Perineal Leiomyosarcoma: avoid sequelae or prevent recurrence?

Abstract

Perineal Leiomyosarcomas are malignant tumors belonging to sarcoma´s family, constituting the 3% of them and the 0,04% of all neoplasias. Perineal location es extremely infrequently and this poses an additional difficulty because of the absence of anatomic boundaries and proximity to and encroachment on multiple organs. The overall local recurrent and metastatic rate are 37% and 62% respectively, that’s why aggressive surgical strategies with wide margins are usually recommended to decrease this rate, in addition to chemo-radiotherapy if needed. We present the case of a 42 year old female, with a right perineal mass that was slowly growing for 9 years. On physical examination we observed a mobile mass not attached to deep structures, painless witch eventually became symptomatic. CT scan cannot rule out the invasion of the right lateral wall of rectum. Discussion of case in tumor´s committee. Surgical approach: marginal resection of tumor. Histopathological study informed a low grade leiomyosarcoma. At the moment she is been strictly followed up with CT and MRI scans, without evidence of local recurrence. We consider that marginal resection might be an alternative in the treatment of this disease when it comes to selected low risks patients, with localized tumor in order to prevent surgeries with high impact in quality of life.  More studies are needed to guarantee the safety and benefits of this therapy.