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)

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

Bone marrow biopsy was performed as part of the initial assessment in 74 patients with soft tissue sarcoma. Infiltration of the marrow by tumour was present in four cases, all from the group of 56 patients who had other evidence of metastatic disease, giving an overall incidence of 7%. The histological subtypes were pleomorphic rhabdomyosarcoma, angiosarcoma, synovial sarcoma and myxoid liposarcoma, of which the first three were high-grade tumours. Although it was not possible to determine whether response to chemotherapy was influenced by marrow involvement, haematological toxicity seemed excessive.
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PMID:Bone marrow involvement in adult soft tissue sarcomas. 629 16

Based on a review of 752 cases coded as soft tissue sarcomas, histologic features were analyzed for a differential diagnosis, dividing the sarcomas in the first diagnostic step into purely descriptive groups, regardless of biologic behavior: spindle cell, small cell, pleomorphic and myxoid varieties. The tumor categories mainly discussed in order were fibrosarcoma, leiomyosarcoma, malignant schwannoma, synovial sarcoma, malignant fibrous histiocytoma, embryonal and alveolar rhabdomyosarcoma, malignant neuroepithelioma , extraskeletal Ewing's sarcoma, and myxoid and other types of liposarcoma. The discussion was focused on the problems we were facing often in the histologic diagnosis of these tumors, emphasizing in particular those microscopic features which were helpful in differential diagnosis. Principal clinical features, important for an accurate diagnosis, were added to the histopathologic considerations in each item, together with prognosis of all these Japanese patients, regardless of the stages. Other benign and malignant lesions confused occasionally with the soft tissue sarcomas were also listed. With the aid of electron microscopy and immunohistochemistry, sometimes each differentiating feature can be identified but these methods are not always contributory to a diagnosis. Routine light microscopy with or without standard histochemical techniques remains essential to avoid pitfalls when attempting to diagnose the tumor.
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PMID:Diagnosis of soft tissue sarcomas. 632 68

The case material was collected from 3 Institutions with a total of 441 patients: 217 with primary bone tumor and 224 with soft tissue sarcomas. In the majority of patients lymphography was performed during the initial diagnostic workup. The lymphograms were interpreted as negative or positive for metastases. In bone tumors, the incidence of metastases was 21%, ranging from 28% for osteosarcoma to 18% for Ewing's sarcoma and 13% for chondrosarcoma. In tumors of the soft tissue, the frequency was somewhat higher (28%), with special regard to rhabdomyosarcoma (53%), anaplastic sarcoma (67%), neurogenic sarcoma (42%) and synovial sarcoma (35%). In the group of bone sarcomas, primary hematogenous spread was 3 times more frequent than lymphogenous spread, while in soft tissue sarcomas, with a higher incidence of lymphatic spread, this finding was inverted. In the more consistent tumor groups, the occurrence of lymphatic metastases indicated a significant worsening of the prognosis. In 96 patients, lymph node biopsies were performed and the radiologic histologic correlation gave evidence of a 97.7% overall diagnostic accuracy.
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PMID:Lymphography in bone and soft tissue sarcomas. Experiences from three institutions. 723 66

The distribution of the neural cell adhesion molecule (N-CAM; CD56) was studied immunohistochemically in acetone-fixed frozen sections of 83 soft tissue tumors and selected normal mesenchymal tissue using Leu-19 monoclonal antibody and avidin-biotin peroxidase immunostaining. Positive N-CAM immunostaining was found in gastrointestinal and uterine cells but not in vascular smooth muscle cells. Normal skeletal muscle was negative, but atrophic muscle fibers within tumors were positive. Strong and consistent immunoreactivity was seen in nerves, pheochromocytoma, malignant schwannoma, and spindle cells, but not in epithelial-like cells of synovial sarcoma, hemangiopericytoma, benign leiomyoma, and rhabdomyosarcoma. Variable staining was seen in benign schwannoma and malignant fibrous histiocytoma. Limited, usually focal N-CAM immunoreactivity was seen in desmoid tumor, dermatofibrosarcoma, and leiomyosarcoma. Although the N-CAM is consistently seen in neural tumors, it is not cell lineage specific. Changes on malignant transformation (comparing corresponding benign and malignant lesions) do not show any consistent pattern. Rather, there is neoexpression of the N-CAM in rhabdomyosarcoma, whereas there is loss of the N-CAM in leiomyosarcoma. Immunohistochemistry of the N-CAM can be a useful adjunct for characterization of soft tissue tumors, and its possible correlation with tumor behavior has to be examined in further studies.
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PMID:Neural cell adhesion molecule distribution in soft tissue tumors. 767 94

We studied the clinical features, radiographic and pathological findings, treatment, and results for twenty-three patients who had been managed for a soft-tissue sarcoma of the hand between 1982 and 1990. The ages of the patients ranged from sixteen to seventy-six years (median age, thirty-one years). The most common clinical finding was a small, painless soft-tissue mass. Twenty of the tumors were high-grade, and eighteen were less than five centimeters in diameter. The most common diagnosis was synovial sarcoma, which was identified in eight patients. Leiomyosarcoma, rhabdomyosarcoma, and malignant fibrous histiocytoma developed in three patients each; epithelioid sarcoma, in two patients; and angiosarcoma, liposarcoma, neuroectodermal tumor, and clear-cell sarcoma, in one patient each. Curative wide excision or amputation was attempted in twenty-two patients; the margins were positive for tumor cells in eight, and local recurrence was seen in nine. Of the twenty-three patients, fourteen had survived, without evidence of disease, after a median duration of follow-up of forty-nine months, and nine had died of disease. The median rate of survival did not differ significantly on the basis of the size or grade of the tumor or the use of adjuvant treatment. However, the rate of survival of the patients who had a soft-tissue sarcoma of the hand that was less than five centimeters in diameter was significantly lower (p = 0.0008) than that of 152 patients who had a similar tumor at another site in an extremity.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Treatment of soft-tissue sarcomas of the hand. 771 73

A dose-intensive regimen of cyclophosphamide (140 mg/kg over 2 days), doxorubicin (Adriamycin, 75 mg/m2 over 3 days), and vincristine (1 mg/m2 on days 1, 2, and 3 and 1.5 mg/m2 on day 9) was tested in 18 children and adolescents with poor-prognosis recurrent or refractory solid tumors. Nine were affected by neuroblastoma, 3 by Ewing's tumors, 2 by rhabdomyosarcoma, 2 by synovial sarcoma, 1 by hepatocellular carcinoma, and 1 by osteogenic sarcoma. All enrolled patients were heavily pretreated, including 2 patients after bone marrow transplantation. Forty courses were applied (median, 2). The overall response rate was 33% (2 complete remissions and 4 partial remissions). Responses were obtained in children with neuroblastoma, Ewing's tumors, and hepatocellular carcinoma. Myelosuppression [World Health Organization (WHO) grade IV after all courses] and cardiac toxicity (3 WHO grade I, 5 WHO grade III, and 3 WHO grade IV) were the main side effects. Nephrotoxicity and hepatoxicity were not observed. With further therapy consisting of surgery, radiotherapy, and high-dose chemotherapy [cisplatin, carboplatin/etoposide (VP16), or ifosfamide/VP16 with or without autologous stem cell reinfusion after conditioning with melphalan/VP16/carboplatin], 3 complete remissions and 5 very good partial remissions were obtained. Ten of 18 patients are alive after a median follow-up of 16 months.
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PMID:High-dose cyclophosphamide, adriamycin, and vincristine (HD-CAV) in children with recurrent solid tumor. 785 84

An immunohistochemical study on frozen sections was carried out on 51 malignant tumours of soft tissue and bone using the FU-3 monoclonal antibody. This antibody is claimed to be specific for malignant fibrous histiocytoma (MFH) and liposarcoma and for normal and tumour cells located in perivascular fields. The results show a lack of specificity in MFH staining: several malignant tumours such as synovial sarcoma, fibrosarcoma, rhabdomyosarcoma, osteogenic sarcoma, and including an anaplastic malignant melanoma, presented positive staining somewhat similar to that found in MFH. The value of this antibody in the differential diagnosis of MFH is doubtful. It might be useful to recognize a common pathway of terminal differentiation expressed by several pleomorphic sarcomatous neoplasms.
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PMID:FU-3 monoclonal antibody: a specific marker for malignant fibrous histiocytoma? An analysis of 32 malignant soft tissue and bone sarcomas. 818 89

The spectrum of soft tissue tumours in young adults is very similar to that in more mature individuals, while those in childhood form a distinct group rarely seen outside the first decade. The majority of these are benign vascular or fibroblastic proliferations; in young children they may be highly cellular and mitotically active, but malignancy should be diagnosed with caution. Congenital soft tissue tumours constitute a special group in which the clinical outcome may be particularly difficult to predict from the histological appearances. This review focuses on those malignant soft tumours which are either peculiar to childhood or which manifest special features in childhood. Some recently recognized benign soft tissue tumours which occur mainly in childhood are also described. The fibromatoses are not discussed. As a guide to the appropriate treatment regime, the main histological distinctions to be drawn are between: 1 tumours of neuroectodermal origin (Ewing's sarcoma and primitive neuroectodermal tumour); 2 other sarcomas; and 3 the fibromatoses and other benign and potentially locally aggressive lesions requiring local excision. Immunohistochemical staining may be of considerable help in achieving the correct diagnosis, but it is vital that a panel of antibodies be applied and the results critically assessed. Cytogenetic analysis is also of growing importance, characteristic karyotypic abnormalities having been demonstrated in Ewing's sarcoma/primitive neuroectodermal tumour, alveolar rhabdomyosarcoma and synovial sarcoma.
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PMID:Soft tissue tumours in childhood. 822 38

Recent cytogenetic studies have revealed that several types of benign and malignant human soft tissue tumors are characterized by highly specific chromosome abnormalities. In this article, we review the primary and secondary chromosome aberrations detected in lipoma, uterine leiomyoma, Ewing sarcoma, rhabdomyosarcoma, myxoid liposarcoma, synovial sarcoma, and clear cell sarcoma of tendons and aponeuroses. The primary aberrations are unique for the particular tumor type and therefore are of diagnostic value. Most recent molecular studies indicate that several sarcoma-specific translocations result in the gene fusion and creation of tumor-specific proteins that are novel DNA transcription factors.
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PMID:[Specific chromosome aberrations in human soft-tissue tumors and their diagnostic significance]. 865 44


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