Introduction

Leiomyosarcoma of the vulva is a rare disease accounting for about 1% of all primary vulvar neoplasia but it is the most common type of vulvar sarcomas. Usually it arises from the smooth muscles, blood vessels, rough ligaments, and erector-pili muscles. No treatment algorithms have been established yet. Tumour excision with clean resection lines is considered sufficient, with radiation therapy applied in certain cases.

Case report

We report a case of a 73-year old patient in whom the disease was manifested by pain syndrome and rapidly growing mass with irregular margins in the symphysis area and satellite nodules. She underwent extensive local excision followed by radiation therapy.

Discussion

Isolated cases and limited series of LMS cases have been described in literature. Leiomyosarcoma is most commonly localized to the labia majora, the Bartholin gland area, clitoris and labia minora. It most often affected perimenopausal women but in younger and pregnant patients was described. The diagnosis is not always easy and different histological markers has to be used. There are no definitive therapeutic algorithms due to the rarity of the disease. The management is surgical treatment and the entire tumour must be removed with histologically verified clean resection margins, followed by radiation therapy in some cases.

Conclusions

Vulvar tumours are difficult to distinguish macroscopically. Accurate histological diagnosis allows adequate treatment.