Eh. Hwang et al., CLINICAL-EXPERIENCE WITH CONSERVATIVE SURGERY FOR VAGINAL ENDODERMAL SINUS TUMOR, Journal of pediatric surgery, 31(2), 1996, pp. 219-222
Two cases of vaginal endodermal sinus tumor (EST), a rare pediatric ma
lignancy, were managed with conservative surgery followed by adjuvant
chemotherapy. The first case involved a 9-month-old girl with EST of t
he vagina, who was treated with a partial vaginectomy and VAC regimen
(vincristine, actinomycin D, cyclophosphamide) during a 2-year period.
The serum alpha-fetoprotein (AFP) level returned to normal after exci
sion of the tumor, and it remained normal throughout the treatment per
iod. There was no evidence of disease 30 months after diagnosis. The s
econd case involved an 8-month-old girl with EST of She vagina, who wa
s treated with local excision of the mass through a vaginotomy. The VA
C regimen was administered, but the serum AFP level remained elevated.
A follow-up abdominopelvic computed tomography scan, taken 4 months a
fter the operation, showed local recurrence of the tumor. The VAC regi
men was then changed to a BEP regimen (bleomycin, etoposide, cisplatin
). The serum AFP level returned to normal after 2 courses of the new r
egimen, and no tumor was visible on the follow-up magnetic resonance i
maging study. For vaginal EST, primary conservative surgery and adjuva
nt chemotherapy are attractive measures to preserve both reproductive
and sexual function. The extent of conservative surgery requires at le
ast a partial vaginectomy. Simple tumor excision may not be adequate t
o achieve cure or to prevent local recurrence, even with adjuvant chem
otherapy. The serum AFP level is useful for diagnosing and monitoring
vaginal EST in the infant. Copyright (C) 1996 by W.B. Saunders Company