Sjögren-Larsson syndrome(SLS) is a rare hereditable disease characterized by congenital ichthyosis, spastic diplegia and mental retardation. Along with the typical triad of symptoms, many patients with this disease have short stature, kyphosis and glistening dots in the retina of the eye. The pathogenesism is unknown but recent studies suggest that SLS might be, at least in part, a disorder of fatty acid metabolism. We describe a patient with a pathognomonic finding in the fundus and with the classic features of SLS.
Cerebral Palsy ; Humans ; Ichthyosis ; Intellectual Disability ; Kyphosis ; Metabolism ; Retina ; Sjogren-Larsson Syndrome*

A case of primary gastric carcinoma with patterns resembling those of hepatocellular carcinoma(HCC) is reproted. This patient who represented high serum alpha-fetoprotein(AFP) underwent a subtotal gastrectomy for stomach cancer and lobectomy of liver metastasis. The hepatoid component of the tumor was characterized by solid nests of large polygonal cells arranged in cords and trabeculae, with central nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm; single-nuclear giant cells were frequently noted. These tumor cells of stomach stained positive immunohistochemically for AFP, ACT(alpha-1-antitrypsin), AACT(alpha-1-antichy-motrypsin). Thus, this hepatoid gastric adenocarcinoma tumor cells demonstrated both morphologic and immunohistochemical features of partial differentiation to the HCC. Careful histologic examination in conjunction with the immunohistochemical demonstration of AFP can provide a useful contribution to the diagnosis of this rare histological type of gastric carcinoma And the presence of metastatic hepatoid adenocarcinoma from the stomach should be considered in a patient with hepatic tumor mimicking HCC. Because of frequent venous and lymphatic invasion, as well as liver metastasis, prognosis appears to be poor. The authors reviewed 45 cases of hepatoid adenocarcinoma of stomach reported in the literature, also.
Adenocarcinoma ; Carcinoma, Hepatocellular ; Neoplasm Metastasis ; Stomach Neoplasms

The spindle and epithelioid cell nevus, is known also as Spitz's nevus or benign juvenile melanoma. The histologic picture of the lesion often closely resembles that of a malignant melanoma, but spindle and epithelioid cell nevus hehaves benignly. Criteria for accurate histological diagnosis of this nevus now exist. However, a difficult lesion occasionally brings one to the worrisome situation even now. We experienced four cases (three children and one adult) of spindle and epithelioid cell neveus with nodular or macular skin lesions. In children (7/F, 9/F, 4/M), all of the lesion show a solitary pinkish red or light brown nodular firm masses, 0.7 cm to 1.0 cm in greatest dimension, which are located at the left posterior thigh, posterior neck and right anterior neck region, respectively. In adult (36/F), the lesion shows a light brown, erythematous macule, 0.8 cm in greatest diameter, in the skin of the back. Microscopically, both spindle and epithelioid cells are found in all the four cases, but epithelioid cells predominate in children and spindle cells predominate in adult.
Child ; Adult ; Male ; Female ; Humans

We experienced an autopsy case of esophageal atresia with tracheoesophageal fistula and other multiple congenital anomalies in a 4 days old male infant. Esophageal atresia with tracheoesophageal fistula was type C by Gross Classification. Combined anomalies were malrotation of left kidney, one left accessory renal artery form right renal artery and one left accessory renal vein from right renal vein, duodenal atresia and annular pancreas. Until now, the esophageal atresia with tracheoesophageal fistula and other multiple congenital anomalies (7 congenital anomalies) are not reported in the korean literature, except our case. Our autopsy case, it suggests that esophageal atresia with tracheoesophageal fistula has often close relation-ship with other multiple congenital anomalies.
Infant ; Male ; Female ; Humans

The occurrence of sqamous cell carcinoma arising from epidermal cyst is unusual. A 54-year-old man with soft tissue mass on the back for 25 years presented an infiltrating squamous cell carcinoma within the wall of epidermal cyst. All tissue excised deserved to be examined to be axamined carefully in longterm epidermal cyst.
Male ; Humans ; Cysts

Synovial sarcoma most commonly affects the extremities, especially the lower thigh and knee region. However, a smaller number develops in a central or axial distribution, an area which encompasses the trunk, orofacial, cervical and parapharyngeal regions. The retroperitoneum is an extremely unusual site and has never been recorded in the literature as primary a site for synovial sarcoma. We investigated a case of retroperitoneal synovial sarcoma in a 40-year-old woman. The specimen consisted of fragmented large bulky multinodular masses separated by slit-like spaces. The tumor was 130 gm in weight. Microscopically, the tumor was composed of nests of plump ovoid to polygonal shaped cells and bundles of spindle shaped cells, which had vesicular nuclei and a small amount of cytoplasm. In some areas, there were cleft like spaces and pseudoglandular structures lined by flat or cuboidal cells. Myxoid change, collagen deposition, foci of calcification and osseous metaplasia were also present. Immunohistochemically, the polygonal cells and some of the spindle cells reacted positively for keratin. The spindle cells, especially in the perivascular area were positive for vimentin. S-100 protein and GFAP were negative in both type of cells.
Female ; Humans

Pulmonary tumors exhibiting neuroendocrine differentiation are classified as typical carcinoid, atypical carcinoid, and small cell lung carcinoma(SCLC). Travis et al. proposed a fourth category of large cell neuroendocrine carcinoma, which is characterized by light microscopic neuroendocrine appearance, cells of large size, polygonal shape, low nuclear cytoplasmic ratio, coarse nuclear chromatin, with prominent nucleoli high mitotic rate and frequent necrosis; and neuroendocrine features by immunohistochemistry or electron microscopy. High grade neuroendocrine carcinoma (LCAC-NE) revealed aggressive clinical course. We report two cases of neuroendocrine tumors of the lung characterized by a trabecular pattern of large pleomorphic cells with frequent mitoses and wide necrosis. The frequent metastatic sites of atypical carcinoid were liver, bone and brain. One of our case is presented, at first, as an ovarian mass, which shows multifocal rosettes and revealed metastasis from lung. Both cases expressed neuroendocrine differentiation by light microscopy and immunohistochemistry. However clinical neuroendocrine symptom were not present.
Brain ; Carcinoid Tumor ; Carcinoma, Neuroendocrine* ; Chromatin ; Cytoplasm ; Female ; Immunohistochemistry ; Liver ; Lung* ; Microscopy ; Microscopy, Electron ; Mitosis ; Necrosis ; Neoplasm Metastasis ; Neuroendocrine Tumors ; Ovary

Thirty cases of gastric adenocarcinoma were examined immunohistochemically for expression of transforming growth factor-beta(TGF-beta) in order to analyze significant correlation with clinical stage and pathologic grade of gastric adenocarcinoma. Specific immunostaining was clearly detected in the cytoplasm of the neoplastic cells. The TGF-,6 expression in the gastric adenocarcinoma is closely related to the depth of invasion, the degree of invasiveness and the presence of metastasis. Thus, we observed the stronger immunohistochemical. expression of TGF-beta in the deeper portion of invasion and in the invasive gastric adenocarcinomas with the lymph nodal metastasis than in the superficial portion of invasion and in those without the lymph nodal metastasis. There results suggest that the transforming growth factors expression in carcinoma cells may play an important role in the carcinomatous invasion resulting in metastasis.
Adenocarcinoma ; Neoplasm Metastasis

Lymphangioma is an uncommon malformation of the lymphatic system that involves a benign proliferation of the lymphatics, with no established treatment method. Multiple colonic lymphangioma, or colonic lymphangiomatosis, is an extremely rare condition. We report a case of colonic lymphangiomatosis that was detected during a colonoscopic examination conducted as part of a general health check-up. The lesion completely resolved after excisional biopsy.
Biopsy* ; Colon* ; Lymphangioma ; Lymphatic System

No abstract available.
Acupuncture* ; Mycobacterium*