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)

A clinicopathologic study of 15 cases of malignant neuroepithelioma (peripheral neuroblastoma) of soft tissues is reported. The patients were chiefly young Japanese adults with a median age of 21 years. The tumors arose mainly in the soft tissues of the lower extremity (seven cases) and the trunk (four cases). Microscopically, there were sheets of closely packed, small round or oval cells, and Homer Wright-type rosettes were seen in all cases, one of which also had Flexner-type rosettes. Immunohistochemical cytoplasmic localization of neuron-specific enolase (NSE) was demonstrated in six of the eight cases, using the peroxidase-antiperoxidase (PAP) method. In no case, however, was there any staining reaction for S-100 protein. Of the 14 patients for whom follow-up information could be obtained, nine died within a period of 2 years and two were alive and well for over 5 years after the initial treatment. Differential diagnosis from other soft-tissue round-cell sarcomas, such as embryonal or alveolar rhabdomyosarcoma, extraskeletal Ewing's sarcoma, and others, are briefly discussed, on a clinicopathologic basis.
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PMID:Malignant neuroepithelioma (peripheral neuroblastoma). A clinicopathologic study of 15 cases. 686 63

Retinoblastoma is a relatively rare malignant pediatric tumor accounting for approximately 3% of childhood cancers and 1% of all cancer deaths in children under 15 years of age. During the clinical course of the disease, a metastasis usually occurs within the first year of diagnosis and is seen in 2% of retinoblastoma patients. Metastases to the intracranial region are common and account for approximately 50% of the metastatic cases. Metastasis to the soft tissue is very rare. Herein, we report a case of metastatic retinoblastoma presenting as a left shoulder soft tissue mass in a 14-year-old female with a 14-year history of familial bilateral retinoblastoma status post radiation therapy. In our case, the FNA cytology shows some features of the small round blue cell tumor group with inconspicuous Flexner-Wintersteiner or Homer-Right rosette formation. The unusual clinical presentation and morphology give rise to a diagnostic dilemma, with the differential diagnosis centering on the small round blue cell tumors such as lymphoma, rhabdomyosarcoma, nephroblastoma (Wilms' tumor), Ewing's sarcoma/PNET, and desmoplastic small round cell tumor. It also prompts concern for the development of a second primary tumor. The purpose of our study is to discuss the FNA cytology of metastatic retinoblastoma, its differential diagnoses, and the utility of immunohistochemistry. An accurate diagnosis is imperative due to the differences in prognosis and treatment implications for the various diseases.
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PMID:Metastatic retinoblastoma presenting as a left shoulder soft tissue mass: FNA findings and review of the literature. 1993 45