Uterine Sarcoma
ICD-10 C54 · ICD-11 2B5F.0

Treatment of Low-Grade Uterine Leiomyosarcoma, Hormone Receptor Positive, with Metastatic Disease

Low-grade uterine leiomyosarcoma (uLMS) that is hormone receptor positive and metastatic represents a distinct, indolent clinical subgroup. This subpopulation behaves differently from the more common high-grade uLMS, and management decisions should account for its characteristic biological behaviour.

Clinical Scenario

Confirmed low-grade uterine leiomyosarcoma, hormone receptor positive, with metastatic spread and no evidence of transformation to high-grade disease. Cases in this category are sometimes initially diagnosed as STUMP (smooth muscle tumor of uncertain malignant potential) and carry a lower recurrence risk than high-grade uLMS. Hormone receptor positivity is a clinically relevant feature of this subgroup.

Management Approach

For indolent low-grade metastatic disease, an initial management strategy exists that does not necessarily begin with systemic cytotoxic therapy — the full protocol details the recommended first-line approach and its supporting evidence.

Complete recommendations, including criteria for intervention and subsequent lines of therapy, are available in the full structured protocol.

References

DOI: 10.1136/ijgc-2024-005823

There is a small subgroup of patients with uLMS that are often diagnosed as STUMP (smooth muscle tumor of uncertain malignant potential) and behave as low-grade malignancies with a lower risk of recurrence than the much more common high-grade uLMS.

Importantly, they can respond to endocrine therapy (with aromatase inhibitors or progestins).

In indolent disease, active surveillance can be offered as initial management (IV, B).


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