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)

Of 30 children treated for embryonal rhabdomyosarcoma the primary site of tumor was the bladder in 14 cases (9 boys and 5 girls) and the prostate in 16 cases. The mean age at diagnosis was 3 years in patients with bladder sarcoma and 6.5 years in patients with sarcoma of the prostate. Over-all survival rate has been 23 per cent--5 children with rhabdomyosarcoma of the bladder and 2 with rhadbomyosarcoma of the porstate are alive from 1 1/2 to 23 years postoperatively. Aggressive coordinated treatment with surgery, radiotherapy and cyclic combination chemotherapy is recommended.
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PMID:Embryonal rhabdomyosarcoma of the bladder and prostate in childhood. 112 15

Primary pulmonary tumors are infrequent in childhood, therefore an accurate diagnosis and treatment is often delayed. We review the English language literature and report the clinical and pathological features of eight tumors arising in the lungs of pre-adolescent children, accessioned between 1960 and 1991 in the pathology department of a children's hospital in South Africa. The ratio of pulmonary primary tumors to secondary neoplasms and to non-neoplastic lesions of the lung examined during this period was 1:5:60. Over the last 31 years we received three plasma cell granulomas, two pleuro-pulmonary blastomas, a mucoepidermoid carcinoma, an endobronchial fibrosarcoma, and a hemangioma. All patients presented with cough unresponsive to medical treatment. The incidence and spread of primary lung tumors in children was similar to that reported from other centers. Plasma cell granuloma is the most common primary tumor in the lungs of children. Aggressive behavior is most frequently encountered with pleuro-pulmonary blastoma and rhabdomyosarcoma, and because of their association with cystic lesions careful examination of lungs is required in such cases. Most other malignant neoplasms, such as muco-epidermoid carcinoma and primary fibrosarcoma, are usually of a low grade of malignancy. A decreasing incidence of bronchogenic carcinoma seems to be reported during the first two decades of life.
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PMID:Primary pulmonary tumors in childhood: a review of 31 years' experience and the literature. 133 97

Thirty-six previously untreated patients younger than 21 years of age with sarcoma arising in the perineal region were entered on the Intergroup Rhabdomyosarcoma Studies (IRS) I and II from 1972 through 1984. The tumor histologic subtype was alveolar rhabdomyosarcoma (RMS) in 20 patients (56%), embryonal RMS in 11 patients (30%), and other types of sarcoma in 5 patients (14%). Fifteen children had grossly complete surgical excision (Clinical Groups I and II), and 15 had localized gross residual tumor (Group III) after initial operative management. Six patients had distant metastases (Group IV) at diagnosis. Twelve patients without distant metastases underwent regional lymph node biopsy; tumor involvement was found in six. Subsequent treatment consisted of chemotherapy with vincristine (VCR) and dactinomycin (AMD) for all patients; patients in Groups III and IV also received cyclophosphamide (CYC) with or without Adriamycin (ADR) (doxorubicin; Adria Laboratories, Columbus, OH). Radiation therapy (RT) was administered to patients in Clinical Groups II, III, and IV. Overall, 28 (78%) patients achieved a complete clinical response. The 3-year disease-free survival rate for these patients was 42%, compared with 52% for all other patients in the combined IRS I and IRS II series (P = 0.44). The overall 3-year survival rate was 59%, compared with 64% for all other patients in IRS I and IRS II (P = 0.48). Aggressive treatment is needed in children with perineal sarcoma to improve their prognosis.
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PMID:Soft tissue sarcoma of the perineal region in childhood. A report from the Intergroup Rhabdomyosarcoma Studies I and II, 1972 through 1984. 218 89

Rhabdomyosarcoma of the urogenital tract is a malignant mesenchymal tumor seen primarily in childhood. Multimodal therapy, encompassing surgery, radiotherapy and chemotherapy, has dramatically improved the survival of patients with this disease. However, the quest for markers of tumor aggression is important to decrease the morbidity of treatment given to patients with good prognosis tumors, while at the same time intensifying treatment of tumors with poor prognosis. Using archival tumor specimens from 13 patients with genitourinary rhabdomyosarcoma, a multivariate analysis of multiple nuclear shape descriptors was done with the Hopkins Morphometry System. Three nuclear shape descriptors clearly separated patients with no evidence of disease recurrence or progression from those with recurrent disease, progressive disease or death of disease. These nuclear shape descriptors were standard error of the chain code standard deviation analysis (p = 0.010), range of the feret ellipticity distribution (p = 0.016) and standard error of the chain code range analysis (p = 0.037). With multivariate analysis these shape descriptors taken together separated patients with good and poor prognoses to a level of significance of p = 0.007. Thus, nuclear morphometric analysis may prove to be useful as an individual prognostic indicator in childhood genitourinary rhabdomyosarcoma and warrants further analysis in a much larger, blinded, controlled study.
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PMID:Nuclear morphometry as a prognostic indicator for genitourinary rhabdomyosarcoma: a preliminary investigation. 223 1

A case of spermatocytic seminoma intimately associated with rhabdomyosarcoma is reported. The patient, a 51-year-old man, presented with a two-year history of right-sided testicular enlargement. Orchiectomy was performed, and a large testicular tumor was excised. Further investigations during hospitalization revealed lung, liver, and retroperitoneal lymph node metastases. Further therapy was refused, and the patient died at home two months after orchiectomy. Autopsy was not permitted. Although the great majority of spermatocytic seminomas occur in pure form, do not metastasize, and have very good prognosis, in addition to the present case, seven cases of spermatocytic seminoma associated either with rhabdomyosarcoma or undifferentiated sarcoma have been reported. Presence of the sarcomatous element is associated with aggressive behavior, metastatic disease, and very poor prognosis. It is considered that the sarcomatous element develops from the spermatocytic seminoma by anaplastic transformation.
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PMID:Spermatocytic seminoma associated with rhabdomyosarcoma. 225 71

Aggressive and multidisciplinary therapy has resulted in the cure of at least half the cases of rhabdomyosarcoma. The author reviews the clinical features of the disease and covers diagnosis as well. The article discusses in detail the improved treatment protocols that have led to increased survival rates.
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PMID:Rhabdomyosarcoma of the head and neck: an update. 272 58

Embryonal and alveolar rhabdomyosarcoma probably have the same histogenesis, yet alveolar rhabdomyosarcoma has a considerably worse prognosis and is generally less responsive to treatment. The metastatic behavior of rhabdomyosarcoma in 22 autopsied subjects was analyzed with respect to the histologic subtypes of the tumors to determine whether the more aggressive behavior of alveolar rhabdomyosarcoma was related to greater tumor burden and/or more widely disseminated metastases. Subjects with embryonal rhabdomyosarcoma tended to survive longer and had greater numbers of metastatic sites at autopsy compared to subjects with alveolar rhabdomyosarcoma, but these differences were not statistically significant. In addition, there were no significant differences in the overall distribution of metastases between embryonal and alveolar rhabdomyosarcoma. The most frequent sites of metastases from both types of tumor were soft tissues (73%), serosal surfaces (64%), lymph nodes (64%), lungs (55%), and bone marrow (50%). The findings suggest that the metastatic behavior of rhabdomyosarcoma does not vary as a function of length of survival, mode of therapy, or histologic subtype. We propose that factors such as stage of disease at presentation and location of the primary tumor, rather than histologic subtype, are probably the most important variables governing the therapeutic responsiveness of rhabdomyosarcoma.
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PMID:Metastatic behavior of rhabdomyosarcoma. 373 70

Eight cases of alveolar rhabdomyosarcoma of the female genitalia were diagnosed from 1963 to 1983 at The University of Texas M. D. Anderson Hospital. The primary sites were vulva in two, perineum in five, and broad ligament in one patient. When possible, therapy was initiated with local tumor excision (five patients). Surgery was followed by local or regional radiation (six patients) and chemotherapy (seven patients). Of the eight patients, five died within 9 months, one died 27 months after diagnosis, and only two are 5-year survivors. The aggressive behavior of this tumor is evidenced by autopsy findings of widespread metastases. Metastatic disease to the bone was present in four patients and to the breast in three patients. Local disease was controlled in two patients who died of distant metastases. Current therapy recommendations include excisional surgery, local radiation, and combination chemotherapy. A need for more effective chemotherapeutic programs is evident.
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PMID:Alveolar rhabdomyosarcoma of the female genitalia. 401 76

Five cases of bladder sarcoma were treated at our Department between January, 1972 and December, 1981. These cases accounted for only 1.5% of the bladder tumors (335 cases) diagnosed during the same period. The patients ranged from 11 months to 67 years old. There were 2 males and 3 females. Rhabdomyosarcoma was seen in 2 cases, leiomyosarcoma in 2 cases and spindle cell sarcoma in 1 case. Treatment consisted of total cystectomy and urinary diversion followed by chemotherapy or radiotherapy in 3 cases and partial cystectomy followed by chemotherapy and/or radiotherapy in 2 cases. Two patients died during adjuvant chemotherapy. One patient, an 11-month-old boy died of septicemia facilitated by bone marrow suppression 1 month postoperatively. The other patient died of gastrointestinal bleeding 2 months postoperatively. Another case died of local recurrence at 1 year postoperatively. One woman has been free of disease for 5 1/2 years and the remaining case was lost to follow up. Aggressive multidisciplinary treatment consisting of surgery, radiotherapy and cyclic combination chemotherapy is discussed, and 203 cases of bladder sarcoma reported in the Japanese literature are reviewed.
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PMID:[Treatment of five cases of bladder sarcoma]. 651 99

We report the clinical and pathologic features of five children wih a botyroid rhabdomyosarcoma of the biliary tract. There were three boys and two girls ranging in age from 11/2 to 51/2 years at diagnosis. Obstructive jaundice was the usual presenting sign, often with fever and hepatomegaly. Of four tumors involving the common bile ducts, two extended into porta hepatis and adjacent liver parenchyma; the fifth tumor arose in the wall of the cystic duct. Each had a typical botryoid (grape-like) configuration with a cambium layer of tumor cells beneath biliary epithelium. Rhabdomyoblasts were identified in all cases and confirmed by ultrastructure in one. Our data, along with review of other cases reported in the English literature, indicate a rather guarded prognosis. Although metastases have developed in 40% of cases, death was usually due to the effects of local invasion by tumor. Aggressive adjuvant therapy currently in use for childhood rhabdomyosarcomas may lessen the high mortality associated with this tumor.
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PMID:Botryoid rhabdomyosarcoma of the biliary tract. 733 57


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