Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1261473 (sarcoma)
 25,952 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Alveolar soft part sarcoma is primarily treated by surgery, and reports of effective chemotherapy have been infrequent. We experienced a patient with alveolar soft part sarcoma in the left thigh, in whom preoperative chemotherapy with cisplatinum (CDDP) and adriamycin (ADM) was effective. This patient was a 13-year-old male who had an elastic hard mass with the size of his fist in the medial thigh. Plain X-ray revealed bone destruction with periosteal reactions in the distal diaphysis of the femur. Biopsy specimen showed alveolar growth of relatively large cells with eosinophilic or clear cytoplasm. PAS staining was positive in microgranular patterns in part of the cells, but no crystalline structure were observed. A diagnosis of alveolar soft part sarcoma was made, and chemotherapy was started. After administration of 150 mg of CDDP intraarterially twice and 50 mg of ADM intravenously 5 times, effectiveness of chemotherapy was estimated by physical and radiological findings. In the resected specimen, the margins of the soft part tumor showed shell-like ossification, suggesting the subperiosteal or intraosteal origin. Histology of the removed specimen showed that the tumor was mostly replaced by fibrous tissues, indicating the effectiveness of the preoperative chemotherapy. This disease has a relatively long course, however, the present case is considered to be another exceptional case that responded to preoperative chemotherapy.
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PMID:[A case of effective preoperative chemotherapy in alveolar soft part sarcoma]. 200 43

In this report a 13-year-old girl with characteristic clinical and pathological features of alveolar soft part sarcoma of the tongue is presented. Alveolar soft part sarcoma is a rare malignant tumor of unknown origin. Forty eight cases of alveolar soft part sarcoma have so far been reported in the head and neck region. Median survival reported for all sites of the body is 79 months following various modes of therapy. In this case, the treatment was surgical excision of the mass combined with radiotherapy and chemotherapy. The patient has been asymptomatic for the past 12 months.
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PMID:Alveolar soft part sarcoma of the tongue. 258 56

A case of alveolar soft part sarcoma of the nasal cavity is presented. Alveolar soft part sarcoma is a rare malignant neoplasm, which often affects females (ratio 2 to 1) in their second decade. It occurs most frequently in the extremities, with an unusual predilection to involve the right side of the body. Excluding the orbit, only a few cases have been reported in the head and neck area. We present the first reported case of alveolar soft part sarcoma limited to the nasal chamber.
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PMID:Alveolar soft part sarcoma of the nasal cavity: report of a case and a review of the literature. 305 74

Alveolar soft part sarcoma is an uncommon soft tissue tumor that has seldom been studied by cytologic methods. The cytomorphologic features of two cases of this sarcoma are described. To enhance diagnostic accuracy in a suspected alveolar soft part sarcoma, the authors present the differential diagnosis and the application of immunocytochemical procedures to cytologic specimens.
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PMID:Alveolar soft part sarcoma. A cytologic and immunohistochemical study. 327 81

Alveolar soft part sarcoma is a histologically distinctive neoplasm of uncertain histogenesis. Since its initial description in 1952, more than 200 cases have been reported. The extremities are most often the sites of involvement; the tongue, bones, and the orbit have been less commonly involved. The present paper describes a case of alveolar soft part sarcoma which was present only within the uterine cervix of a 37-year-old woman. Histologically, the tumor cells were arranged in the characteristic alveolar pattern; diagnostic PAS-positive diastase-resistant needle-shaped crystals were observed within the cytoplasm of the tumor cells. After the initial biopsy, the patient underwent a radical hysterectomy and pelvic lymph node dissection. Although no residual tumor was found within the cervix, a microscopic focus of tumor was detected in an obturator lymph node. The patient is at present clinically free of disease.
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PMID:Alveolar soft part sarcoma of the uterine cervix. 405 22

Alveolar soft part sarcoma is a soft tissue neoplasm of unknown histogenesis which has a distinctive morphology. It is a relatively rare tumor with approximately 200 cases described in the literature. Only two cases have previously been reported as occurring in the vagina. The purpose of this paper is to report the third case of alveolar soft part sarcoma occurring in the vagina and to emphasize the role of electron microscopy in the differential diagnosis.
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PMID:Alveolar soft part sarcoma of the vagina: an immunohistochemical and electron microscopic study. 405 22

Alveolar soft part sarcoma is a rare malignancy. To our knowledge, 13 cases in the head and neck have been reported previously. It occurs more commonly in the extremities, where it is associated with a poor prognosis. Its clinical course resembles that of adenoid cystic adenocarcinoma in that late metastases often cause death in 10 to 15 years. A 5-year-old girl with alveolar soft part sarcoma was successfully treated with partial glossectomy and is free of disease at two-year follow-up.
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PMID:Alveolar soft part sarcoma of the tongue. 663 26

Alveolar soft part sarcoma is a neoplasm that usually presents as a slow-growing mass located in an extremity and is most often initially discovered by the patient, who then seeks medical evaluation (1, 2). Local pain or symptoms produced by metastases as the initial manifestations of this neoplasm are unusual (2). A case is presented in which CT demonstrated multiple metastases to the central nervous system that produced headaches as the first symptoms in a patient with alveolar soft part sarcoma.
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PMID:Alveolar soft part sarcoma. 726 51

Alveolar soft part sarcoma of the tongue is a rare malignancy. Until 1988, only 23 cases have been documented in the English literature. In this report, a 21 year old man with typical features of alveolar soft part sarcoma of the tongue is presented. Treatment was wide surgical resection with reconstruction using a pectoralis major myocutaneous flap.
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PMID:Alveolar soft part sarcoma of the tongue: a case report. 831 6

Nine cases of alveolar soft part sarcoma were studied in terms of their immunoreactivity towards intermediate filaments and muscle markers, and their ultrastructure with regard to muscle features and the cytoplasmic crystals characteristic of this tumour. Inconsistent expression of alpha-smooth muscle actin, vimentin and desmin were found. No unambiguous muscle cell characteristics were identified by electron microscopy, although multiple subplasmalemmal densities with overlying lamina were emphasised as a typical but not specific muscle feature. Classical alveolar soft part sarcoma crystals at the ultrastructural level were absent throughout, but pseudo-crystals with a pre-crystalline finely filamentous content were numerous in one case. The results were integrated into a comprehensive comparison with the literature. It is clear that immunohistochemical evidence of striated muscle differentiation is detectable in some but not all cases of ASPS. The detection of Myo D1 protein in the small numbers of ASPSs so far studied for this gene product raises the possibility that in the majority of ASPSs this is the only evidence of early striated muscle differentiation and that only in the minority is it able to induce desmin production. Study of a substantial series for Myo D1 is required to explore this possibility.
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PMID:Immunophenotype and ultrastructure of alveolar soft part sarcoma. 840 38


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