Case Report: Primary Yolk Sac (Endodermal Sinus) Tumour of the Vulva

Objective: To report a case of primary yolk sac (endodermal sinus) tumour (YST) of the vulva which is very rare and to highlight some unusual features of this tumour in the vulva. Clinical Presentation and Intervention: A 23-year-old woman presented with a 1-month swelling in the right labium majus that was non-tender and rapidly increasing in size. The mass was excised; it grossly measured 6 × 5 × 3 cm and was firm with a grey white cut surface. Microscopically it showed features of YST. The patient returned to the clinic only after 2 months with a recurrent mass of almost the same size with ipsilateral lymphadenopathy. At that time serum α-fetoprotein showed only a mild increase (13.4 µg/l, normal: 0–10 µg/l). Further management was not possible as she decided to return to her native country for treatment. A literature review shows that local excision with lymphadenectomy followed by cisplatin-based chemotherapy is the best mode of treatment for primary YST of the vulva. Conclusion: This case is presented for its rarity and to highlight the fact that serum α-fetoprotein estimation may not aid in the primary diagnosis or detection of recurrence in patients on follow-up for YST arising in the vulva unlike those seen in the gonads.