Peripheral Neuroectodermal Tumor of the Vulva

Abstract

In Brief Background. Peripheral neuroectodermal tumors arising from vulva are extremely rare; only 12 cases have been reported so far. These tumors belong to the Ewing sarcoma family of tumors. They occur in bones and variety of soft tissues of chest wall, pelvis, paravertebral region, and lower extremities. Being aggressive tumors, they have poor prognosis. Case. We report a case of a 20-year-old woman who presented with a 20 × 15 × 10-cm pedunculated mass arising from the right labium majus. Judicious use of a panel of markers led to diagnosis of peripheral neuroectodermal tumor. The patient died of metastatic disease. Conclusions. Judicious use of a panel of markers and a molecular test, if necessary, helps in confirming the diagnosis. Surgery followed by chemotherapy with or without radiation is the treatment of choice. Peripheral neuroectodermal tumors of the vulva are rare and aggressive tumors. Judicious use of a panel of markers and a molecular test helps confirm the diagnosis.