New perspectives on therapy for vaginal endodermal sinus tumors.
OBJECTIVE
Malignant germ cell tumors account for 3% of childhood cancers. Endodermal sinus tumor, the most common malignant germ cell tumor, requires treatment primarily with chemotherapy and surgery is reserved as a last resort. It is rare for the vagina to be the primary site of endodermal sinus tumor, and we report on our experiences with this phenomenon at a single institution.


METHODS
We retrospectively reviewed the clinical features, treatment and outcomes of 3 children with vaginal endodermal sinus tumor.


RESULTS
Initial treatment was combination chemotherapy, in 2 patients. alpha-fetoprotein decreased rapidly and returned to normal in both. One patient is disease-free 7 years after chemotherapy and the other patient is currently disease-free with a normal alpha-fetoprotein 3 months after induction chemotherapy. In the remaining case progressive disease developed following initial chemotherapy and subsequent salvage surgery combined with radiation, chemotherapy and ultimately autologous bone marrow transplant was performed. The patient has remained disease-free for 6.5 years since completing this extensive therapy.


CONCLUSIONS
Endodermal sinus tumor of the vagina is rare. All of our patients presented with painless bleeding of no obvious source. In such cases one must maintain a high index of suspicion for possible underlying pathological conditions even if ultrasound is negative. Evaluation must include endoscopic examination of the lower genitourinary tract. Bone marrow transplant should be considered as a last therapeutic resort in salvage cases of unresponsive vaginal endodermal sinus tumor.
Department of Pediatric Urology, The Children's Hospital and The University of Colorado School of Medicine, Denver, Colorado, USA.
Liann N
LN
Handel
Stephen M
SM
Scott
Roger H
RH
Giller
Brain S
BS
Greffe
Mark A
MA
Lovell
Martin A
MA
Koyle
D003131:Combined Modality Therapy; D018240:Endodermal Sinus Tumor / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D020360:Neoadjuvant Therapy; D009367:Neoplasm Staging; D012189:Retrospective Studies; D016879:Salvage Therapy; D015996:Survival Rate; D014621:Vagina / Q000473:pathology; D014625:Vaginal Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*