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

Rhabdomyosarcomas (RMS) occurring in pelvic sites are almost entirely of the embryonal histologic subtype and are relatively responsive to chemotherapeutic agents. When treated by radical surgery, pelvic irradiation, and intensive chemotherapy for 2 years, survival rates of higher than 85% have been achieved. However, the effects of such therapeutic approaches have resulted in major changes in the quality of life of survivors, because of the loss of pelvic organs, primarily the bladder. Current efforts are directed towards achieving long range survival with procedures that are less extensive than pelvic exenteration or total cystectomy and by regimens that eliminate or reduce the intensity of radiotherapy. Until the past 5 years, these efforts have been relatively unsuccessful in both North America and Europe. However, the most recent studies of the IRS and European cooperative groups suggest that a majority of these patients can survive with intact pelvic organs when treated with currently available chemotherapeutic agents in conjunction with radiotherapy and limited surgery.
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PMID:Bladder/prostate rhabdomyosarcoma: results of the multi-institutional trials of the Intergroup Rhabdomyosarcoma Study. 828 71

The pleomorphic subtype of rhabdomyosarcoma (RMS) is now rarely diagnosed in both children and adults. Most cases previously called pleomorphic RMS are probably diagnosed as something else, most often embryonal RMS in children and malignant fibrous histiocytoma in adults. To analyze the concept of pleomorphic RMS in children, we reviewed the tumors of patients entered on the Inter-group Rhabdomyosarcoma Study (IRS I, II, and III). The presence of cells with lobated, hyperchromatic nuclei at least three times larger than the common tumor cell population (anaplastic cells) was selected as the main criterion. Of about 3,000 cases, 110 showed these types of cells, had sufficient histologic material, and had available follow-up data. These tumors were divided into two subgroups: Subgroup I tumors contained only scattered anaplastic cells, and tumors with foci or large sheets of anaplastic cells were classified as subgroup II. Besides the anaplastic-pleomorphic areas, most of these tumors had distinctive features of embryonal RMS (105 cases) and rarely had characteristics of alveolar RMS (five cases). The age distribution of these patients did not differ significantly from those whose tumors did not show the anaplastic features, the average being 6 years and the median 4 years. Lower extremity, retroperitoneum, and the head and neck region were the most common primary tumor sites. The 5-year survival rate was 60% for subgroup I tumors and 45% for subgroup II tumors compared with the survival rate of 68% for 482 IRS II embryonal RMS cases with no anaplastic-pleomorphic features. The lower survival rate for patients in subgroup II was statistically significant (p = 0.004) and similar to the unfavorable survival of patients with alveolar RMS and undifferentiated sarcoma. Because anaplastic cells are seen in many soft tissue sarcomas and in both embryonal and alveolar RMS in children, this feature is not sufficiently unusual to separate a pleomorphic subtype of RMS. The presence of anaplastic cells in aggregates or diffuse sheets throughout the tumor, however, portends a poor survival for these patients.
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PMID:Childhood rhabdomyosarcoma with anaplastic (pleomorphic) features. A report of the Intergroup Rhabdomyosarcoma Study. 847 Jul 59

Exenteration is no longer required for most patients who have rhabdomyosarcoma (RMS) of the prostate. This site comprised only about 5% of newly diagnosed cases in the IRS-III (1984-1991). The mean age at the time of diagnosis was 5.3 yrs (range, 0 to 19 years). Most tumors were relatively large, had embryonal histology, and were clinically localized but unresectable without major loss of organ function. The 44 patients with group III tumors (gross residual disease) were treated according to the IRS-III protocol. Forty-three of them underwent biopsy only, and one patient had subtotal resection as the initial procedure. The average number of surgical procedures per patient was two (range, one to five). Six of the 44 patients had no additional surgery. The second-look procedures performed in the other 38 patients included exenteration (14), prostatectomy (7), cystoscopic/perineal needle biopsy (8), laparotomy with biopsy (6), and subtotal excision with bladder salvage (3). Additional surgery was required for four patients, for evaluation of a residual mass, postoperative fistula, ureteral stricture, or small bowel obstruction. Six patients with relapse or residual disease underwent additional chemotherapy and late exenteration (3), prostatectomy (1), or biopsy (2). Four of the six have been cured, one is in treatment for a second malignancy, and the other has residual disease after exenteration. Thirty-six of the 44 patients with group III tumors have been cured (minimum follow-up period, 6 years; range, 6 to 11 years), compared with 23 of the 47 in IRS-II (1978-1984) (P = .001). Two of the six deaths in this group were caused by infection. The bladder salvage rate for those cured of their disease also was better (64% v 57% for IRS-II). The two patients with group IIA tumors were cured by gross primary excision, local radiotherapy, and vincristine and actinomycin therapy. By contrast, all patients with metastatic disease (group IV) died of the tumor. Conservative, delayed surgery, performed after intensive chemotherapy with or without radiotherapy, yields a better cure rate while maintaining a high rate of bladder salvage in children with group III prostatic RMS.
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PMID:The argument for conservative, delayed surgery in the management of prostatic rhabdomyosarcoma. 886 39

The results of treating 155 children with rhabdomyosarcoma using protocols that (RMS) changing over the years between 1962-1990 in reported to progress in chemotherapy (CHT), introduction of megavoltage radiotherapy (RTX) and conservative surgery with attempts to preserve vital organs are presented. In the first period between 1962-1980 when mainly surgery was applied with orthovoltage RTX and low intensity CHT, only 20 of 74 children (27%) survived. In the second period 1981-1985 systemic CHT containing new cytostatic, megavoltage RTX and limited surgery applied in advanced cases after induction of CHT were introduced. Nineteen of 46 children (41.3%) survived. In the last period a 1986-1990 more intensive CHT with an own modified protocol VACA/VAIA and intensification phases containing cisplatinum, etoposide and/or carboplatinum were introduced. Twenty seven of 35 patients (60%) survived. Comparative analysis of the last two periods pointed to significant progress in treatment in III clinical group (IRS classification), (20.7% vs 62.9%) and parameningeal RMS (0% vs 58%). Meaningful improvement concerned also young children below 5 years of age (42.8% vs 73.9%). The main prognostic factors and treatment failures of the recent years were analyzed.
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PMID:[Progress in the treatment of 155 children with rhabdomyosarcoma obtained in one center between 1962-1990]. 892 69

A case of paratesticular rhabdomyosarcoma in a 22-year-old male is reported. Radiological examination revealed a gross metastatic mass in the paraaortic lymph node. Right high orchiectomy was followed by chemotherapy consisting of cisplatin, vincristine, cyclophosphamide and adriamycin (IRS-III regimen 35). A complete response was seen 4 months after start of therapy. However, recurrent disease developed in the same paraaortic lymph node 25 months postoperatively. The patient died of tumor progression 11 months later.
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PMID:[A case of paratesticular rhabdomyosarcoma with a paraaortic lymph node metastasis]. 912 62

Children and adolescents who develop rhabdomyosarcoma (RMS) and related sarcomas in the orbit and treated on Intergroup Rhabdomyosarcoma protocols have had an extremely high cure rate. This study evaluates the possible relationship between their tumor morphologic subtypes and this high cure rate. The histology of tumors was re-reviewed from 229 of the 264 patients with tumors of the orbit, conjunctiva, and eyelids treated on Intergroup Rhabdomyosarcoma Studies (IRS I, II, III, and IV pilot protocols, and followed through July, 1992. Immunohistochemistry was applied in selected cases. Clinical correlations were done on all 264 cases including both the re-reviewed cases and those reviewed only by the IRS Pathology committee. The 5-year survival rate of 24 children with alveolar RMS was 74% (p < .001). All five infants diagnosed to have an alveolar RMS died before the age of one. Two hundred and twenty-one patients (84%) had embryonal RMS. About three-fourths of the re-reviewed embryonal RMS tumors showed only minimal rhabdomyoblastic differentiation. Thirty-one had a spindle cell RMS, two were anaplastic variants. The 5-year survival rate for patients with embryonal RMS subtypes combined was 94%, and 97% for the 144 patients with poorly differentiated embryonal RMS. In contrast, 90 of 432 IRS II patients treated for poorly differentiated embryonal RMS located in extraocular sites had a 66% survival estimate.
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PMID:Orbital rhabdomyosarcomas and related tumors in childhood: relationship of morphology to prognosis--an Intergroup Rhabdomyosarcoma study. 914 7

Rhabdomyosarcoma (RMS) rarely occurs in adult and intra thoracic activity. This is a report on a case we experienced of adult RMS, which was subjected to tumorectomy and lobectomy. The patient was a 23 years old male, who first consulted a hospital in his neighborhood due to anterior chest pain suffered since May 1995. Since the examination showed an abnormal shadow in right anterior mediastinum, the patient was referred to our department. By biopsy under CT, the patient was diagnosed as having embryonal type of RMS and underwent surgery. The outcome was good and the patient is currently in the course of chemotherapy using IRS (Inter-group Rhabdomyosarcoma study)-III.
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PMID:[Report on a case of adult rhabdomyosarcoma subjected to tumorectomy and lobectomy]. 915 36

Between November 1978 and July 1991, ten children between one and eight years of age with Group III pelvic rhabdomyosarcoma (IRS classification) and considered inoperable at diagnosis were treated primarily with intensive poly-chemotherapy. Complementary radiotherapy and conservative surgery were added as needed. Eight of the ten survive free of disease with functioning bladders for periods ranging from 5.7-18.4 years.
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PMID:Organ-preserving management of rhabdomyosarcoma of the prostate and bladder in children. 932 47

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood, and 75% of such cases in the United States are reviewed at the Pathology Center for the Intergroup Rhabdomyosarcoma Study Group (IRSG). The first four generations of IRSG therapeutic trials (IRS I-IV) and supportive pathologic studies have generated a new International Classification of Rhabdomyosarcoma (ICR) that offers new morphologic concepts to the practicing pathologist. The objective of this report is to clearly define emerging histopathologic categories of RMS as defined by the ICR, and to emphasize correlative immunohistochemical or molecular studies. Emerging ICR variants of RMS place the patient in widely divergent prognostic categories (superior, botryoid or spindle cell variants; poor, solid alveolar or diffusely anaplastic variants). The cardinal histopathologic features of the ICR combined with results of studies of fusion genes seen with t(1;13) and t(2;13) will help delineate therapeutic subgroups of RMS for the fifth generation (IRS V) of IRSG studies. Consequently, it is imperative for the practicing pathologist to be familiar with the practical workup and diagnosis of RMS in childhood.
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PMID:Intergroup Rhabdomyosarcoma Study: update for pathologists. 972 44

A case of prostatic rhabdomyosarcoma in an 8-year-old boy is presented. He was referred to Kurobe City Hospital with chief complaints of urinary retention and fever. Radiologic examinations revealed a huge prostatic tumor. Prostatic needle biopsy was performed and the pathological diagnosis was embryonal rhabdomyosarcoma of the prostate. He was referred to our hospital and treated with chemotherapy consisting of cisplatin, vincristine, cyclophosphamide, adriamycin, actinomycin-D and radiotherapy based on the regimen of IRS III. Total prostatectomy was performed 6 months after the start of therapy. Viable tumor cells were found in the prostate and the left obturator lymph nodes. After the operation, we continued chemotherapy. No recurrence was observed 8 months after the operation. However, local recurrence occurred in the pelvis 10 months after the operation and he died 2 months after the recurrence.
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PMID:[Rhabdomyosarcoma of the prostate in childhood: a case report]. 978 2


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