Leiomyosarcoma (LMS)
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-LEIOMYOSARCOMA |
|---|---|
| Type | Disease |
| Aliases | Cutaneous / soft-tissue leiomyosarcomaLMSLeiomyosarcomaRetroperitoneal leiomyosarcomaUterine leiomyosarcoma (uLMS)Лейоміосаркома (LMS) |
| Status | reviewed 2026-05-19 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-ESMO-SARCOMA-2024 SRC-NCCN-SARCOMA SRC-NCCN-UTERINE-2025 |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 8890/3 |
|---|---|
| icd_o_3_topography | ["C49", "C54.2"] |
| icd_10 | C49, C54.2 |
| who_classification | WHO Soft Tissue and Bone Tumours 5th ed (2020): Leiomyosarcoma; WHO Female Genital Tumours 5th ed (2020): Uterine leiomyosarcoma |
Notes
Leiomyosarcoma is a smooth-muscle-origin sarcoma occupying ~15% of adult soft-tissue sarcoma volume. This entity is authored as a prevention / hereditary anchor for genetic-syndrome surveillance workstreams (HLRCC, Li-Fraumeni); for general STS systemic-therapy decision support the parent entity DIS-SOFT-TISSUE-SARCOMA remains the canonical reference, which treats LMS as one of several histological subtypes. Three anatomic subsets with distinct biology + clinical context: - Retroperitoneal / abdominal-pelvic LMS (~40%): often arises from inferior vena cava or great vessels; surgery is principal modality; chemo-sensitivity moderate (doxorubicin-based 1L). - Uterine leiomyosarcoma (uLMS, ~25%): post-menopausal; presents as rapidly enlarging uterine mass; often misdiagnosed pre-operatively as benign leiomyoma. Total abdominal hysterectomy + bilateral salpingo-oophorectomy for localized disease; chemo benefit modest (LMS-02 / GeDDiS). uLMS has a distinct hereditary anchor (HLRCC, germline FH). - Cutaneous + extremity LMS (~25%): better prognosis when small + superficial; wide local excision often curative. Hereditary syndromes and prevention workstream relationships: - HLRCC (germline...
Used By
Disease
DIS-RETINOBLASTOMA- Retinoblastoma
Red flag
RF-IATROGENIC-PRIOR-RADIATION-PREVENTION- History of therapeutic radiation exposure ≥5 years ago to a body region — most commonly m...RF-LI-FRAUMENI-CONFIRMED-CARRIER- Patient has a confirmed germline TP53 pathogenic variant (positive germline test, distinc...