Cases reported "Sclerosis"

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1/22. 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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2/22. Fine needle aspiration cytology of well-differentiated liposarcoma. A report of two cases.

    BACKGROUND: Well-differentiated liposarcoma is difficult to diagnose on fine needle aspiration cytology (FNAC) smears and may create considerable diagnostic problems. CASES: Males aged 60 and 45 years presented with a swelling in the groin and retroperitoneal region, respectively. FNAC showed large cells with multilobulated nuclei and mature-looking fat tissue. A soft tissue tumor with bizarre cells was diagnosed cytologically in case 1 and liposarcoma in case 2. Histologically, both cases were diagnosed as well-differentiated sclerosing liposarcoma. CONCLUSION: The cytologic diagnosis of well-differentiated liposarcoma should be done with caution, and the sites should be taken into consideration. Deep-seated tumors with large, bizarre, giant cells should have wide excision as they recur more frequently.
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3/22. Giant cell collagenoma: case report and review of the literature.

    BACKGROUND: Giant cell collagenoma (GCC) is a recently described cutaneous fibrous neoplasm that usually affects young to middle-aged adults. Despite its similar histological appearance with circumscribed storiform collagenoma, no association of GCC with Cowden's syndrome has been described so far. To the best of our knowledge only five cases of this rare fibrous tumor have been reported so far. methods: We report a case of a 79-year-old male patient presenting with a slow growing flesh-colored dome-shaped lesion in his left forearm, with a clinical diagnosis of fibroma. No stigma of Cowden's syndrome was depicted. RESULTS: The histological analysis showed a symmetrical and well-circumscribed flat-dome-shaped lesion covered by an atrophic overlying epidermis. The neoplasm was composed of hyalinized collagen bundles disposed in a whorled storiform pattern. Admixed with the collagen matrix, there were two distinct cell populations, one composed by spindle-shaped mononuclear cells, and the other composed by bizarre multinucleated giant cells. Immunohistochemical analysis showed positivity for vimentin and actin HHF35 in the mononucleated. The multinucleated cells only immunoexpressed vimentin. CONCLUSION: GCC is an unusual cutaneous fibrous tumor that should be differentiated from circumscribed storiform collagenoma, pleomorphic fibroma, regressive forms of dermatofibroma, and solitary myofibroma based on its histological features.
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4/22. Sclerosing extramedullary hematopoietic tumor.

    A 73-year-old woman presented with abdominal pain, weakness, and weight loss. Pertinent medical history included myelofibrosis, severe anemia, and lumpectomy for breast cancer. Computed tomography showed marked splenomegaly and numerous soft tissue masses in the mesentery. Excisional biopsy of the mass showed an encapsulated tumor with yellow-tan nodular growth pattern. Microscopically, the tumor was characterized by the presence of bizarre giant cells in background of prominent dense fibrous stroma. The atypical giant cells were positive for FVIII antibody staining. Many other cells were positive for CD43 antibody staining, indicating that the tumor was hematopoietic in nature, so-called sclerosing extramedullary hematopoietic tumor. Extramedullary hematopoiesis is a common phenomenon associated with severe anemia of any cause. When associated with myelofibrosis, however, it has different morphology from extramedullary hematopoiesis of the other causes. The significance of recognizing sclerosing extramedullary hematopoietic tumor is that it is often mistaken for sarcomas and other malignant neoplasms, especially when the clinical history is unknown.
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5/22. Successful treatment of multiple bursal cysts in systemic sclerosis.

    BACKGROUND: bursitis frequently occurs in the various conditions of autoimmune disorders including rheumatoid arthritis, but there have been few cases of effusive bursitis in systemic sclerosis. OBJECTIVE: To present a case of systemic sclerosis with multiple bursitis on upper, lower extremities, and trunk with or without joint involvement. methods: Case report and review of the literature. RESULTS: Multiple asymptomatic cystic masses contained yellow and chalky sterile fluid, all of which were diagnosed as effusive bursitis. Most of them were treated with a surgical resection, a continuous drainage, and an injection of highly concentrated ethanol into their internal spaces. However, an intrabursal injection of emulsified triamcinolone acetonide was the only effective treatment for the giant mass that occurred on the right chest wall. CONCLUSION: Successful treatment of multiple bursal cysts with systemic sclerosis was presented.
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6/22. Primary sclerosing lipogranuloma with broad necrosis of the scrotum.

    A-25-year-old man was admitted because of a painless tumor of the scrotum. The patient denied a history of exogenous material injection and trauma in the scrotum. Physical and radiological examination revealed a mass in the scrotum, and blood laboratory tests showed no significant findings except for mild eosinophilia (5.6%). Resection of the mass was performed. The mass was isolated and located in the subcutaneous tissue of the scrotum. The mass was rectangular and symmetrical, and measured 65 x 45 x 15 mm. Histologically, the mass was composed of adipose tissue with fibrosis. Many epithelioid granulomas with multinucleated giant cells of foreign body and Langhans' types and heavy infiltrates of lymphocytes and eosinophils were recognized. Characteristically, the lesion showed broad coagulative and lytic necrosis. Congestion and edema suggestive of ischemia were seen in some areas. Special stains for acid-fast bacteria, gram-positive bacteria and fungi failed to detect any microorganisms. polymerase chain reaction for mycobacterium tuberculosis revealed no reaction products. Immunohistochemically, the majority of lymphocytes were CD45RO-positive T cells, and S-100 protein-positive cells and CD68-positive macrophages were scattered in small amounts. The appearances were typical for sclerosing lipogranuloma except for the necrosis. Although the pathological mechanism of the broad necrosis is unclear, the necrosis might be the result of ischemia. Our case suggests that primary sclerosing lipogranuloma of the scrotum might show broad necrosis, and that T-cell-mediated immune response might play a part in the formation of lipogranuloma.
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7/22. Atypical fibroxanthoma with prominent sclerosis.

    BACKGROUND: Malignant cutaneous spindle cell lesions with marked sclerosis are uncommon. Only a few cases of cutaneous leiomyosarcoma and dermatofibrosarcoma protuberans with sclerosis have been published. methods: We report a case of atypical fibroxanthoma (AFX) with prominent sclerosis and hyalinization occurring on the scalp of an 81-year-old male. RESULTS: Histopathologic examination revealed an exophytic, well-delineated, focally ulcerated tumor arising in sun-damaged skin. The lesion was composed of atypical spindle cells arranged in a fascicled and vaguely storiform pattern. Occasional multinucleated giant cells were present. The tumor cells were strongly positive for CD99 (O13), vimentin, and smooth muscle actin, and focally positive for CD68. There was striking sclerosis with hyalinization throughout the lesion. CONCLUSIONS: Rarely, AFX may exhibit marked sclerosis with areas of complete replacement of tumor by hyalinized collagen. In a small biopsy, such hyalinization may be a diagnostic pitfall leading to an erroneous diagnosis.
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8/22. Sclerosing encapsulating peritonitis: differential diagnosis to peritoneal encapsulation and abdominal cocoon--a case report.

    A 59 year old man presented with symptoms of partial bowel obstruction. Small bowel x-ray studies did not allow to identify the nature of the intestinal process in the upper ileum. At laparotomy small bowel encapsulation with a whitish membrane was encountered. Despite partial removal of this membrane small bowel obstruction persisted and two weeks postoperatively the patient died of peritonitis and cardiac insufficiency. autopsy findings revealed massive fibrous adhesions in the abdomen with granulomatous inflammation. The presence of foreign body giant cells and bifringent crystals were characteristic for talcum powder. The latter suggested a causal role of an appendectomy 45 years earlier. The diagnosis of sclerosing encapsulating peritonitis as established in our patient needs to be separated from peritoneal encapsulation, a congenital malformation, and abdominal cocoon, which contains histological elements of inflammation. This case report should draw attention to these entities in the differential diagnosis and surgical management of small bowel obstruction.
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9/22. Membranocystic lesion in sclerosing lipogranuloma of the scrotum: an ultrastructural study.

    Two cases of intrascrotal sclerosing lipogranuloma are reported in which affected adipose tissues showed peculiar membranocystic lesions (MCL) similar to the features described in Nasu-Hakola disease. On light microscopy, the MCL were seen in the degenerative fatty tissues and within multinucleated giant cells, which were positive for periodic acid-Schiff stain and resistant to diastase digestion. Ultrastructurally, two types of MCL were observed; one type was characterized by thick membranes composed of vesicular to minute tubular or fibrillar substructures, and the other type was characterized by thin membranes composed of only small numbers of tiny vesicles. The substructures in both types of MCL contained lipid droplets similar to those in the inner space. These results support the concept that the membranes in the MCL are derived from degenerated fat droplets.
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10/22. Pathologic features of dysplasia and accompanying alterations observed in surgical specimens from patients with intractable epilepsy.

    Malformations caused by abnormalities of cortical development, or cortical dysplasias, were examined in surgical specimens from 108 patients with medically intractable epilepsy to determine the scope of histopathologic changes. The relevance of the clinical findings was also evaluated. Various types and degrees of dysplastic features were observed in various combinations, including architectural abnormalities, an increased number of neurons in the molecular layer and/or cortical layer II, neuronal clustering, an increased number of satellite oligodendrocytes, abnormal gyration, single and/or aggregates of heterotopic neurons in the white matter, and the appearance of cytologically abnormal cells, such as giant or dysmorphic neurons and balloon cells. In the temporal lobe specimens, microdysgenesis (corresponding to mild malformations caused by abnormalities of cortical development and type IA/B focal cortical dysplasias) was more frequently observed than Taylor-type focal cortical dysplasia (type IIA/B), whereas in the frontal lobe specimens, the frequency of occurrence of both types was even. The ages at seizure onset and surgery of patients with the latter type were significantly lower than those of patients with the former. On the other hand, prominent astrocytosis in the cortex and white matter was evident in all cases, and many corpora amylacea and neurofibrillary tangle-like inclusions were observed in a subset of cases. An ultrastructural investigation revealed dilatation of the postsynaptic dendritic spines and shafts in the cortex and features indicating the occurrence in the white matter of demyelination followed by remyelination. Thus, with regard to the epileptogenic lesions, although dysplastic changes constitute the pathogenetic basis, the overlapping subsequent degenerative processes involving synapses, dendrites, and axons might contribute to the development of epileptogenic processes. astrocytes might also actively participate in the development of the pathogenesis of epilepsy.
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