Cystic clear cell epithelioid leiomyoma: An unusual presentation

Epithelioid leiomyoma is a rare variant of leiomyoma. The tumour includes three distinct entities, leiomyoblastoma, clear cell leiomyoma and plexiform leiomyoma. It is histologically characterised by epithelioid cells, clear cells or plexiform pattern in varied proportions. We report a case of a 49-year-old female with complaints of palpable mass per abdomen and spotting per vagina. Radiology revealed a complex cystic mass with obscured right ovary. She underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy. Pathological examination showed a posterior wall lesion with central 9 × 8 cm cyst with multiple septations and surrounding rim of solid areas. Microscopic examination showed a clear cell epithelioid leiomyoma. No mitosis/tumour cell necrosis was seen. The clinical course of epithelioid leiomyoma is still unclear owing to absence of large studies. Small size, clear cell histology, mitosis of < 2/10 High Power Field (HPF) and hyalinization are features of a benign course, whereas cellular atypia, mitosis of > 5/10 HPF are definite indicators of a malignant outcome. An epithelioid leiomyoma with, size >6 cm moderate atypia, necrosis and mitosis of 2-5/10 HPF are termed as smooth muscle tumours of uncertain malignant potential and should be under close follow up.