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)

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

Of a total of 1561 patients registered in the Intergroup Rhabdomyosarcoma Study (IRS) as of May 1983, 78 patients (5%) were younger than 1 year of age. These infants did not differ from the older children (1-20 years of age) in male/female ratio, clinical grouping, or survival rates. Infants younger than 1 year of age had a significantly greater frequency of undifferentiated sarcoma, 18% versus 7% in older children (P less than 0.005). Infants also had a significantly greater proportion of cancers with botryoid pathology, 10% versus 4% in older children (P less than 0.005). When reviewed by the newly proposed IRS cytopathologic classification, there was no difference in pathologic types between the two age groups. Infants younger than 1 year of age had a higher rate of bladder-prostate-vagina primary tumor sites than older children, 24% versus 10% (P less than 0.05). In addition, infants tended to receive less of the prescribed doses of chemotherapy and radiation therapy than older children, and to develop more toxicity to treatment than older children. Despite these differences, the overall survival curve for the two age groups appears to be similar. In contrast to Wilms' tumor and neuroblastoma, in which age (less than 1 year) is a favorable prognostic factor, age does not appear to be an important prognostic factor in rhabdomyosarcoma.
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PMID:Infants younger than 1 year of age with rhabdomyosarcoma. 377 10

During a 12-year period (1972-1984), 43 patients with sarcomas of the female genital tract were admitted to the Intergroup Rhabdomyosarcoma Study (IRS), including 31 with primary tumors of the vagina; and 12 with tumors of the uterus, including the cervix. Thirty-four of these can be evaluated on the basis of periods of observation from 18 months to 12 years. Primary tumors of the uterus were a distinct group, distinguished from those arising in the vagina by patient age-range and probably by prognosis, as well as site. Patients with vaginal tumors, with a mean age of 1.8 years, responded to a multimodality approach employing combinations of chemotherapy (vincristine sulfate and actinomycin D, or the aforementioned two drugs with cyclophosphamide +/- doxorubicin hydrochloride), irradiation, and/or surgery, with only one tumor-related death, among 24 evaluable patients. In contrast, among the patients with primary uterine tumors, in which the mean age was greater than 14 years, four of ten evaluable patients died secondary to tumor relapse or progression.
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PMID:Sarcomas of the vagina and uterus: the Intergroup Rhabdomyosarcoma Study. 391 Jul 85

The outlook for children with rhabdomyosarcoma has change significantly in these last years. With an adeguate combined modality therapy more than 50% of these children may be cured. The results of the IRS-I indicate that the 3 year relapse-free survival rates are 85% for patients in group I 70% for those in group II, 45% for those in group III and 15% for those in group IV. In addition to the clinical group other significant prognostic factors are histologic cell type (alveolar, unfavorable) and primary site (disease in extremities and in retroperitoneal area, unfavorable). The chemiotherapy must be used in all patients for 12-24 months. The effective drugs are VCR, ACT-D, CTX, ADR combined in different schedules. It has been demonstrated that the effective doses of radiotherapy range from 4000 to 5000 rad and that radiotherapy may be omitted in patients in group I. Now a less aggressive surgical procedures may be employed, and patients with primary tumor in the orbit or in the pelvic organs may be cured saving the eye, or the bladder, the vagina and the uterus.
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PMID:[Rhabdomyosarcoma: therapeutic results and prospectives]. 654 8

We report on 27 children with rhabdomyosarcoma of the bladder, prostate, uterus, cervix and vagina treated with combined therapy of surgery, radiotherapy and multiple drug chemotherapy. Of the 27 patients 18 are alive for 13 to 147 months (median 86 months) and 9 have retained a functional bladder. Extirpative surgery followed by adjuvant chemotherapy and occasionally radiation therapy was most effective for local tumor control and 7 of 10 patients survived, including 5 with an intact functional bladder. Primary use of chemotherapy in 13 patients with extensive disease resulted in complete tumor control in only 3 but 7 survived, including 4 with an intact bladder. Radiation therapy used concomitantly with chemotherapy failed to control the local tumor but with subsequent extirpative surgery all 4 patients survived.
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PMID:Treatment of genitourinary rhabdomyosarcoma in children. 673 85

Childhood gynecologic problems are different from adult problems in physiology, disease, psychology, evaluation and management. The most common is vulvovaginitis. The child is susceptible to vulvovaginitis because of a relatively exposed vulva, a thin vaginal wall and poor hygiene. Additional problems are heat and moisture, clothing, coverings and possible sexual abuse. Most vulvovaginitis is primary, nonspecific vulvitis with secondary vaginitis. It is set off by poor hygiene and responds to keeping the vulva clean, cool and dry. Persistent or recurrent vulvovaginitis may be due to a variety of causes, including vulvar skin disease, a foreign body in the vagina, primary vaginitis, ectopic ureter and rhabdomyosarcoma. Vaginoscopy is indicated for recurrent vulvovaginitis or bleeding and for suspicion of a foreign body, neoplasm or congenital anomaly. The extent of evaluation depends on the anxiety of the child versus the extent of the problem, and therefore individualization is required.
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PMID:Pediatric vulvovaginitis. 674 69

During the initial 6 yr (1972-1978) of the Intergroup Rhabdomyosarcoma Study (IRS), 27 patients with rhabdomyosarcoma of the female urogenital tract were treated, and the results of therapy were evaluated on the basis of 3-7 yr of observation. By primary site these tumors were: bladder, 14; vagina, 9; vulva, 2; and cervix uterus, 2. Twenty-three presented with localized tumors; the four remaining patients (1 bladder, 1 vagina, and 2 cervix) had distant metastases at diagnosis. Two patients with bladder tumors underwent pelvic exenteration, local irradiation, and chemotherapy (2 yr), and were without relapse. Ten patients were treated by partial cystectomy and postoperative chemotherapy (with or without local irradiation) and fatal recurrence occurred in three. The remaining seven patients are disease-free for 36-74 mo (mean, 51.8 mo). Six of the eight patients with nondisseminated vaginal tumors were treated by initial pelvic exenteration (2), hysterectomy-vaginectomy (2), or hemivaginectomy (2). All received postoperative chemotherapy and three received local irradiation. Delayed hysterectomy-vaginectomy after primary chemotherapy was performed in the two additional patients with vaginal tumors. Relapse has occurred in one of these eight patients with localized vaginal disease. The remaining seven have been observed for 3-5.5 yr (mean, less than 4 yr). Two patients with vulval lesions treated by a combined therapy regimen are without recurrence. Our conclusion is that chemotherapy has significantly increased survival in females with urogenital tract rhabdomyosarcomas.
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PMID:Rhabdomyosarcoma of the female urogenital tract. 733 62

Fifty-eight children with genitourinary rhabdomyosarcoma are reported. Lesions involved the bladder (22), prostate (14), vagina/uterus (6), and paratesticular tissues (16). Fifteen of 58 had positive sampling of regional lymph nodes. Eleven of 15 received radiation to no more than 4500 rad in most cases, and 9 of 11 are diseases free. Two of 15 had no radiation and are disease free also. Twenty-three of 58 had negative nodes. Six of 23 had radiation to these nodal areas and 4 of 6 are disease free. Fifteen of 17 patients had no radiation and are disease free also. Sixteen of 20 patients with no node sampling are disease free; 9 of 16 had radiation but 7 of 16 did not. All patients in the Study had intensive systemic maintenance chemotherapy. One 1 patient failed in regional nodes despite 4500 rad to these node echelons. We suggest chemotherapy and radiotherapy, not to exceed 3500 rad in four weeks, to known residual disease including regions from which positive nodes have been obtained.
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PMID:Radiation to regional nodes for rhabdomyosarcoma of the genitourinary tract in children: is it necessary? A report from the Intergroup Rhabdomyosarcoma Study No. 1 (IRS-1). 738 51

From 1967 to 1992, 39 patients with urogenital rhabdomyosarcoma were admitted to our hospital. Of them 30 were male and 8 female and their age ranged from 4 months to 14 years (32 were less than 5 years). Primary tumor was located in bladder or/and prostate in 26, paratesticule 9, vagina 3, and penis 1. Among the 31 patients receiving treatment, 23 were followed up. There were 15 (65%) survivors, including 13 who were free from tumor for over 4 years. The eldest two patients were 20 and 21 years of age. Two patients with vaginal tumor and 4 with bladder tumor preserved their organs with normal function. Biopsy were done only in 8 patients. In recent years, conservative surgery combined with chemotherapy for urogenital rhabdomyosarcoma has been justified.
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PMID:[Urogenital rhabdomyosarcoma in children: analysis of 39 cases]. 784 48

Eleven survivors of pelvic rhabdomyosarcoma underwent bladder function studies and upper urinary tract evaluation at a mean of 6.6 years after completion of therapy, which included a conservative, bladder-sparing surgical policy. Primary tumour sites were: bladder base/prostate, 6; bladder dome, 1; vagina, 2; and pelvic side wall, 2. Seven children (five bladder base/prostate, one vagina and one pelvic side wall tumours) had received irradiation to the pelvis with external beam alone, brachytherapy or both. All seven of these patients had markedly reduced functional bladder capacity (11-48% of mean expected value for age) and abnormal voiding patterns, though bladder compliance was not reduced and bladder emptying was almost complete in five cases. Four of these children also had upper tract dilatation and two required reconstructive bladder surgery because of severe bilateral hydronephrosis. By contrast, each of four children treated without radiotherapy had a normal functional bladder capacity and a normal voiding pattern. all survivors of pelvic rhabdomyosarcoma, especially those who have received radiotherapy, should be carefully monitored for dysfunction of both lower and upper urinary tracts. The frequency-volume voiding chart is a sensitive and easily accomplished method of assessing bladder function in these patients.
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PMID:Bladder and kidney function after cure of pelvic rhabdomyosarcoma in childhood. 794 75


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