Vulvar sarcoma in a young patient with neurofibromatosis syndrome with missed opportunities: A case report

Abstract

Background: Vulvar malignancies are the fourth commonest of genital malignancies. The sarcoma type is associated with neurofibromatosis disease.

Case presentation: A 29-year-old nulliparous presented to the outpatient gynecological clinic with a five-year history of a recurrent right vulvar mass. The mass was initially excised without histological analysis. Histological examination of a reexcised mass diagnosed a spindle cell sarcoma. She was then lost to follow-up for one year. The mass recurred and she received external beam radiation. On admission, she was in good general status with generalized café-au-lait spots and neurofibromas. Perineal examination revealed an ulcerated right vulvar mass. Toilet vulvectomy and diversion colostomy were performed followed by chemotherapy. She was discharged after five months with a well-granulated wound. Colostomy was reversed six months later.

Conclusion: Vulvar sarcoma is a rare vulvar malignancy type usually associated with neurofibromatosis. This case highlights the missed opportunities in its management. A high suspicion threshold is critical for early diagnosis and management to curb the morbidity and mortality rate.