Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thirty-two patients with advanced, inoperable nonhematologic soft-tissue and osseous sarcomas were treated with Methyl CCNU administered via controlled intravenous infusion in doses of 130-170 mg/m2 every 6 weeks in a Phase II trial. All 28 evaluable patients were no longer responsive to adriamycin. Greater than 50% tumor regression was seen in one of two patients with chondrosarcoma and one of five patients with rhabdomyosarcoma. Less than 50% tumor regression occurred in one of five patients with rhabdomyosarcoma, one of two patients with malignant giant cell tumor, and one of three patients with malignant fibrous histiocytoma. Stabilization of previously advancing disease occurred in two of seven patients with leiomyosarcoma. The drug preparation was well tolerated. Nausea and vomiting occurring in three of 32 patients. Major toxicity was myelosuppression, characterized chiefly by thrombocytopenia with lesser degrees of leukopenia. This drug preparation appears to have activity in this group of tumors.
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PMID:A phase II study of intravenously- administered methyl CCNU in the treatment of advanced sarcomas. 76 46

Rhabdomyosarcoma, the most common primary malignant childhood orbital tumor, is composed of neoplastic striated muscle cells (rhabdomyoblasts) in various stages of differentiation and in patterns suggestive of neoplastic analogs of normal muscle embryogenesis. Orbital rhabdomyosarcoma is most commonly seen in children and adolescents, the average age of onset of symptoms being 7.8 years. The tumor usually presents as a rapidly evolving exophthalmos, often associated with drooping of the upper eyelid. A mass is palapable in only 25% of cases, loss of central vision at the time of presentation is uncommon, and laboratory studies are often of little help in diagnosis. The best diagnostic aid is a high index of suspicion whenever one sees a rapidly progressive exophthalamos in a child. Orbital rhabdomyosarcoma is almost always of the embryonal type, believed to originate in the orbital soft tissues from undifferentiated pluripotential embryonic mesenchyme. In the past, orbital exenteration has been the primary therapy. Review of 162 literature cases of orbital rhabdomyosarcoma, generally treated by unassisted surgery, revealed that only 25% of the patients survived 3 or more years. Recently, it has been shown that radiation therapy, alone or combined with chemotherapy, can be successful. A multidisciplinary approach, utilizing surgery, radiation therapy and chemotherapy has also been advocated. Both approaches appear to offer greater survival than unassisted orbital exenteration. The possibility of primary radiation therapy is extremely promising; if it becomes increasingly effective, a mutilating surgical procedure may become obsolete.
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PMID:Ophthalmic striated muscle neoplasms. 79 29

The case of a child with a stage I perianal embryonal botryoid rhabdomyosarcoma is presented, with description of coordinated management utilizing local excision, irradiation, and cyclic chemotherapy resulting in a 3-year tumor-free survival. Previously reported pediatric cases are summarized.
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PMID:Perianal rhabdomyosarcoma in childhood. 82 44

Clinico-pathologic reviews of series of children with rhabdomyosarcoma have yielded conflicting information regarding frequency of lymphatic spread of this disease. The 264 eligible entries in the Intergroup Rhabdomyosarcoma Study (from November 1972 to September 1975) have been categorized by a prospective staging system and pre-treatment characteristics and pathologic findings reviewed. Data accumulated thus far have revealed a higher than expected incidence of lymphatic metastases from extremity (17%) and genito-urinary sites (19%) with a somewhat lower incidence from the orbit (0%), the head and neck region (3%), and trunk (10%). These differences in regard to lymphatic metastases were found to have no relationship to age, sex, tumor size, or histologic type when these factors were simultaneously examined.
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PMID:Lymphatic metastasis with childhood rhabdomyosarcoma. 83 39

The immunobiology of heterotransplanted human tumors was investigated following transplantation into nude mice of human bronchogenic, colon, rectal, ovarian, gastric, endometrial, vaginal, bladder, renal, esophageal, embryonic cell, pancreatic, and breast carcinoma, as well as fibrosarcoma, rhabdomyosarcoma, malignant melanoma, astrocytoma, Wilm's tumor, endometrial hyperplasia, and hydatidiform mole. Several of these tumors were passaged up to 15 generations. During these passages no changes in latency period for tumor development or in histology were noted. There were significant differences between several tumors in the minimum number of cells required for successful transplantation; such differences were independent of the basic biologic aggressiveness of the individual tumors. Nude mice that received transplants of fibrosarcoma and endometrial carcinoma had increased serum IgM and numbers of spleen cells and complement receptor lymphocytes. No such changes were noted for mice that received transplants of malignant melanoma, In contrast, there were no apparent differences in the responses of nude mice, who were given transplants of human tumors, to be T-cell mitogens concanavalin A or phytohemagglutinin or in the number of theta-bearing spleen cells. The success rate for transplantation was significantly improved when explants, rather than single-cell suspensions, were performed. Tumors transplanted to nude mice derived from strictly homozygous matings behaved like tumors transplanted to mice born of heterozygous mothers. Finally, despite the dramatic size of subcutaneous tumor nodules, there were no examples of invasion or distant metastases.
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PMID:Immunobiology of heterotransplanted human tumors in nude mice. 85 33

Pleomorphic carcinoma of the pancreas is a well defined histopathological entity characterized by non-cohesive, sarcoma-like growth pattern, and bizarre mono- and multinucleated tumor giant cells with abundant eosinophilic cytoplasm. Fifteen cases are identified in autopsy files of the Department of Pathology, Washington University School of Medicine, which represent 7.1% of all the non-endocrine pancreatic malignancies found at autopsy. Pleomorphic carcinoma is comparable to pancreatic adenocarcinoma in clinical features such as age, sex, and presenting symptoms except that it is more likely to occur in the body and tail of the pancreas, metastases invariably develop, hematogenous spread is more common, and the median survival is worse. Pleomorphic carcinoma could be distinguished from the pancreatic tumors that resemble giant cell tumor of the bone. Differential diagnostic features between it and amelanotic melanoma, hepatocellular carcinoma, choriocarcinoma, pleomorphic liposarcoma, pleomorphic rhabdomyosarcoma, fibroxanthosarcoma, poorly differentiated epidermoid carcinoma, and giant cell carcinomas of the lung and thyroid are discussed.
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PMID:Pleomorphic carcinoma of the pancreas: an analysis of 15 cases. 87 Jan 68

Thirty-nine previously untreated children with rhabdomyosarcoma were managed by a coordinated program of surgery, radiation therapy, and chemotherapy during the years 1960 to 1973. The primary tumor was located in the head and neck (24), chest wall (1), abdomen (1), pelvis (10), and lower extremity (3). Radiation therapy consisted of tumor doses of 5000 to 6000 rads delivered in five to six weeks. Combination chemotherapy with actinomyctin-D, vincristine and cyclophosphamide was used after 1968. Seventeen of 25 cases (68%) treated after 1968 are alive two to five years following treatment. Only four of 14 cases (29%) who received less radical therapy before 1968 are alive. A relatively high incidence of local failure (23%) was noted in spite of adequate doses of radiotherapy. None of the four cases with metastatic disease at the time of diagnosis survived. Major complications were mainly noted in patients with orbital rhabdomyosarcoma. Correlation of absolute survival with dose of irradiation, primary site, extent of disease and histologic subtypes of the tumor are discussed.
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PMID:Combined treatment modalities of rhabdomyosarcoma in children. 87 41

Rhabdomyosarcoma of the urinary bladder in adults is exceedingly rare. Reported cases have been of the embryonal botryoid type, as seen in children. We will describe a case of pleomorphic rhabdomyosarcoma occurring in the urinary bladder of an adult. In this case, we noted striking intraepidermal migration of tumor cells, resembling the epidermotropic growth characteristic of tumor cells in Paget disease of the breast. The pathological features of this apparently unique case form the subject of this report.
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PMID:Rhabdomyosarcoma of the urinary bladder with intraepithelial spread in an adult. 94 59

A case is reported of a primary rhabdomyosarcoma occurring in the right subfrontal region of a 16 year old girl. The patient suffered from dull frontal headache and proptosis for three months before hospitalization. The circumscribed and demarcated neoplasm involved the dura mater, and invaded the frontal sinus and roof of the orbit on the same side. The pathogenesis of the tumour is thought to be related to aberrant differentiation of unstable mesenchyme. A suggestion is made that the "medullomyoblastoma" should be classified as a type of neoplasm of mixed mesenchymal and neuroepithelial origin.
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PMID:Primary intracranial rhabdomyosarcoma producing proptosis. 95 May 63

A case of pleomorphic rhabdomyosarcoma of the larynx occurring in a 33-year-old male is reported. The clinical and histopathological aspects of this undoubtedly rare neoplasm are described. After a quick review of the most important classifications of rhabdomyosarcoma so far made, the authors suggest a division into two basic groups, i.e. pleomorphic and fetal rhabdomyosarcoma, while the botryoid sarcoma and the alveolar rhabdomyosarcoma should be considered only as two subgroups of fetal rhabdomyosarcoma. From a comprehensive review of world literature on the subject, the well-documented cases up to now described are only seven, including the authors. The present case is the third one after the instances of pleomorphic rhabdomyosarcoma reported by Filipo and Crifo (1964 and by Rodriquez and Ziskind (1970).
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PMID:Pleomorphic rhabdomyosarcoma of the larynx. A case report and review of the literature. 95 8


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