Abstract
The current management of pediatric vaginal and vulvar rhabdomyosarcoma (RMS) uses cytoreductive chemotherapy before surgical intervention. During the Intergroup Rhabdomyosarcoma Study (IRS)-III (1984 to 1988), 27 evaluable patients were entered on a preoperative chemotherapy protocol. Among 24 patients with vaginal primaries, 20 had an initial biopsy with gross residual (group III), 3 had resection with positive margins (group IIA) and 1 had metastatic disease (group IV). At subsequent surgery, 7 patients underwent partial or complete vaginectomy and 6 of them had no viable tumor identified in the specimen. Only 1 of these 7 patients underwent a cystectomy, whereas 5 underwent hysterectomy. Seventeen patients in this group have no evidence of disease 66 to 108 months after diagnosis; 2 died of chemotoxicity and 1 of unknown causes after achieving a complete response. Ten of these 17 patients were treated with biopsy and chemotherapy only. Four of these 10 had radiotherapy as well. All group IIA patients have no evidence of disease. The 1 group IV patient had rapidly progressive disease with early death. This primary chemotherapy protocol resulted in less need for operative intervention or irradiation compared with previous experience. There was no local recurrence, and 20 of 24 patients remain continuously relapsefree with no evidence of disease. All 3 patients with vulvar primaries were treated by wide local excision and chemotherapy and have no evidence of disease. Conservative surgical intervention for vaginal RMS with primary chemotherapy and adjunctive radiation when necessary appears to result in excellent disease-free survival.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 1034-1037 |
| Number of pages | 4 |
| Journal | Journal of pediatric surgery |
| Volume | 30 |
| Issue number | 7 |
| DOIs | |
| State | Published - Jan 1 1995 |
Fingerprint
Keywords
- Rhabdomyosarcoma
- pediatric
- vaginal vulvar
ASJC Scopus subject areas
- Surgery
- Pediatrics, Perinatology, and Child Health
Cite this
Conservative surgical management of vaginal and vulvar pediatric rhabdomyosarcoma : A report from the intergroup rhabdomyosarcoma study III. / Andrassy, Richard J.; Hays, Daniel M.; Raney, R. Beverly; Wiener, Eugene S.; Lawrence, Walter; Lobe, Thom E.; Corpron, Cynthia A.; Smith, Malcolm; Maurer, Harold Maurice.
In: Journal of pediatric surgery, Vol. 30, No. 7, 01.01.1995, p. 1034-1037.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Conservative surgical management of vaginal and vulvar pediatric rhabdomyosarcoma
T2 - A report from the intergroup rhabdomyosarcoma study III
AU - Andrassy, Richard J.
AU - Hays, Daniel M.
AU - Raney, R. Beverly
AU - Wiener, Eugene S.
AU - Lawrence, Walter
AU - Lobe, Thom E.
AU - Corpron, Cynthia A.
AU - Smith, Malcolm
AU - Maurer, Harold Maurice
PY - 1995/1/1
Y1 - 1995/1/1
N2 - The current management of pediatric vaginal and vulvar rhabdomyosarcoma (RMS) uses cytoreductive chemotherapy before surgical intervention. During the Intergroup Rhabdomyosarcoma Study (IRS)-III (1984 to 1988), 27 evaluable patients were entered on a preoperative chemotherapy protocol. Among 24 patients with vaginal primaries, 20 had an initial biopsy with gross residual (group III), 3 had resection with positive margins (group IIA) and 1 had metastatic disease (group IV). At subsequent surgery, 7 patients underwent partial or complete vaginectomy and 6 of them had no viable tumor identified in the specimen. Only 1 of these 7 patients underwent a cystectomy, whereas 5 underwent hysterectomy. Seventeen patients in this group have no evidence of disease 66 to 108 months after diagnosis; 2 died of chemotoxicity and 1 of unknown causes after achieving a complete response. Ten of these 17 patients were treated with biopsy and chemotherapy only. Four of these 10 had radiotherapy as well. All group IIA patients have no evidence of disease. The 1 group IV patient had rapidly progressive disease with early death. This primary chemotherapy protocol resulted in less need for operative intervention or irradiation compared with previous experience. There was no local recurrence, and 20 of 24 patients remain continuously relapsefree with no evidence of disease. All 3 patients with vulvar primaries were treated by wide local excision and chemotherapy and have no evidence of disease. Conservative surgical intervention for vaginal RMS with primary chemotherapy and adjunctive radiation when necessary appears to result in excellent disease-free survival.
AB - The current management of pediatric vaginal and vulvar rhabdomyosarcoma (RMS) uses cytoreductive chemotherapy before surgical intervention. During the Intergroup Rhabdomyosarcoma Study (IRS)-III (1984 to 1988), 27 evaluable patients were entered on a preoperative chemotherapy protocol. Among 24 patients with vaginal primaries, 20 had an initial biopsy with gross residual (group III), 3 had resection with positive margins (group IIA) and 1 had metastatic disease (group IV). At subsequent surgery, 7 patients underwent partial or complete vaginectomy and 6 of them had no viable tumor identified in the specimen. Only 1 of these 7 patients underwent a cystectomy, whereas 5 underwent hysterectomy. Seventeen patients in this group have no evidence of disease 66 to 108 months after diagnosis; 2 died of chemotoxicity and 1 of unknown causes after achieving a complete response. Ten of these 17 patients were treated with biopsy and chemotherapy only. Four of these 10 had radiotherapy as well. All group IIA patients have no evidence of disease. The 1 group IV patient had rapidly progressive disease with early death. This primary chemotherapy protocol resulted in less need for operative intervention or irradiation compared with previous experience. There was no local recurrence, and 20 of 24 patients remain continuously relapsefree with no evidence of disease. All 3 patients with vulvar primaries were treated by wide local excision and chemotherapy and have no evidence of disease. Conservative surgical intervention for vaginal RMS with primary chemotherapy and adjunctive radiation when necessary appears to result in excellent disease-free survival.
KW - Rhabdomyosarcoma
KW - pediatric
KW - vaginal vulvar
UR - http://www.scopus.com/inward/record.url?scp=0029146896&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0029146896&partnerID=8YFLogxK
U2 - 10.1016/0022-3468(95)90336-4
DO - 10.1016/0022-3468(95)90336-4
M3 - Article
VL - 30
SP - 1034
EP - 1037
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
SN - 0022-3468
IS - 7
ER -