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)

Four embryonal rhabdomyosarcomas, one tumor diagnosed as an undifferentiated sarcoma, probably a rhabdomyosarcoma, and six different non-muscular sarcomas were investigated with antibodies specific for different intermediate filament types. The tumor cells in the rhabdomyosarcomas and the undifferentiated tumor were stained clearly by antibodies to desmin, the intermediate filament type characteristic of muscle. The staining of tumor cell by antibodies to vimentin, the intermediate filament type characteristic of certain cell types of mesenchymal origin including myoblasts, was different in these 5 cases. In one case of embryonal rhabdomyosarcoma nearly all tumor cells were stained, but in the remaining cases few or no tumor cells were positive with the vimentin antibody. In these rhabdomyosarcomas not only the large rhabdomyoblasts, but also the small undifferentiated cells were labeled by antibodies to desmin. In the latter cell type the desmin filaments were arranged typically in coils. In contrast, tumor cells in the non-muscular mesenchymal sarcomas were stained only by antibodies to vimentin but not by antibodies to desmin or prekeratin. The retention of the desmin marker characteristic of normal muscle in cases of rhabdomyosarcoma not only allowed the undifferentiated desmin-positive sarcoma to be classified as rhabdomyosarcoma but also suggests that the use of antibodies to desmin could be very helpful in the future for the diagnosis of undifferentiated rhabdomyosarcomas.
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PMID:Diagnosis of human childhood rhabdomyosarcoma of antibodies to desmin, the structural protein of muscle specific intermediate filaments. 617 91

Combination therapy consisting of vincristine, actinomycin-D, and cyclophosphamide with or without adriamycin is the most common chemotherapy for rhabdomyosarcoma in childhood. But the effective chemotherapy for rhabdomyosarcoma resistant to these four drugs has not been established. We report a case with rhabdomyosarcoma, which was resistant to these four drugs but responded completely to three drug combination chemotherapy consisting of vinblastine, cis-platinum, and bleomycin (VPB therapy). A 11-months-old boy was referred to us because of giant abdominal tumor. Postoperative diagnosis was Group III embryonal rhabdomyosarcoma of the urinary bladder. Partial resection was followed by vincristine, actinomycin-D, cyclophosphamide, and adriamycin, but his residual tumor was growing. Then VPB therapy was administered and the first course of the chemotherapy reduced the size of tumor. After three courses of VPB therapy the second-look operation was performed. At operation no residual tumor was found and a complete remission was confirmed. During the course of VPB therapy no severe adverse effect was detectable.
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PMID:[Rhabdomyosarcoma of the urinary bladder: complete remission induced by vinblastine, cis-platinum, and bleomycin]. 619 95

A case of rhabdomyosarcoma of the urinary bladder in an 11-month-old boy is presented. The chief complaint was complete urinary retention and histological examination showed embryonal rhabdomyosarcoma of the bladder. Initially, he was treated with vincristine, actinomycin-D and radiation therapy. This therapy was not effective, and he was next treated with vincristine, bleomycin, and cis-DDP. This therapy was significantly effective, and the tumor became non-palpable on physical examination. After six courses he was discharged and maintenance chemotherapy was continued until the tumor relapsed 1 year later. Finally he died of dyspnea due to diffuse pulmonary metastases approximately 2 years after the first diagnosis. We herein discuss several points which affect the prognosis and the usefulness of chemotherapy, especially combination therapy with vincristine, bleomycin, and cis-DDP for recurrent cases.
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PMID:[Rhabdomyosarcoma of the bladder in a child: report of a case]. 620 68

Two permanent, transplantable strains of rhabdomyosarcoma (SAS) and synovial sarcoma (KUSHI) were established by transplanting them serially into nude mice. The original SAS tumor and its transplant were histologically identical and gave an appearance of so-called embryonal rhabdomyosarcoma. The KUSHI tumor changed after the second passage, showing the biphasic pattern of synovial sarcoma, while undifferentiated myoblast-like cells were observed throughout the original tumor. Three in vitro cell lines from the SAS and one from the KUSHI tumor were successfully established. The human origin of the SAS and KUSHI transplanted tumors could be verified by the presence of human chromosomes. The cells cultured from the SAS tumor were more sensitive to anticancer drugs than those of the KUSHI tumor. However, as the number of serial passages on nude mice increased, the sensitivities of the SAS cells decreased, while those of the KUSHI cells increased. The modal chromosome number of the SAS tumor changed from 51 to 49 to 46 during serial passages on nude mice. These observations suggest that the sensitivities to anticancer drugs not only differ among soft tissue sarcomas of different histologic types, but also change continuously during the growth of the tumor itself.
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PMID:Characteristics of human soft tissue sarcomas in xenografts and in vitro. 629 38

A case of testicular teratoma metastasized to the retroperitoneum and after cytoreductive chemotherapy was noted to contain areas of frank sarcoma. Sarcomatous areas included embryonal rhabdomyosarcoma with a pattern of sarcoma botyroides, alveolar rhabdomyosarcoma, and fibrosarcoma. These areas differed markedly from areas of immature teratoma, which composed the remainder of the retroperitoneal lesion and which also characterized the primary tumor. These sarcomatous areas were characterized by numerous mitoses, marked cellular pleomorphism and diagnostic histologic, ultrastructural, and immunocytochemical features. Residual germ cell tumors following cytoreductive chemotherapy are traditionally categorized as teratoma only or teratoma with embryonal carcinoma or choriocarcinoma for therapeutic and prognostic reasons. This case does not conform to this simple categorization and raises serious questions concerning subsequent therapeutic decisions.
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PMID:Sarcoma arising in a residual testicular teratoma after cytoreductive chemotherapy. 647 36

With the development of combined chemotherapy and radiation therapy for embryonal rhabdomyosarcoma, the role and extent of surgical resection of these pelvic tumors need to be defined. Thirty-six children with pelvic genitourinary rhabdomyosarcoma seen at St. Jude Children's were managed on protocols combining surgical resection and radiation, and chemotherapy. Ten children presented with cervical-vaginal tumors, which were managed with combined therapy; the surgical resection was histovaginectomy in eight and pelvic exenteration in one. Eight of the ten are free of disease from 1 to 14 years. Twelve children presented with bladder and prostate tumors, which were resected with segmental cystectomy in four cases, biopsy in five, and pelvic exenteration in three. All received combination therapy and six of the twelve are surviving free of disease from 6 months to 16 years. Fourteen children presented with paratesticular rhabdomyosarcoma. Chemotherapy was combined with radical orchiectomy in all cases. Retroperitoneal node dissection was done in nine and five had inguinal node dissection. Nine of the 14 are surviving NED from 2 to 16 years. One patient died, free of disease, with complications of combination therapy. The results of this review supports the approach of combining chemotherapy, radiation, and complete surgical resection.
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PMID:The role of surgical resection when combined with chemotherapy and radiation in the management of pelvic rhabdomyosarcoma. 672 1

Sixteen autopsy cases of the primary sarcomas of the mouth and jaws were reviewed, which included 1 chondrosarcoma, 3 osteosarcomas, 1 leiomyosarcoma, 7 rhabdomyosarcomas and 4 histiocytic lymphoma (reticulum cell sarcoma). Although the case materials studied were too small, the pattern of the spread of the sarcomas of the mouth and jaw was likely to be divided into two groups, localized and generalized. The former is comprised of chondrosarcoma osteosarcoma, leiomyosarcoma and pleomorphic rhabdomyosarcoma. The latter is comprised of alveolar rhabdomyosarcoma and histiocytic lymphoma. Embryonal rhabdomyosarcoma belongs to either depending on the case. The former, being resistant to local therapy, tended to recur with a contiguous spread but was slow to metastasize. Metastases were limited to the lung and pleura through the hematogenous route. The primary sarcoma of the latter group were not so extended in local spread and occasionally cured with therapy. However, metastases, both hematogenous and lymphogenous, were generalized.
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PMID:Sarcomas of the mouth and jaws--a study of 16 autopsy cases. 675 65

The main problems in the diagnosis of rhabdomyosarcoma are 1) distinction of undifferentiated examples from other small cell malignancies, especially soft-tissue Ewing's tumor and lymphoma; 2) distinction of spindling examples from fibrosarcoma, leiomyosarcoma, malignant fibrous histiocytoma, polyhistioma, and other sarcomas; 3) recognition of minimal criteria on small samples such as needle biopsy specimens or frozen sections; and 4) recognition of rhabdomyosarcoma in uncommon sites such as bone (mandible), perineum, retroperitoneum, and chest. In 95 pediatric cases diagnosed and treated at Royal Alexandria Hospital for Children--45 after the introduction of combined therapy--minimal diagnostic criteria were assessed. Cross-striations were found in only one third of cases; longitudinal myofibrils were more common and more helpful. There was much overlap between histologic types, and the microscopic patterns had little bearing on prognoses in preadolescent children. Fourteen cases could not be further differentiated ("embryonal sarcoma, probably rhabdomyosarcoma")--nine small-cell tumors; four tumors from genitourinary tract or head for which very small biopsy specimens were available, and one spindling retroperitoneal neoplasm. In all, slight evidence suggested embryonal rhabdomyosarcoma; this evidence included oat-shaped nuclei and, in a few cells, deeply eosinophilic cytoplasm, small elongated processes, or myxoid or alveolar foci--features that exclude lymphoma and Ewing's tumor. In six cases that were originally classified as poorly differentiated or undifferentiated, later material confirmed the presence of rhabdomyosarcoma by showing a predominantly well-differentiated (pleomorphic) or alveolar pattern after therapy. In 14 remaining undifferentiated cases, immunoperoxidase staining with antihuman-myoglobin serum was positive in five. With combined therapy there was 100 per cent survival among patients with paratesticular, limb, and stage I and stage II tumors; considerably improved survival among patients with head and neck, pelvic, and stage III tumors; and 100 per cent mortality among patients with intra-abdominal and stage IV tumors.
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PMID:Diagnosis and behavior of juvenile rhabdomyosarcoma. 686 31

From March 1973 to December 1981 embryonal rhabdomyosarcoma of the orbit was diagnosed in 5 children. In two children exenteration was done after a previous local radiotherapy. In one child the exenteration of the orbit was done after a primary chemo- and radiotherapy because of local progression of the tumor. In two children the exenteration was avoidable by this therapy. In four of these five children this was followed by vincristine, actinomycin D and cyclophosphamide for 16 to 24 months. After the diagnosis these four children survived until now from 3 1/2 to more than 8 years. They are free of treatment and free of disease. Seven months after diagnosis one child died with an acute respiratory distress syndrome (at post mortem: hyaline membranes). In patients with rhabdomyosarcoma of the orbit it is justified to avoid the mutilating exenteration by primary chemotherapy followed by irradiation of the reduced manner. If exenteration becomes subsequently necessary the chance for survival is not diminished in our opinion.
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PMID:[Treatment of orbital rhabdomyosarcoma (author's transl)]. 709 86

A correlative, histologic and cytologic study of seven cases of alveolar rhabdomyosarcoma and chromosome analysis using the G-banding technique (in case 1) is presented. The histologic pattern was characterized by pseudo-alveolar structures, separated by fibrous septa. Primitive, dark, round cells predominated, but differentiated rhabdomyoblasts were found in all cases, and so were multi-nucleated giant cells. Unequivocal cross striation was seen in two cases. Cytologically, the tumors were highly cellular and small, undifferentiated tumor cells predominated. Large polymorphous, sometimes multi-nucleated, cells often with an elongated cytoplasm and eccentric nucleus were observed in all cases. The cell-types found could be well correlated to those identified in the histologic sections. The cytogenetic study revealed a karyotype profile, showing three 2q markers and only one chromosome type No. 13. The observations are different from the karyotype profile of embryonal rhabdomyosarcoma. However, further studies are necessary to elucidate this question.
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PMID:Alveolar rhabdomyosarcoma: a cytogenetic and correlated cytological and histological study. 714 52


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