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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Embryonal rhabdomyosarcoma is the most frequent of tissue sarcomas in children. Its location in the subcutaneous tissue makes it a dermatological diagnosis. In childhood, the exact histological type of the tumour, sometimes difficult to determine, is absolutely necessary since prognosis and treatment differ according to the histogenetic form. Rhabdomyosarcoma is rare in adults. One must rule out malignant pleomorphic histiocytoma which has a more favourable prognosis. Among the antisera recently made available, those directed against desmin, foetal skeletal myosin and/or specific skeletal muscle myofilament seem to be most useful when associated with the anti-myoglobin antibody.
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PMID:[Subcutaneous rhabdomyosarcoma in children. Clinical, immunologic and ultrastructural aspects]. 322 87

The analysis of 7358 autopsies performed in 1966-1986 showed that cardiac tumors were detected in 94 cases (1.27%) and accounted for 4.9% of cancer and 5.48% of cardiac disease cases. Detailed characteristics are provided for 9 primary tumors, of these 3 were assessed clinically and morphologically, (left ventricular elastofibroma, left ventricular pleomorphic rhabdomyosarcoma, left atrial embryonal rhabdomyosarcoma). Primary metastases of pulmonary localization were recorded in 47.9% of cases. Metastatic involvement of the heart presented in 85 cases (1.15% of autopsies, 4.43% of cancer cases, 5% of cardiac affections, 90.42% of cardiac tumors). Cardiac compartments were evaluated for the frequency of tumor emergence.
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PMID:[Clinico-morphologic analysis of primary and metastatic tumors of the heart]. 325 Mar 83

A case of rhabdomyosarcoma of the right kidney is presented. A 78-year-old man was admitted with the complaint of abdominal pain and abdominal fullness on March 15, 1985. Radiological examination showed a giant tumor of the right kidney. Radical nephrectomy and right hemicolectomy were performed. Histological findings were embryonal rhabdomyosarcoma of the kidney. Residual tumor in the duodenal area recurred and he died of peri-duodenal abscess 2 months after admission. This case is the 17th case of rhabdomyosarcoma of the kidney in Japan. The literature is reviewed and discussed.
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PMID:[Rhabdomyosarcoma of the kidney: report of a case]. 332 38

This article reports on the diagnostic features, radiographic findings, staging, operative management, treatments, and treatment results for 95 children (mean age, 10 years) with paratesticular sarcoma; they were treated on the Intergroup Rhabdomyosarcoma Study (IRS) I and II protocols. Embryonal rhabdomyosarcoma (RMS) was the most common histologic subtype (97% of cases). Patients were randomly assigned to receive various therapeutic regimens according to IRS protocols. Among 81 patients (85%) with localized disease, most (57) were in Clinical Group I (localized, completely excised tumors), and 20 were in Group II (gross excision with tumor-involved, regional retroperitoneal lymph nodes or microscopic residual). Only four were in Group III (gross residual disease in the primary site or retroperitoneal lymph nodes). Three of them achieved a complete response (CR) after induction therapy and two then relapsed. The relapse-free survival (RFS) estimates at 3 years from diagnosis were 93% in Group I and 90% in Group II. Distant metastases were present at diagnosis in only 14 patients. Twelve of them achieved CR, but four subsequently relapsed; their 3-year RFS estimate was 67%. Overall, survival rates among the 95 children were excellent (89% at 3 years) compared to the entire IRS series (63%). Treatment with radical orchidectomy and chemotherapy was sufficient for Group I patients. Orchidectomy, chemotherapy, and radiation therapy were highly effective in Group II patients and in a large proportion of those with more advanced tumors. Recommendations for the diagnostic examinations and management of future patients with paratesticular sarcoma are given.
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PMID:Paratesticular sarcoma in childhood and adolescence. A report from the Intergroup Rhabdomyosarcoma Studies I and II, 1973-1983. 344 Feb 40

A case of embryonal rhabdomyosarcoma of the femur observed in an 11-year-old boy is reported, which simulated an osteosarcoma clinically and radiographically. To our knowledge, this is a second documented case of rhabdomyosarcoma of the long bone.
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PMID:Primary rhabdomyosarcoma of long bone. A case report. 345 39

A review of the literature and analysis of 40 cases of genital malignancies girls are presented. 31 patients had ovarian tumor, 2--cervical, 4--vaginal and 3--tumor of the vulva. Most embryonal rhabdomyosarcoma of the vagina or the vulva occurred in patients under 4 whereas ovarian neoplasms (mostly germ and sex cord tumors) were observed at prepubertal and pubertal age. Early dissemination and unfavorable prognosis were typical of rhabdomyosarcoma of the vagina and vulva. Stage I dysgerminoma and granulosa cell tumor usually make the case for sparing surgery whereas stage II and III malignancies--for a radical one, irrespective of histological pattern. Prognosis is better when surgery is supplemented with individually-tailored chemotherapy.
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PMID:[Malignant tumors of the genitalia in girls]. 351 9

544 malignant soft tissue tumors have been collected at the Pediatric Tumor Registry in Kiel including 300 cases of rhabdomyosarcoma (55%). In 237 of the 300 cases the diagnosis is certain. Liposarcoma and malignant fibrous histiocytoma which are typical tumors of adult age are rare in our material. Among rhabdomyosarcomas embryonal rhabdomyosarcoma (eRMS) clearly predominates accounting for almost 72% of all rhabdomyosarcomas. Differentiation of tumor cells in eRMS may vary considerably. Therefore, three groups of eRMS were distinguished and analyzed for clinico-pathologic features: 1. Primitive eRMS with less than 10 rhabdomyoblasts. 2. Intermediate eRMS with 10-50% rhabdomyoblasts. 3. Well differentiated eRMS with greater than 50% rhabdomyoblasts. By immunohistochemistry, vimentin positive cells were found in all three groups. The number of desmin positive cells depended upon the grade of differentiation. Thus, there were more desmin positive cells in well differentiated eRMS. Primitive and well differentiated eRMS were predominantly located in the head and neck area, intermediate eRMS in the abdomen. Primitive eRMS were noted in higher stages than tumors of the other two groups. Response to chemotherapy as evaluated in the 7th week of treatment was better in well differentiated eRMS. Moreover, patients of this group achieved more often complete remission. It is concluded from the present study that differentiation in eRMS may have an influence on the clinical presentation and clinical course of the disease. Therefore, this question should be investigated in more detail in a larger prospective study.
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PMID:[Rhabdomyosarcoma: morphology and cellular differentiation]. 352 25

Two cases of paratesticular rhabdomyosarcoma are reported. A 15-year-old boy was admitted in July, 1983, with a 3-week history of gradually increasing painless scrotal swelling on the right side. With the suspicion of testicular cancer, right radical orchiectomy was performed and pathological examination revealed an embryonal rhabdomyosarcoma of the paratesticular region. Chemotherapy and retroperitoneal lymphadenectomy were performed, and the latter revealed lymph node metastasis at the bifurcation of the aorta. Radiotherapy was not done because of adherent ileus. About 8 months after the orchiectomy, he died of recurrence at pelvic cavity and brain metastasis. A 6-year-old boy presented in March, 1985, with a complaint of right scrotal swelling that was painless and gradually increasing for about a month. Transinguinal exploration revealed a paratesticular neoplastic lesion and right radical orchiectomy was performed. Pathological diagnosis was paratesticular embryonal rhabdomyosarcoma. No evidence of metastasis was found by chest X-ray, excretory urogram, abdominal CT-scan or lymphangiography. As post-operative treatment, only chemotherapy was performed, and 9 months after the orchiectomy, the patient was asymptomatic. Ninety four cases of paratesticular rhabdomyosarcoma found in the Japanese literature are reviewed and mainly the policy of treatment is discussed.
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PMID:[Two cases of paratesticular rhabdomyosarcoma]. 361 37

The clinicopathological features of 10 children with paratesticular rhabdomyosarcoma treated between 1965 and 1984 are reviewed. Of the patients 9 had embryonal rhabdomyosarcoma and 1 was pleomorphic. Median age was 4 years (range 2 to 11 years). Staging was based on clinical findings, chest x-ray, lymphoangiography, computerized tomography and histological studies. The disease was stage I in 5 patients, stage II in 2 and stage IV in 3. Treatment included radical orchiectomy in all patients, chemotherapy in 8 and lumboaortic radiotherapy in 5. No retroperitoneal node lymphadenectomy was performed. Of the 10 children 7 are free of disease after 2 to 19 years (median 7 years) of followup, including all of those with stages I and II disease.
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PMID:Paratesticular rhabdomyosarcoma in children. 372 82

A new human embryonal rhabdomyosarcoma cell line, designated JR-1, is described that closely resembles the tumour from which it was derived. Comparative studies, by light and electron microscopy reveal morphological features such as myofibre formation, that are concordant with embryonal rhabdomyosarcoma. Immunohistological investigations using a panel of monoclonal antibodies indicate that the cell surface antigen profile of the JR-1 cell line is similar to other embryonal rhabdomyosarcomas. In addition the cell line expresses the cytoplasmic intermediate filament protein desmin, only found in cells of rhabdoid origin. Karyotyping JR-1 shows the cells to contain variable numbers of chromosomes (range 44-100). DNA flow cytometry indicates that cells have an DNA content which is approximately twice normal. The JR-1 cell line has a doubling time of 29 h in culture and, in common with several other human cell lines, produces xenografts in nude mice within 6 weeks of inoculation. With detailed studies on the original tumour and the JR-1 cell line, the latter should prove an excellent model system for investigating the biology of rhabdomyosarcoma.
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PMID:Comparative studies between a new human rhabdomyosarcoma cell line, JR-1 and its tumour of origin. 373 Feb 58


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