DOI: 10.36347/sasjs.2024.v10i07.005

Pages: 762-766

Pleomorphic leiomyosarcoma is a rare entity that is part of the pleomorphic sarcomas with a complex genome with a high metastatic capacity and a usually poor prognosis. The most common location remains the lower extremities but other sites have been reported in the literature, particularly in the chest wall. Leiomyosarcoma (LMS) is an exceptionally rare tumor in the chest wall. Classical LMS may exhibit focal areas of pleomorphic cells; however, the coexistence of a large pleomorphic component allows the tumor to be designated as pleomorphic leiomyosarcoma (PLMS). The first cases of pleomorphic leiomyosarcoma were reported in the literature by Brooks in 1985. The pleomorphic component of these tumors is often heterologous with either cartilaginous differentiation; bony or rhabdomyosarcomatous. Since pleomorphic myogenic sarcomas, including high-grade leiomyosarcomas and rhabdomyosarcomas, have a poorer prognosis hence the importance of an accurate and valid histological diagnosis. The thoracic location of a leiomyosarcoma remains exceptional or even extremely rare and management requires multidisciplinary consultation involving the surgeon; the oncologist and the radiotherapist with additional molecular study to properly detect genetic anomalies in order to consider possible targeted therapy.