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Vulvar epithelioid sarcoma in pregnancy.
Richard G Moore1, Margaret M Steinhoff, Cornelius O Granai
1Department of Obstetrics and Gynecology, Program in Women's Oncology, Brown University, Providence, Rhode Island 02905, USA. rmoore@wihri.org
Gynecologic Oncology
|April 2, 2002
Summary
This case report details the first known instance of epithelioid sarcoma on the vulva during pregnancy. Aggressive surgical resection is recommended, though optimal management for this rare vulvar cancer remains uncertain.
Area of Science:
- Oncology
- Gynecologic Oncology
- Soft Tissue Pathology
Background:
- Epithelioid sarcoma is a rare soft tissue tumor, infrequently occurring centrally and exceptionally rare on the vulva.
- Vulvar sarcoma in pregnancy is exceedingly rare, with only five cases reported previously, none being epithelioid sarcoma.
Purpose of the Study:
- To report the first case of vulvar epithelioid sarcoma presenting during pregnancy.
- To review the literature on vulvar epithelioid sarcoma and discuss its biological behavior and management.
Main Methods:
- Case report of a 29-year-old woman with vulvar epithelioid sarcoma at 36 weeks gestation.
- Patient underwent radical hemivulvectomy postpartum, followed by doxorubicin and ifosfamide chemotherapy.
- Literature review of 18 previously reported cases of vulvar epithelioid sarcoma outside of pregnancy.
Main Results:
- The patient developed pulmonary metastasis despite radical surgery and chemotherapy, succumbing to tumor-related pulmonary failure 6-12 months post-diagnosis.
- This represents the first documented case of vulvar epithelioid sarcoma diagnosed during pregnancy.
Conclusions:
- Optimal management for vulvar epithelioid sarcoma is not yet established.
- Early and aggressive surgical resection appears to offer the best chance for cure.
- The roles of radiation and chemotherapy in managing this condition require further investigation.