Primary Ewing sarcoma of vulva, confirmed with molecular cytogenetic analysis: A rare case report with diagnostic and treatment implications.
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Date
2015-07
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Abstract
Primary vulvar Ewing sarcoma (ES)/PNET is an uncommonly documented tumor,
especially with molecular results. A 10-year-old girl presented with left vulvar swelling,
a year ago. Her abdominopelvic ultrasound revealed a 12 cm × 8 cm sized, mixed
echogenic blood-fi lled lesion in the left vulva; radiologically considered as a hematoma.
Vulvectomy revealed a multinodular grey-brown tumor, microscopically comprising
malignant round cells. Immunohistochemically, tumor cells diffusely expressed MIC2/
CD99 and Fli1 and subsequently displayed EWSR1 rearrangement, confi rming
diagnosis of ES/PNET. Subsequently, PET-CT scan revealed residual local lesion
with lung metastases. The patient was induced on EFT 2001 chemotherapy
protocol. Three months after chemotherapy completion, there was no metabolically
active disease on PET scan. Four months later, MRI disclosed recurrent primary
and metastatic pulmonary lesions. She was planned for scar excision and adjuvant
radiotherapy, but unfortunately defaulted further treatment. This forms the eighth case
of primary vulvar ES/PNET confi rmed with molecular cytogenetic result, underscoring
therapeutic value of objective diagnosis in such cases.
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Keywords
Ewing sarcoma/primitive neuroectodermal tumor, EWSR1 rearrangement, female genital tract sarcomas, vulvar tumors
Citation
Rekhi Bharat, Chinnaswamy Girish, Vora Tushar, Shah Sneha, Rangarajan Venkatesh. Primary Ewing srcoma of vulva, confirmed with molecular cytogenetic analysis: A rare case report with diagnostic and treatment implications. Indian Journal of Pathology & Microbiology. 2015 Jul-Sept 58(3): 341-344.