Introduction/Background Epithelioid sarcoma (ES) of the vulva is a rare and an aggressive malignant soft tissue neoplasm. It is classified into distal, conventional, or classic type and proximal type. To the best of our knowledge, there are only less than 45 cases described in the English literature.

Methodology A retrospective reporting of a rare case of para-vulval epithelioid sarcoma proved histologically to be undifferentiated tumor with yolk sac elements.

Results We report a case of 25-year-old lady, medically free; was referred to our hospital following an excisional biopsy of a painless 7X5 cm-sized lump just outside the right labia majora, at a private hospital. Histopathologic examination revealed proximal type ES with infiltrated skin margin. Chest/Abdomen/Pelvis CT with contrast was free. After MDT discussion; wide local excision and bilateral inguinal lymphadenectomy were performed.

The postoperative histopathology revealed free tumor safety margin while right inguinal lymph nodes were invaded with undifferentiated tumor with yolk sac elements. Immunehistochemical study was done and confirmed diagnosis of ‘INI 1-deficient malignant neoplasm with yolk sac tumor’ that was confirmed by strong positive SALL4 and focal positive alpha-fetoprotein.

The case was discussed again in the tumor board and advised to start adjuvant chemotherapy.

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Abstract #237 Figure 1

Conclusion Para-vulval Epithelioid sarcoma of the vulva is rare. A multidisciplinary approach of management is needed.

Disclosures Non