Cases reported "Sclerosis"

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1/47. Possible development of idiopathic sclerosing encapsulating peritonitis.

    We report a rare case of idiopathic sclerosing encapsulating peritonitis (SEP). During a laparotomy before undergoing a distal gastrectomy with Billroth II reconstruction for early gastric cancer, the patient was found to have a membranous encapsulation wrapping each small bowel loop, unlike peritoneal encapsulation or typical SEP. He had complained of persistent heartburn, distension and diarrhea for 2 months in the post-operative course. The second laparotomy, which was performed to improve prolonged transit, revealed typical SEP with a thick and fibrotic membrane that encased the small bowel entirely. Stripping of the sclerosing encasing membrane, separation of the adherent loops of the proximal small bowel, and Braun's anastomosis were performed. The patient complained of epigastric fullness and diarrhea after he was relieved from the complete bowel obstruction for 45 days post-operatively. trimebutine maleate was administrated 5 months after the second operation and this markedly improved his symptoms. This case might reflect the developmental process of idiopathic SEP. In addition, the use of a motility regulator may improve symptoms related to the abnormal intestinal motility by this disease.
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2/47. Giant cell angiofibroma of the buccal mucosa.

    We report a case of a patient with a recently described entity (giant cell angiofibroma) occurring in the oral cavity. A submucosal solid tumor arising in the right buccal mucosa of a 46-year-old woman was histologically characterized by a patternless spindle-cell proliferation within a generally myxoid stroma containing areas of perivascular sclerosis. Numerous multinucleated giant cells of the floret type were present. Immunohistochemically, the tumor cells and multinucleated giant cells exhibited positivity for CD34. To the best of our knowledge, this is the first report of a giant cell angiofibroma arising in the oral cavity.
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3/47. Histopathological findings of X-linked retinoschisis with neovascular glaucoma.

    BACKGROUND: X-linked retinoschisis (XLRS) is rarely complicated by neovascular glaucoma. Only a few reports of XLRS histopathological findings with neovascular glaucoma have been published. methods: A 41-year-old man with XLRS complicated by neovascular glaucoma in his left eye was examined with electroretinography, B-scan, ultrasound biomicroscopy and computed tomography. He was examined by ophthalmoscopy and fluorescein angiography in the other eye. An enucleation was performed in his left eye due to uncontrollable high intraocular pressure and persistent ocular pain. We examined the enucleated eye histopathologically. RESULTS: Examination of the enucleated eye showed nuclear sclerosis of the lens, pigmented retrolental membrane and retinoschisis which separated the inner layer of the retina and made a large space in the vitreous cavity without any apparent detachment of the outer layers of the retina. Sclerotic vessels were present histopathologically in both the inner and outer layers of the retina. There was a peripheral anterior synechia, ectropion uveae and a fibrovascular membrane, which contained many lumina of neovascularization, indicating marked rubeosis iridis. Small cystic spaces were observed in both the schitic retina in the peripheral region and the foveal schisis at the outer layer of the retina. The photoreceptor cells had become markedly atrophied and multiple regions of calcification were observed. The optic nerve showed severe atrophy with gliosis, but the central retinal artery and vein were still open within the nerve. CONCLUSIONS: These histopathological findings suggest that rubeosis iridis may have developed secondarily to retinal ischemia due to occlusion of the retinal blood vessels.
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4/47. Sclerosing inflammatory myofibroblastic tumour of the tongue: an immunohistochemical and ultrastructural study.

    A case of inflammatory myofibroblastic tumour (IMT) arising in the tongue of a 27-year-old man is described. The patient presented with a solitary, well-circumscribed submucosal mass of 4 months duration. The tumour showed in its largest part a paucicellular sclerosing lesion resembling a hyalinizing granuloma surrounded by a thin rim of an admixture of myofibroblasts, plasma cells and foamy histiocytes. myofibroblasts expressed vimentin and alpha-smooth muscle actin positive immunophenotypes. Ultrastructurally the hyaline areas were composed of abundant collagen fibres with sparse myofibroblasts. Extensive scar-like change in this IMT may be related to a traumatic insult.
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5/47. Sclerosing encapsulating peritonitis: regional changes of peritoneum.

    Sclerosing encapsulating peritonitis (SEP) is characterized by the diffuse appearance of marked sclerotic thickening of the peritoneal membrane. We experienced a case with SEP accompanied by regional changes of peritoneum. A 37-year-old woman with end-stage renal failure was started on continuous ambulatory peritoneal dialysis in 1985 and was transferred to hemodialysis in 1997. She was admitted because of ileus in 1998 with SEP and died of septicemia. The diagnosis of SEP was confirmed via the autopsy. The root of the mesentery was retracted and shortened. Since the peritoneal change was marked in the regions with free margin of mesentery and was less apparent in the regions not adhered to mesentery, it is indicated that mechanical stress also contributes to the occurrence of SEP. Since calcification and ossification were only seen in a free margin of small bowel from mesentery, it is suggested that there is a close relationship between calcification and ossification. Since fibrosis invaded into the muscle layer, dysfunction of bowel movement as well as bowel obstruction contributed to the appearance of ileus. It is suggested that mechanical stress by the root of mesentery which is retracted and shortened also contributes to the appearance of SEP.
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6/47. Asymptomatic chronic intestinal ischemia caused by idiopathic phlebosclerosis of mesenteric vein.

    Phlebosclerosis of the mesenteric vein is a rare condition causing chronic intestinal ischemia, it has only been reported in japan. A 56-year-old man with liver cirrhosis and hepatic tumor presented with phlebosclerosis of mesenteric vein without any abdominal symptoms. He was admitted for examination of suspected hepatic tumor. Abdominal plain x-ray films and computed tomography revealed calcification of the mesenteric vein. barium enema revealed narrowing and thumbprinting from the cecum to transverse colon. On colonoscopic examination, blue-black vessels were visible in the terminal ileum, and hyperemic nodular mucosa with small irregular ulcers surrounded by dark purple mucosa was found from the cecum to transverse colon. The etiology of mesenteric vein phlebosclerosis is unknown, although a physical mechanism rather than inflammatory changes appear to be involved in this rare and usually progressive condition of chronic intestinal ischemia.
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7/47. Mesenteric phlebosclerosis: a new disease entity causing ischemic colitis.

    PURPOSE: Nonthrombotic stenosis or occlusion of the mesenteric veins is a rare cause of intestinal ischemia. The aim of this study was to describe a new disease entity causing chronic ischemic colitis. methods: Seven patients were diagnosed as having mesenteric phlebosclerosis. All seven patients had calcifications in the small mesenteric veins and their intramural branches. No evidence of vasculitis or portal hypertension was recognized. None of the patients had a history of gastrointestinal disease or of prolonged drug use. We report clinical, laboratory, radiographic, endoscopic, and histopathologic findings. RESULTS: Clinical findings included abdominal pain and diarrhea of a gradual onset and chronic course. A positive fecal occult blood test and mild anemia were often found. The patients had linear calcifications and stenosis in the right colon, which were discovered by plain abdominal radiography and barium enema, respectively. Endoscopic findings included edematous, dark colored mucosa and ulcerations. Four patients underwent a subtotal colectomy because of persistent abdominal pain or ileus. The histopathologic findings were macroscopically characterized by a dark purple or dark brown colored colonic surface, the swelling and disappearance of plicae semilunares coli, and marked thickening of the colonic wall, while they were microscopically characterized by marked fibrous thickening of the venous walls with calcifications, marked submucosal fibrosis, deposition of collagen in the mucosa, and foamy macrophages within the vessel walls. CONCLUSIONS: These peculiar lesions have not previously been fully described. The cause and pathogenesis still remain unknown. We conclude that such lesions represent a new clinicopathologic disease entity and propose the term "idiopathic mesenteric phlebosclerosis."
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8/47. Sclerosing hemangioma with lymph node metastasis.

    Sclerosing hemangioma (SH) of the lung is an uncommon type of tumor, which is composed of polygonal and cuboidal cells. This disease is generally regarded as benign but extremely rare cases with lymph node metastasis have been reported. We report a case of SH with a metastasis to the regional lymph nodes. A 19-year-old girl presented with a 2-year history of coughing. A chest X-ray and a CT scan indicated a large mass in the lower lobe. As a result, a left lower lobectomy with a dissection of the hilar and interlobar lymph nodes was performed. The tumor was a well-defined huge mass with partial adhesion to the mediastinal and parietal pleura. The dissected hilar, interlobar, and intrapulmonary lymph nodes demonstrated metastasis. Histologically, the primary and metastatic tumor consisted of polygonal and cuboidal cells. Both types of tumor cells were uniformly immunoreactive to the epithelial membrane antigen (EMA) and the thyroid transcription factor-1 (TTF-1). However, the cuboidal cells tested positive for pancytokeratin, whereas the polygonal cells tested consistently negative. Postoperatively, the patient received chemotherapy and no recurrence or metastasis 2 years after surgery was noted. Although a pulmonary SH is considered to be benign, this case highlights the need for the evaluation of lymph node metastasis.
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9/47. Sclerosing encapsulating peritonitis (SEP) as a delayed complication of continuous hyperthermic peritoneal perfusion (CHPP): report of a case.

    A 47-year-old woman underwent curative resection of advanced gastric cancer, followed by continuous hyperthermic peritoneal perfusion (CHPP). She was readmitted to our hospital 6 months after the operation with a diagnosis of postoperative adhesional ileus. An exploratory laparotomy revealed that the small intestine, which had normal serosa, was folded and enveloped in thickened peritoneum like a "cocoon," suggesting sclerosing encapsulating peritonitis (SEP). Because of tight adhesion in the ileocecal region, resection of the membrane was performed only in the feasible areas, followed by side-to-side anastomosis between the ileum and ascending colon. The patient has remained well for 15 months since this operation with no radiological signs or laboratory findings of recurrence. When small bowel obstruction does not show improvement with conservative treatment, and if the possibility of peritoneal cancer recurrence is excluded by thorough examinations, it is important to perform laparotomy early to resolve the symptoms of bowel obstruction and restore the patient's quality of life.
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10/47. Capillary changes in skeletal muscle of patients with Ullrich's disease with collagen VI deficiency.

    We examined the capillaries in muscle biopsy specimens from two patients with Ullrich's disease with collagen VI deficiency by light and electron microscopy. collagen VI plays an important role in platelet aggregation for binding von willebrand factor. Using immunohistochemistry, collagen VI was shown to be absent on capillaries from patients with Ullrich's disease, while von willebrand factor, collagen IV, and vascular endothelial growth factor were normally expressed. Electron microscopy revealed narrow lumens, large nuclei in endothelial cells, and fenestration of a capillary. The number of pinocytotic vesicles per unit endothelial cytoplasm was increased. The cytoplasm of endothelial cells was strongly stained with uranyl acetate and lead citrate. Replication of the capillary basement membrane was observed. On the other hand, easy bleeding and coagulation were not observed in the two patients. These findings suggested that the collagen VI deficiency might have caused the electron microscopic changes of capillaries, while the function of the capillaries is apparently retained.
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