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

Abstract

A major objective of the second Intergroup Rhabdomyosarcoma Study (IRS-11) (1978(IRS-11) ( 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 %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-I1 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-I1 (97%) than in IRS-I (58%). However, the percentages of 95 patients with bladder-prostate (BP) tumors in IRS-I1 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-11, failed to improve the eventual bladder salvage rate. Cancer 662072-2081Cancer 662072- ,1990. HE Intergroup Rhabdomyosarcoma Study Commit-T tee has been investigating the therapeutic management of previously untreated patients younger than 21 years of age with rhabdomyosarcoma, undifferentiated sarcoma, or extraosseous Ewing's sarcoma since 1972. The prognosis for children with these diseases depends pri-

Bladder and kidney function after cure of pelvic rhabdomyosarcoma in childhood

“…Although the numbers in our study are small, the overall 3 year survival rate of 73% and bladder salvage rate, in our survivors, of 89% compare favourably with other reported series (Ortega, 1979;Voute et al, 1981;Grosfeld et al, 1983;Koff, 1983;Ghavimi et al, 1984;Pratt, 1984;Maurer et al, 1988;McClorie et al, 1989;Crist et al, 1990;La Quaglia et al, 1990;Raney et al, 1990;La Quaglia, 1991;Massad et al, 1991). The high bladder salvage rate is the consequence of (a) our policy of treating local residual disease with irradiation rather than radical surgery, unless there was unequivocal persistent tumour-, (b) during serial endoscopic follow-up, cautious interpretation of 'positive' histopathological reports on biopsies taken from the site of previous tumour-bearing areas that appear macroscopically normal (Atra et al, 1994); and (c) cautious interpretation of follow-up pelvic computerised tomographic (Cr) scans (Atra et al, 1994).…”

“…Ghavimi et al (1984) for instance, reported a 50% bladder salvage rate among 18 survivors, and Pratt et al (1984) have reported a 73% survival and 81% bladder salvage rate. However, very few reports even mention the functional status of the bladders or the upper urinary tracts (Ortega, 1979;Voute et al, 1981;Hays et al, 1982Hays et al, , 1990Grosfeld et al, 1983;Ghavimi et al, 1984;Pratt, 1984;Maurer et al, 1988;McLorie et al, 1989;Crist et al, 1990;Raney et al, 1990;La Quaglia, 1991;Massad et al, 1991) and to our knowledge no detailed studies, such as this one, have been published.…”

“…Overall 3 year survival rates of 70-78% have been reported (Maurer et al, 1988;Raney et al, 1990). The challenge now is to find a suitable combination of treatment modalities that will maintain this high cure rate with a minimum of morbidity -'cure at least cost'.…”

Treatment modalities of bladder/prostate rhabdomyosarcoma: a review

“…Bladder preservation in those alive 2 and 3 y after starting treatment were 26 and 23%, respectively. 31 Some crucial points were learned in IRS I. Specifically related to B/P RMS, lymph node involvement could not be correlated with recurrence or survival rates.…”

“…Specifically related to B/P RMS, lymph node involvement could not be correlated with recurrence or survival rates. 31 Lawrence et al 32 recommended 'routine regional lymph node dissection for all G/U primary sites that are primarily treated by surgical resection.' The IRS specifically looked at nodal involvement in GU B/P, recommending combined CTX/radiation therapy (RT), with RT doses delivered over 4 weeks.…”