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Primary Ewing sarcoma/peripheral primitive neuroectodermal tumor of the vulva.
Y Erika Fong1, Dolores López-Terrada, Qihui Jim Zhai
1Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA.
Human Pathology
|July 8, 2008
Summary
This case study details a rare vulvar Ewing sarcoma/primitive neuroectodermal tumor (ES/pPNET) in an adolescent. Molecular analysis confirmed the diagnosis, highlighting the rarity of this cancer in the female genital tract.
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Extraskeletal Ewing sarcoma/primitive neuroectodermal tumor (ES/pPNET) is a rare malignancy.
- Primary involvement of the female genital tract by ES/pPNET is exceptionally uncommon.
Observation:
- A 17-year-old adolescent presented with a vulvar tumor.
- Histological examination revealed a monomorphic small round blue cell population with cytoplasmic glycogen.
- Immunohistochemistry showed positivity for CD99 and Fli-1.
Findings:
- The tumor exhibited characteristic histological and immunohistochemical features of ES/pPNET.
- Reverse transcription polymerase chain reaction and sequencing confirmed the presence of an EWS/Fli-1 fusion transcript.
- This confirmed the diagnosis of ES/pPNET in the vulva.
Implications:
- This case adds to the limited literature on vulvar ES/pPNET.
- Highlights the importance of molecular confirmation in diagnosing rare soft tissue tumors.
- Underscores the need for awareness of ES/pPNET in gynecological oncology, even in extraskeletal locations.
