Leiomyosarcoma

Excerpt

Soft-tissue sarcoma (STS) arises mainly from the embryonic mesoderm with some contribution from the neuroectoderm. STS is a rare malignancy that accounts for less than 1% of all adult cancers. It encompasses a heterogeneous group of tumors with over 175 molecular subtypes. Leiomyosarcoma (LMS) is one of the more common subtypes of STS, comprising up to 10-20% of all sarcomas. Classically, LMS would either originate directly from the smooth muscle cells or from the precursor mesenchymal stem cells that would eventually differentiate into smooth muscle cells.

Although these cells are present everywhere, LMS shows a predilection for soft tissues and abdominopelvic organs compared to extremities. The genetic abnormalities in LMS are very complex and make it moderately sensitive to chemotherapy. Although disputed in literature, the behavior of LMS and sensitivity to treatment seems to depend on the organ of origin. An interprofessional approach is deemed necessary for the treatment of LMS. Patients with STS, when treated at centers that experience a high volume of such patients, have been shown to have better outcomes. The advent of targeted agents and immunotherapy, along with our increasing understanding of molecular subtypes of various STS, has ushered in a new era of treatment for STS.