Peripheral primitive neuroectodermal tumor (PNET) of the vulva: a case report

Ewing's sarcoma/peripheral primitive neuroectodermal tumor (ES-PNET) is a high-grade malignant neoplasm that often develops in the skeletal system. Primary extraskeletal ES-PNET is an uncommon condition that rarely affects the female genital tract. Tumors in the ovary, cervix, and uterine corpus and vulva are occasionally reported. Reports on the Ewing family of tumors involving the vulva are extremely rare in the relevant literature. Only a few cases of vulvar ES-PNET have so far been reported.

A 14-year-old adolescent girl presented to the clinic with a 4-month history of a left vulvar mass. The mass was excised under general anesthesia, and re-resection was performed three weeks later to obtain negative microscopic margins. The patient received chemotherapy and radiotherapy; however, she died of pulmonary metastasis within nine months of the initial surgery.

In summary, we describe a rare case of vulvar ES-PNET with distinct rosette-like structures in a 14-year-old adolescent girl with a very poor prognosis.