Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Malignant primary cardiac tumors have uniformly been associated with poor long-term survival. We reviewed data on 11 patients with such tumors seen between 1956 and 1986. There were five men and six women, with a mean age at presentation of 44 years. Nine (82 percent) of 11 patients presented initially with respiratory symptoms, and 45 percent (five) presented with chest pain. No patient was asymptomatic on initial presentation. Forty-five percent (five) of the patients had elevated jugular venous pressure or a systolic murmur. Cardiomegaly was present in eight (73 percent) of 11 patients. Nine of ten patients had a hemoglobin level less than 12 g/100 ml, and 30 percent (3/10) presented with thrombocytosis. Seventy percent (eight) of the patients underwent surgical biopsy, and in 30 percent (three), surgical excision of the tumor was attempted. Angiosarcoma was the most frequent type of tumor (four patients), followed by malignant fibrous histiocytoma (three patients), mesothelioma (two patients), rhabdomyosarcoma (one patient), and primary lymphoma (one patient). Long-term follow-up data reveals that ten (91 percent) of 11 patients had died, with a mean duration from diagnosis until death of 9.7 months. Newer pathologic techniques for identification of sarcomas, aggressive surgical resection, and advanced chemotherapy for certain subgroups may contribute to better survival for patients with these tumors in the future. In addition, cardiac transplantation may play an important role in the future for those patients with localized disease.
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PMID:Malignant primary cardiac tumors. The Cleveland Clinic experience, 1956 to 1986. 366 1

Rhabdomyosarcoma is the most common soft tissue sarcoma in adolescence and childhood, which manifests by the locally destructive growth of the primary tumor or its metastases. We report on a 29-year-old man with an alveolar rhabdomyosarcoma presenting with an unusual leukemia-like picture. On admission, the patient suffered from diffuse bone pain and renal insufficiency. Peripheral blood analysis showed anaemia, thrombocythaemia and blast-like cells. A bone marrow aspirate revealed extensive infiltration by atypical blast-like cells which were interpreted as acute lymphoblastic leukemia. Although confirmation of this diagnosis by immunophenotyping did not succeed chemotherapy was started immediately and led to partial remission. Histologic analysis of a bone marrow biopsy from the iliac crest, however, revealed an extensive solid tumor with alveolar spaces, lined by primitive round cells with positive PAS-reaction in the cytoplasm. Immunostaining demonstrated a positive reaction of the tumor cells for desmin and in a few tumor cells for smooth-muscle-actin. Chromosomal analysis showed a t(2;13) translocation typical for alveolar rhabdomyosarcoma. Although multiple lytic lesions of the skeletal system became evident during the further clinical course, the site of origin of the primary tumor could not be defined retrospectively. In conclusion, rhabdomyosarcoma should be included in the differential diagnosis of systemic diseases with extensive bone marrow infiltration by tumor cells that could otherwise be misinterpreted as a haematologic malignancy.
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PMID:[Alveolar rhabdomyosarcoma presenting as acute leukemia]. 1009 56