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)

Three children with rhabdomyosarcoma (sarcoma botryoides) of the vagina or uterus were managed by modified radical resection combined with pre- and/or postoperative chemotherapy and high-dose irradiation. This plan of therapy contrasts sharply with the conventional approach: i.e., pelvic exenteration consisting of cystectomy, hystovaginectomy, and oophorectomy, with urinary diversion by ureteroileostomy or ureterosigmoidostomy. Two patients had complete regressions of tumor following preoperative chemotherapy and irradiation. The third patient received no preoperative therapy, but was given postoperative radium implantation, irradiation, and chemotherapy. The surgical approach consisted of hystovaginectomy and oophorectomy without urinary diversion. These patients are free of tumor for 32, 44, and 54 months, respectively. There were no serious toxic reactions to the drugs, nor any significant postoperative urinary tract problems. The results reported here suggest that hystovaginectomy and oophorectomy coordinated with chemotherapy and irradiation is an acceptable alternative to pelvic exenteration in patients with sarcoma botryoides of the vagina or uterus.
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PMID:Combined therapy to prevent complete pelvic exenteration for rhabdomyosarcoma of the vagina or uterus. 124 48

Pure rhabdomyosarcomas originate in the female genital tract. They are uncommon and most often occur in infancy or childhood as sarcoma botryoides (embryonal rhabdomyosarcoma) which involve the vagina and cervix. Such tumors rarely occur in adults. A pure rhabdomyosarcoma of the uterus that arose in a postpartum patient is described. The pertinent literature is discussed.
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PMID:Pure rhabdomyosarcoma of the corpus uteri in a postpartum patient: report of a case and review of the literature. 132 92

Embryonal rhabdomyosarcoma of the vagina in adults per se is uncommon and pregnancy complicated by such tumors is extremely rare. We wish to report one case of vaginal embryonal rhabdomyosarcoma, diagnosed at the age of 23 years who was carrying at the time of initial presentation. The problem of management of rhabdomyosarcoma in adults, particularly when they are associated with pregnancy is discussed. We advocate the use of multiagent chemotherapy and pelvic radiotherapy in its overall management.
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PMID:Adult embryonal rhabdomyosarcoma of the vagina complicating pregnancy: a case report and review of the literature. 209 25

Pure rhabdomyosarcomas of the female genital tract most commonly occur in infancy or childhood as sarcoma botryoides (embryonal rhabdomyosarcoma) and involve the vagina and cervix. Such tumors rarely occur in adults. A pure rhabdomyosarcoma of the uterus that arose in a postmenopausal patient is described. The pertinent literature is discussed.
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PMID:Rhabdomyosarcoma of the uterus in a postmenopausal patient. 219 Aug 75

A major objective of the second Intergroup Rhabdomyosarcoma Study (IRS-II) (1978 to 1984) was to preserve the bladder without compromising the survival of children with localized genitourinary sarcomas arising in or near the bladder. After incisional biopsy, 109 patients with localized, gross residual sarcoma of the prostate (43 patients), bladder (43 patients), vagina (20 patients), or cervix/uterine corpus (3 patients) were treated with vincristine, dactinomycin, and cyclophosphamide (VAC). After two to four drug courses, radiation therapy and/or surgery were used to treat patients with residual or recurrent tumor. The 3-year survival rate of patients treated on this primary chemotherapy regimen (70%) was similar to that of the primary surgery regimens of IRS-I (78%; P = 0.46), but the 3-year disease-free survival rate was significantly inferior (52% versus 70%; P = 0.02). Since the IRS-II encouraged bladder preservation at the onset of therapy, the percentage of patients with bladder and prostate tumors who retained the bladder was initially substantially higher in IRS-II (97%) than in IRS-I (58%). However, the percentages of 95 patients with bladder-prostate (BP) tumors in IRS-II who retained the bladder and were alive at 2 and 3 years after starting treatment were only 33% and 22%, respectively, compared with 26% and 23%, respectively, in the 66 patients with BP tumors in IRS-I. Thus, sequential treatment with primary chemotherapy, followed by radiation therapy and/or surgery as given in IRS-II, failed to improve the eventual bladder salvage rate.
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PMID:Primary chemotherapy with or without radiation therapy and/or surgery for children with localized sarcoma of the bladder, prostate, vagina, uterus, and cervix. A comparison of the results in Intergroup Rhabdomyosarcoma Studies I and II. 222 61

Between 1970 and 1978, 17 girls with rhabdomyosarcoma (RMS) of the vulva or vagina were treated at the Institut Gustave-Roussy (IGR) by conservative treatment including surgery, brachytherapy, and chemotherapy. Twelve pubescent or postpubescent girls were studied for long-term sequelae. Eleven of 12 patients have had a normal puberty, two have a total of three healthy children, 11 have normal menses, and 10 normal menarche (one after hormonal replacement). Only one patient underwent hysterectomy following low-dose brachytherapy. Five girls have no vaginal complications, and three are sexually active. Three have had minimal vaginal sequelae, which required surgical correction to permit sexual intercourse. Four girls sustained serious sequelae (colorectal, vaginal, urethral, and ureteral stenosis). These sequelae, secondary to irradiation, are potentially avoidable in the future given the current advances in brachytherapy and improvements in dosimetry. This conservative treatment is useful and appropriate for girls with RMS of vulvar or vaginal origin when complete remission cannot be obtained with chemotherapy and partial colpectomy.
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PMID:Long-term sequelae of conservative treatment by surgery, brachytherapy, and chemotherapy for vulval and vaginal rhabdomyosarcoma in children. 223 Aug 71

We present the result obtained by GICOP in the treatment of 12 genitourinary rhabdomyosarcoma, located in vagina-uterus (5), bladder-prostate (4) and paratesticular (3). The age of the patients ranging from 1 to 15 years. Embryonal rhabdomyosarcoma was the most common histologic type (58 for 100) being the major number of cases in stadium II. The treatment was based in the combination of chemotherapy protocolised with vincristine, d-actinomycin, cyclophosphamide (VAC), vincristine, adriamycin (VAC-VAD) and/or iphosphamide, vincristine, d-actinomycin (IVA), use of radiotherapy, marrow auto-transplantation and radical surgery avoidance. Complete remission was achieved in 11 out of 12 cases (92 for 100), relapsing in 5 cases (38 for 100) of which 4 (80 for 100) achieved a second remission. An 82 for 100 disease free survival was achieved in a 5 year period.
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PMID:[Genitourinary rhabdomyosarcoma in childhood]. 248 79

Wilms' tumor, embryonal rhabdomyosarcoma, and yolk sac tumor are the most frequently diagnosed malignant genitourinary tumors in children. The randomized therapeutic trials developed by the National Wilms' Tumor Study (NWTS) committee have produced significant improvements in the survival of Wilms' tumor patients. Those with Stage IV, favorable histology Wilms' tumor now have a 2-year survival rate of 87% to 93%. Patients with recurrent Wilms' tumor are being prospectively treated with new drugs and drug combinations with the goal of identifying active agents for inclusion in future therapeutic trials. Girls with localized embryonal rhabdomyosarcoma of the vagina have a 100% survival rate after treatment on the Intergroup Rhabdomyosarcoma Study (IRS) (IRS-1 or IRS-2). Primary treatment of patients with tumors of the prostate, bladder neck, or trigone with chemotherapy has demonstrated the need to establish local tumor control using surgery or radiation soon after diagnosis. The IRS-III currently is evaluating new drug combinations in this group of patients with locally advanced tumors. Patients with advanced yolk sac tumor have been treated using platinum-containing combination chemotherapy regimens with very encouraging results. Future research will evaluate the timing of second-look surgical procedures and the efficacy of etoposide-containing combination chemotherapy regimens.
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PMID:The treatment of advanced or recurrent malignant genitourinary tumors in children. 303 35

Two hundred and fifty three children with newly diagnosed nonmetastatic rhabdomyosarcoma, from 30 European centers, were registered by the International Society of Pediatric Oncology (SIOP) between 1975 and 1982, and received a VAC-VAD chemotherapy. Multivariate statistical methods were used to study prognosis. The most important predictors for survival time were as follows: primary site (P less than 0.001), clinical stage (P = 0.02), and sex (P = 0.03). Three primary sites had a favourable prognosis (vagina, paratestis, orbit) as opposed to the most unfavorable ones (parameningeal head and neck sites). Clinical state I had better survival curves than stages II and III. Males had a better prognosis than females, regardless of stage and primary site. The analysis of the evolutive modes of the tumour showed that the lack of local control (no complete remission, local recurrence) was the first reason of failure.
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PMID:[Prognostic factors in rhabdomyosarcomas in childhood. Study carried out with 253 children registered by the International Society of Pediatric Oncology]. 335 65

Fifteen children with rhabdomyosarcoma of the bladder and prostate or vagina treated by combined chemotherapy radiotherapy and surgery, are reported. Eleven were submitted to preoperative chemotherapy. Ten children are alive with a follow up of 7 month to 16.2 years after treatment. All five deceased patients were boys. Nine children were submitted to a conservative approach of the lesions; 8 of there ar alive, 3 of which with sequelae. Four boys were treated with total cystectomy and one girl had a colpohysterectomy: 2 of these children also have sequelae. The point of this report is to underline the possibility of a conservative surgical approach when possible, especially in cases where tumor reduction is obtained by preoperative chemotherapy and completed by local irradiation when necessary. The late sequelae of these treatment modalities are also analysed.
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PMID:[Conservative therapy in genitourinary rhabdomyosarcomas in children]. 344 69


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