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Peripheral primitive neuroectodermal tumor (PNET) of the vulva: a case report
1Department of Obstetrics and Gynecology, Turkish Kidney Foundation, Hizmet Hospital, Istanbul, Turkey. saithalil@hotmail.com
European Journal of Gynaecological Oncology
|March 31, 2011
Summary
A rare vulvar Ewing's sarcoma/peripheral primitive neuroectodermal tumor (ES-PNET) occurred in a 14-year-old girl. Despite treatment, the adolescent patient died from metastasis, highlighting the aggressive nature of this rare vulvar cancer.
Area of Science:
- Oncology
- Pathology
Background:
- Ewing's sarcoma/peripheral primitive neuroectodermal tumor (ES-PNET) is a high-grade malignant neoplasm typically affecting the skeletal system.
- Primary extraskeletal ES-PNET is rare, with infrequent occurrences in the female genital tract, including the vulva.
Observation:
- A 14-year-old adolescent presented with a four-month history of a left vulvar mass.
- Surgical excision and re-excision were performed, followed by chemotherapy and radiotherapy.
Findings:
- The case involved a rare vulvar ES-PNET with distinct rosette-like structures.
- The adolescent patient experienced a very poor prognosis, succumbing to pulmonary metastasis within nine months.
Implications:
- This case underscores the extreme rarity of vulvar ES-PNET and its aggressive behavior in adolescents.
- Highlights the need for awareness and further research into effective treatment strategies for this rare malignancy.
