Cases reported "Sclerosis"

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1/71. Occult sclerosing carcinoma of the thyroid.

    A case of occult sclerosing thyroid carcinoma is reported. The primary requirement for diagnosis is that the cancer is less than 1.5 cm in diameter. In addition, a fibrosing component with varying degrees of sclerosis is present. Lymph node metastases are frequent and may appear as benign thyroid follicles. Occasional direct invasion or vascular invasion may occur. Distant metastases were not reported in the series reviewed. Adequate therapy would seem to be total thyroidectomy of the involved lobe and radical subtotal thyroidectomy of the opposite lobe with excision of all enlarged or involved lymph nodes.
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ranking = 1
keywords = cancer
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2/71. 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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ranking = 1
keywords = cancer
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3/71. Sclerosing hyaline necrosis of the liver in bloom syndrome.

    bloom syndrome is a rare autosomal recessive disorder characterized by normally proportioned but strikingly small body size, a characteristic facies and photosensitive facial skin lesion, immunodeficiency, and a marked predisposition to development of a variety of cancers. We describe here, we believe for the first time, pronounced sclerosing hyaline necrosis with mallory bodies in the liver of a patient with bloom syndrome. mallory bodies are cytoplasmic eosinophilic inclusions, which are more common in visibly damaged, swollen hepatocytes in various liver diseases but are never found in normal liver. The possible pathogenesis of this finding in bloom syndrome is discussed.
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ranking = 1
keywords = cancer
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4/71. Sclerosing Mucoepidermoid carcinoma with eosinophilia of the thyroid glands: a case report with clinical manifestation of recurrent neck mass.

    Sclerosing mucoepidermoid carcinoma with eosinophilia (SMECE) is a recently recognized malignant neoplasm of the thyroid gland. About 14 cases of SMECE have been reported and this is the first reported case in korea. A 57-year-old woman presented with right neck mass for 20 years. Total thyroidectomy was performed under the impression of thyroid carcinoma. The resected thyroid gland showed a poorly circumscribed hard mass. Histologically, the tumor consisted of solid nests of large atypical cells with dense fibrous stroma. The tumor cells showed squamoid appearance with abundant eosinophilic cytoplasm. There were also rare mucin-containing cells within the nests. Within the hyalinized stroma, numerous eosinophils were found. The surrounding thyroid parenchyma displayed Hashimoto's thyroiditis. There was metastasis in a regional lymph node. Two years after initial surgery, she underwent a modified radical neck dissection due to recurrent neck mass. After the radiation therapy for eight weeks, laryngectomy and esophagectomy were performed due to a recurrent carcinoma in the esophageal wall. We report an additional case of SMECE, with metastasis to regional lymph nodes and esophagus. The tumor appears to be more aggressive than previously reported and a correct diagnosis can be rendered by just examining the metastatic lesions.
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ranking = 2.347702356818
keywords = neoplasm
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5/71. Chronic sclerosing sialadenitis of the submandibular gland associated with idiopathic retroperitoneal fibrosis.

    We report a case of a 57-year-old man who developed a fibrosclerosing lesion in the submandibular gland and idiopathic retroperitoneal fibrosis (IRF) involving the unilateral periureteral region within a year. Both lesions were resected surgically because of the suspicion of neoplasm. Pathologic examination revealed similar histologic and immunohistochemical features for both lesions, namely, fibrosclerosis with prominent hyalinizing collagen bundles and proliferation of myofibroblastic cells, and a non-neoplastic reactive nature. There was infiltration by lymphocytes with prominent lymph follicles, plasma cells and macrophages. The histologic and immunohistochemical findings suggest that the two lesions were of a similar pathogenesis, which was possibly mediated by macrophages. We think that the present case may be an unusual form of multifocal fibrosclerosis. Although sialolithiasis is thought to be a major pathogenic factor for chronic sclerosing sialadenitis of the submandibular gland, the present case suggests that certain cases might have an etiology similar to IRF.
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ranking = 2.347702356818
keywords = neoplasm
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6/71. In situ demonstration of parathyroid hormone-related protein mRNA in sclerosing hepatic carcinoma.

    A 69-year-old man had a hepatic tumour occupying the left and half of the right lobe, with portal vein thrombus. There were hypercalcaemia and hypophosphataemia with increased nephrogenous cyclic adenosine monophosphate; bone metastases were excluded. serum parathyroid hormone-related protein (PTHrP) was elevated, but no increase in intact parathyroid hormone (PTH) or vitamin D3 metabolites was found. At autopsy the histological features were typical of sclerosing hepatic carcinoma. By immunohistochemistry PTHrP was detected in cancer cell nests but not in the fibrous stroma. PTHrP transcripts were demonstrated by in situ hybridization using a polymerase chain reaction (PCR)-derived single-stranded dna probe. Tumour cells expressed AE1 and CA19-9 (markers for cholangioepithelium) and CEA (for bile canaliculi). Electron microscopy revealed microvilli on the apical surface, and secretory granules 100 nm in diameter were observed. These findings indicate that this case is one of cholangiocellular sclerosing hepatic carcinoma. The interaction between cancer and stromal cells may be the cause of PTHrP overexpression.
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ranking = 2
keywords = cancer
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7/71. Expression of CD34 in sclerotic ("plywood") fibromas.

    CD34 antigen is expressed in normal human skin on endothelium, in spindle cells located around adnexal structures, and in a subset of interstitial cells in the reticular dermis. CD34 expression has also been identified in a number of fibrohistiocytic neoplasms, such as dermatofibrosarcoma protuberans and solitary fibrous tumors of soft tissue. CD34 expression has not previously been described in sclerotic, or "plywood" fibromas. Here presented are three lesions from three patients, in which histologic examination revealed a well-circumscribed dermal nodule composed of spindled cells with focal nuclear pseudo-inclusions. There was extensive fibrosis with hypocellular, storiform areas, characteristic of sclerotic fibroma. The spindled cells strongly expressed CD34, but not factor xiiia or markers of melanocytic, neural, or muscular differentiation. A diagnosis of Cowden syndrome was considered in one of the cases. These cases provide evidence that CD34 expression can occur in sclerotic fibromas, either solitary or associated with Cowden syndrome. When diagnosing a sclerotic fibroma, one should comment in the report regarding the possibility of Cowden syndrome.
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ranking = 2.347702356818
keywords = neoplasm
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8/71. Histopathologic diagnosis of superficial soft tissue tumors, related lesions, and simulators: an algorithmic approach based on colors: tumors with predominance of pink.

    An algorithmic approach based on colors for histopathologic diagnosis of soft tissue tumors and their simulators is proposed. At scanning magnification, in specimens stained with hematoxylin and eosin, mesenchymal tumors can be classified according to their color. The color of a tumor is basically determined by density, morphology, and distribution of neoplastic cells, and by density and quantity of the stroma. The basic colors that can be observed by neoplasms stained with hematoxylin and eosin are white, pink, red, and blue. Colors may be used as a first step in choosing the algorithm for specific diagnosis of a given mesenchymal neoplasm. Furthermore, colors may be helpful in understanding the histogenesis of a tumor, and this is especially important in soft tissue pathology, because criteria for benignancy and malignancy vary according to the nature of the neoplasm. In this article, tumors with a predominance of pink are analyzed. Pink tumors are composed of cells with abundant eosinophilic cytoplasm and sparse chromatin in their nuclei, such as leiomyoma. In other cases, the pink color in a tumor is determined by abundant fibrous stroma rather than by characteristics of neoplastic cells, such as sclerotic fibroma.
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ranking = 7.0431070704539
keywords = neoplasm
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9/71. Sclerosing, pseudovascular rhabdomyosarcoma in adults. Clinicopathological and immunohistochemical analysis of three cases.

    rhabdomyosarcoma in adults represents a rare soft tissue neoplasm which is seen most frequently in its pleomorphic subtype in this age group. Very rarely, clear cell and spindle-cell variants have been reported. In this study we describe three cases of rhabdomyosarcoma in adult patients, characterised by prominent hyaline sclerosis and a pseudovascular growth pattern. All cases were identified in the consultation files of one of the authors and routinely processed. Immunohistochemical studies were performed on paraffin sections with the alkaline phosphatase-antialkaline phosphatase method. The patients, two women and one man, were 40, 41, and 56 years old. One developed a deep-seated soft tissue mass in the left lower leg, and one, a tumour of the left upper jaw. In one patient a bone tumour in the proximal body of the sacrum without extension into soft tissues was seen. The patients were treated by wide excision, piecemeal excision and incomplete excision in one case each; additional radiotherapy was performed in all three cases, and chemotherapy in two patients. In one patient multiple pulmonary metastases were noted, which showed progression despite systemic chemotherapy. Histologically, the neoplasms were composed of round/polygonal and spindle-shaped tumour cells including typical rhabdomyoblasts. In all cases a pseudovascular pattern and prominent hyaline sclerosis of the intercellular matrix was seen. Immunohistochemically, tumour cells stained positively for desmin and muscle actin (HHF35) and also for markers of striated muscle differentiation (myogenin, MyoD1, fast myosin). In this paper an unusual morphological variant of rhabdomyosarcoma arising in adult patients is described, which should be added to the morphological spectrum of these neoplasms.
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ranking = 7.0431070704539
keywords = neoplasm
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10/71. Sclerosing epithelioid fibrosarcomas involving the neuraxis: report of three cases.

    OBJECTIVE AND IMPORTANCE: Sclerosing epithelioid fibrosarcoma (SEF) is a rare mesenchymal neoplasm composed of rounded, vimentin-immunoreactive tumor cells disposed in nests and cords within a hyalinized collagenous matrix. Most examples arise in the deep skeletal muscles of adults. The cases recorded to date have been characterized by protracted clinical evolutions with a tendency for stubborn local recurrence, followed by late metastasis. Accordingly, SEF has been regarded as a low-grade sarcoma. A single instance of brain and vertebral metastasis has been described. We report three examples of SEF distinguished by primary involvement of the neuraxis at initial presentation. CLINICAL PRESENTATION: Two tumors had intracranial, calvarial and extracalvarial, soft-tissue components, whereas the third tumor manifested as a paraspinal mass with extension into the T12-L1 neural foramen and invasion of the T12 nerve root. INTERVENTION: All three affected patients experienced local recurrence and distant metastasis after resection of the primary site. These complications appeared early in the disease course in two cases. In no case was there a response to adjuvant chemotherapy or radiotherapy. CONCLUSION: Our experience indicates that SEFs arising along the neuraxis may demonstrate unexpectedly aggressive clinical behavior, compared with those arising in the more typical location of deep skeletal muscles.
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ranking = 2.347702356818
keywords = neoplasm
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