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

Rhabdomyosarcoma (RMS) of the hepatobiliary system is extremely rare in adults. To our knowledge only three cases have been reported in the literature, all involving the gallbladder. The present case concerns a 40-year-old woman who presented with epigastric pain and obstructive jaundice and was found to have a fusiform, submucosal neoplasm in the common bile duct. Histologically, the tumor presented a diagnostic problem due to a predominant sclerotic growth pattern suggesting an epithelial tumor. Extensive sampling revealed a focal alveolar growth pattern with rhabdomyoblasts, although cross striations were not seen. Electron microscopy failed to demonstrate the characteristic thick myofilaments and/or Z-band material. The diagnosis was supported by strongly positive immunohistochemical staining for myoglobin and desmin; the keratin stain was negative. A subsequent supraclavicular metastasis showed the typical histology of an alveolar RMS. The histologic features of the primary tumor suggest that RMS in this location may be underrecognized due to regional similarities to either primary or metastatic infiltrating carcinomas.
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PMID:Rhabdomyosarcoma of the common bile duct in an adult. 351 54

A rare case of primary jejunal malignant mixed tumor arising in a 49-64-old Japanese male with von Recklinghausen's disease is reported. The patient, who had a past history of partial gastrectomy due to duodenal ulcer, was admitted with a complaint of epigastric pain. Upper gastrointestinal examinations showed a huge polypoid tumor located in the efferent loop of the gastrojejunostomy site. Because the tumor was strongly suggestive of leiomyosarcoma on histological examination of biopsy specimens, laparotomy was performed. The resected tumor measuring 10 X 7 X 7 cm was composed of adenocarcinoma admixed with various sarcomatous components, including rhabdomyosarcoma, osteosarcoma, and other sarcomas. Immunohistochemical analysis also supported this diagnosis. The features of this tumor closely resembled malignant mixed mullerian tumor of heterologous type that develops in female genital organs. It is well known that patients with von Recklinghausen neurofibromatosis have an increased incidence of mesenchymal tumors and malignant neoplasias, and therefore, it seems that there is a possible relationship between the histogenesis of this peculiar tumor and the genetic abnormality in this patient.
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PMID:Primary jejunal malignant mixed tumor in a patient with von Recklinghausen neurofibromatosis. 867 54

A rare case of carcinosarcoma (sarcomatoid carcinoma) with rhabdomyoblastic and osteoblastic differentiation occurring in the gastric remnant is reported. A 69-year-old Japanese man who had undergone a partial gastrectomy for a duodenal ulcer 30 years earlier, presented with anemia, epigastralgia, and an abdominal mass. The diagnosis of gastric carcinosarcoma was made based on the findings of endoscopic biopsies. The patient was thus scheduled to undergo a surgical operation, but he died of respiratory failure. At autopsy, a huge polypoid tumor measuring 20 x 18 x 8 cm was located on the greater curvature of the gastric remnant. Microscopically, the tumor consisted of intimately mixed tubular adenocarcinoma and heterologous mesenchymal elements containing rhabdomyosarcoma and osteosarcoma. Between these components, a morphological transition from the adenocarcinoma element to the sarcomatous element was observed. Ultrastructually, rhabdomyoblastic differentiation was confirmed in the sarcomatous areas. Immunocytochemical expressions of epithelial markers including epithelial membrane antigen and cytokeratins (35bH11 and 34bE12) were recognized not only in the carcinomatous cells but also in the sarcomatous cells. These findings suggest that carcinomatous cells appear to transform into cells with sarcomatous features.
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PMID:Gastric carcinosarcoma (sarcomatoid carcinoma) with rhabdomyoblastic and osteoblastic differentiation. 929 37