Background: Myxoid leiomyosarcomas originating from the Bartholin gland are exceedingly rare. Only one other case of a Bartholin gland myxoid leiomyosarcoma has been reported.
Case: Our patient presented with a 10 cm mass on her vulva, which was presumed to be a Bartholin gland cyst. Pathology showed a high-grade myxoid leiomyosarcoma with positive margins. She was treated with left radical hemivulvectomy, laparoscopic hysterectomy with bilateral salpingo-oophorectomy, and six cycles of adjuvant chemotherapy. She is now 1 year from completion of therapy and remains disease-free.
Conclusion: All solid vulvar masses should be thoroughly evaluated with a low threshold for biopsy. Treatment should consist of complete resection. Hormonal manipulation, chemotherapy, and radiation should be considered as potential adjuvant treatment options.