Papillary ependymoma is a rare variant of ependymoma and often gives rise to confusion with choroid plexus papilloma because of topographic, light microscopic and ultrastructural similarities. Here, we report two cases of papillary ependymomas regarding their unique clinicopathologic features and differential points from choroid plexus papilloma. Brain MRI revealed a large mass in the left lateral ventricle in one case and a 3cm sized mass in the pineal area and the 3rd ventricle in the other. Microscopically, the tumor was characterized by papillary and tubular structures. Immunohistochemically, the tumor cells in both cases expressed cytokeratins(CK22 and CAM 5.2) but did not express glial fibrillary acidic protein(GFAP), vimentin, epithelial membrane antigen, and S100 protein. This is a very unusual immunohistochemical feature for papillary ependymoma. Ultrastructurally, the tumor showed a mosaic pattern of tumor cells with frequent intercellular microrosettes having a few stubby microvilli, a few cilia and zonulae adherentes. The cytoplasmic processes were markedly reduced compared to conventional ependymoma. The cytoplasm did not contain intermediate filaments. Interestingly, the mitochondria showed abnormal features with a pleomorphic shape and abnormal cristae in both cases. These ultrastructural features enabled differentiation between papillary ependymoma and choroid plexus papilloma in addition to the light microscopic findings.
Adult ; Carcinoma, Papillary/*pathology/surgery ; Case Report ; Diagnosis, Differential ; Ependymoma/*pathology/surgery ; Fatal Outcome ; Female ; Follow-Up Studies ; Glioma/*pathology ; Human ; Magnetic Resonance Imaging ; Middle Age

Inclusion body myositis is a rare myopathy that clinically resembles a chronic polymyositis and histopathologically is characterized by the presence of rimmed vacuoles containing ultrastructural cytoplasmic degradation products with filamentous intranuclear and cytoplasmic inclusions. Since clinical features are not uniform, histopathologic and ultrastructural studies are necessary to confirm the diagnosis. We report a typical case of inclusion body myositis with histopathologic and ultrastructural study. The patient was a 31 year old male who presented with progressive weakness of both forearms, hands and lower extremities for 10 years.
Adult ; Case Report ; Human ; Male ; Muscles/pathology ; Myositis, Inclusion Body/*pathology/physiopathology

A case of spinal meningeal melanocytoma is reported along with clinicopathologic, immunohistochemical and ultrastructural studies. This patient presented clinically with paraparesis, tingling sensation and numbness of both lower extremities of 4 months duration. No mucocutaneous pigmented nevi were found. On operation, scattered coal-black pigmented lesions were found in the meninges between T3 and T4-5 interspace level. Nearly total removal was carried out. The tumor was composed of spindle and epithelioid cells with heavy brown-black pigmentation. There was no pleomorphism, mitosis, hemorrhage, necrosis or invasion to the underlying cord tissue. In Korea, this case appears to be the first example of this disease. Neurologic deficit improved after surgical excision.
Adult ; Female ; Humans ; Immunoenzyme Techniques ; Meningeal Neoplasms/chemistry/*pathology/ultrastructure ; Microscopy, Electron ; Spinal Cord

No abstract available.
Down Syndrome* ; Leukemia, Megakaryoblastic, Acute*

No abstract available.
Exostoses, Multiple Hereditary* ; Sarcoma, Synovial*

Chordoma is relatively uncommon tumor comprising 1~4% of primary malignant bone tumors, and believed to arise from the remnants of notochordal tissue. Because of its occurrence in the thoracic spine, we report a case of chordoma in volving the thoracic spine. A 45-year-old male was suffered from chest pain radiating to the back. Chest CT showed a well marginated, round huge mass with multiseptated enhancement at the thoracic spine from T5 to T8 level, After percutaneous needle aspiration, piecemeal resection of the tumor was done. On cytologic smears. two types of neoplastic cells were arranged in sheets and cords in mucinous background. One type of cells consisted of medium sized cells with pink cytoplasm and round nuclei. The other type had voluminous bubbly or clear cytoplasm divided by intracytoplasmic septae imparting a feathery or basket-like appearance. Histologically, the tumor showed lobulated feature divided by fibrous septae and the tumor cells were pink eosinophilic or physaliphorous in morphology. Immunohistochemically, the tumor cells revealed strong positivity for low(AE1) and high (AE3) molecular weight cytokeratins.
Chest Pain ; Chordoma* ; Cytoplasm ; Eosinophils ; Humans ; Keratins ; Male ; Middle Aged ; Molecular Weight ; Mucins ; Needles ; Notochord ; Spine* ; Tomography, X-Ray Computed

The authors present a case of central neurocytoma in right lateral ventricle. Central neurocytoma is a rare clinicopathological entity that has been recently recognized and is characterized by 1) intraventricular location, 2) predominant occurance in young adults, 3) oligodendroglioma-like histology, 4) benign course, 5) immunohistochemical and ultrastructual evidence of neuronal differentiation. Twenty one year old female patient was admitted to the Department of neurosurgery of Chung Ang Gil hospital because of diplopia and headache which developed and progressed gradually 2 months prior to admission. The neurological examination showed paresis of right lateral rectus muscle, bilateral optic papilledema, nystagmus and mild left hemiparesis. MRI scan showd an intraventricular tumor of lateral ventricle that had isosignal and high signal intensity with cortex on T1-and T2-weighted and proton density images, respectively. Serpiginous flow voids representing blood vessels in the tumor were seen an all pulse sequences.
Blood Vessels ; Diplopia ; Female ; Headache ; Humans ; Lateral Ventricles* ; Magnetic Resonance Imaging ; Microscopy, Electron ; Neurocytoma* ; Neurologic Examination ; Neurons ; Neurosurgery ; Papilledema ; Paresis ; Protons ; Young Adult

Bednar tumor was described by Bednar in 1957. The histologic pattern of this tumor shows similar to dermatofibrosarcoma protuberans (DFSP) but melanin pigments are scattered within the tumor, It's rare neoplasm accounting for approximately 1-5% of all case of DFSP. Clinically, this tumor is considered to be intermediated malignancy, because of slow growth and frequent local recurrence and lack of distant metastasis. The majority are located on the trunk and the upper and lower extremities, but extremely rate in the head and neck area. Microscopically, this tumor is characterized by tight storiform spindle cells and long slender cells that admixed with a small population of melanin containing dendritic cells. This dendritic cells are the primary features distinguising this lesion from conventional DFSP. Complete surgical excision and close follow-up case are necessany for this neoplasm because of probable intermediate malignancy. A patient was admitted to Our Department of Oral and Maxillofacial Surgery due to swelling on right parotid area and numbness of the right lower lip on September, 1994, By clinical examinations and C-T finding, we dignosed tentatively as myxoma or pleomorphic adenoma. Surgical excision of this tumor was performed with parotidectomy and mandibular osteotomy under the frozen biopsy. By final microscopic and electromicroscopic examination and immunohistochemical study, this tumor was diagnosed as Bednar tumor. So, we report a case of pigmented DFSP with review of literatures.
Adenoma, Pleomorphic ; Biopsy ; Dendritic Cells ; Dermatofibrosarcoma* ; Follow-Up Studies ; Head ; Humans ; Hypesthesia ; Lip ; Lower Extremity ; Mandibular Osteotomy ; Melanins ; Myxoma ; Neck ; Neoplasm Metastasis ; Recurrence ; Surgery, Oral

Adenomatous polyps of the duodenum is uncommon in general population, but duodenal adenomas are found in the majority of patients with both familial adenomatous polypasis and Gardner's syndrome. These polyps are usually small, multiple and most commonly found in the second portion of the duodenum, and may involve ampulla of Vater. They also have malignant potential so that routine surveillance of the duodenum including ampulla of Vater has been recommended in patients with adenomatous polyposis coli. The adenoma of ampulla of Vater can be found in approximately 50% of patients with familial adenomatous polyposis and usually is asymptomatic, but sometimes, especially villous adenoma, may exhibit clinical problems including partial gastric outlet obstruction, pancreatitis, bleeding, obstructive jaundice, in addition to a high incidence of malignancy. Among the non-malignant complications of the ampullary tumor, there are only a few case reports of acute pancreatitis as the presenting manifestation of an ampullary adenoma or carcinoma in patients with familial adenomatous polyposis in the English literature, but no cases have been reported in Korea. We present here a case of symptomatic adenoma of the ampulla of Vater presenting as acute pancreatitis in a patient with familial adenomatous polyposis.
Adenoma* ; Adenoma, Villous ; Adenomatous Polyposis Coli* ; Adenomatous Polyps ; Ampulla of Vater ; Duodenum ; Gardner Syndrome ; Gastric Outlet Obstruction ; Hemorrhage ; Humans ; Incidence ; Jaundice, Obstructive ; Korea ; Pancreatitis* ; Polyps

Juvenile xanthogranuloma (JXG) is a benign and self-limiting non-Langerhans-cell histiocytosis that generally occurs during infancy and childhood. It develops frequently in the head and neck but is very rare in the nasal cavity. To date, only five cases of JXG in the nasal cavity have been reported. Here, we report the second case of JXG in the nasal cavity in Korea. A 19-year-old male patient presented with a protruding 1.1 cm mass in the left nasal vestibule. Histologically, a dense dermal infiltrate of histiocytes with Touton giant cells was observed. Immunohistochemically, the histiocytes tested positive for CD68 and the S-100 protein but negative for CD1a. This shows that a S-100-positive histiocytic lesion dose not exclude a diagnosis of JXG.
Diagnosis ; Giant Cells ; Head ; Histiocytes ; Histiocytosis, Langerhans-Cell ; Histiocytosis, Non-Langerhans-Cell ; Humans ; Korea ; Male ; Nasal Cavity* ; Neck ; S100 Proteins ; Xanthogranuloma, Juvenile* ; Young Adult