Chapter 5: Leiomyosarcoma and Smooth Muscle Sarcomas

This is a tumor of smooth muscle (or its precursors) and can arise anywhere in the body. Leiomyosarcoma is one of the most common types of sarcoma. Common initial sites for this tumor are the uterus, small intestine or stomach, or the wall of a blood vessel in the abdomen, extremity, or skin. These sarcomas have a highly variable behavior, but when they metastasize they characteristically travel to the lungs and less commonly to the liver, bone, and soft tissues.

Leiomyosarcoma (LMS)

Alternative diagnostic terms
None

Common anatomical locations
Lower and upper extremities, retroperitoneum, abdomen/pelvis, and trunk.

Pathology comment
Fascicles of eosinophilic spindled cells with blunt-ended nuclei showing variable pleomorphism; immunolabeling for SMA, desmin, and/or caldesmon.

Source
Bridge JA, Dry SM, Fröhling S. Leiomyosarcoma. In: WHO Classification of Tumors Editorial Board. Soft tissue and bone tumors [Internet]. Lyon (France): International Agency for Research on Cancer; 2020 [2024 Dec 29]. (WHO Classification of Tumors Series, 5th ed.; vol. 3). Available from: https://tumourclassification.iarc.who.int/login?redirecturl=%2Fchapters%2F33


For diagnosis or treatment
Any center listed in the Sarcoma Centers Directory »

Patient advocacy
Any organization with an established program for patient triage, support, and education. See Patient Advocacy Directory »

Diagnostic-specific organizations:
National Leiomyosarcoma Foundation
Leiomyosarcoma
The Cynthia Soloman Holmes Foundation

Clinical trials
SARC Clinical Trials – None
Find clinical trials worldwide »


Inflammatory Leiomyosarcoma

Alternative diagnostic terms
None

Common anatomical locations
Most common in the deep soft tissues of the lower extremity.

Pathology comment
Fascicular proliferation of variably atypical eosinophilic spindle cells, which show mitotic activity; dense inflammatory infiltrate, most often lymphoid or histiocytic/xanthomatous, but the composition is variable; immunopositivity for smooth muscle antigens.

Source
Bridge JA, Fletcher CDM, Mertens F. Inflammatory leiomyosarcoma. In: WHO Classification of Tumors Editorial Board. Soft tissue and bone tumors [Internet]. Lyon (France): International Agency for Research on Cancer; 2020 [2024 Dec 29]. (WHO Classification of Tumors Series, 5th ed.; vol. 3). Available from: https://tumorclassification.iarc.who.int/chapters/33.


For diagnosis or treatment
Any center listed in the Sarcoma Center Directory »

Patient advocacy
Any organization with an established program for patient triage, support, and education. See Patient Advocacy Directory »

Clinical trials
SARC Clinical Trials – None
Find clinical trials worldwide »


Benign and Low-Grade Smooth Muscle Tumors

Alternative diagnostic terms
Pending

Common anatomical locations
Pending

Pathology comment
Pending

Source
Pending


For diagnosis or treatment
Any center listed in the Sarcoma Center Directory »

Patient advocacy
Any organization with an established program for patient triage, support, and education. See Patient Advocacy Directory »

Clinical trials
SARC Clinical Trials – None
Find clinical trials worldwide »


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