chyluria demostrated by lymphangiography. The etiology of these two caseswere unknown exactly, but the clinical diagnosis were filariasis. These cases are reported with emphasis on thelymphangiographic findings of chyluria.
Aneurysm ; Brugia malayi ; Diagnosis ; Filariasis ; Fistula ; Korea ; Lymphography ; Pregnancy