# Leiomyosarcoma

Source: https://onco.cc/cancers/leiomyosarcoma/  
OnCo record `leiomyosarcoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Leiomyosarcoma is a cancer of smooth muscle, most often in the womb, the abdomen behind the bowel or the wall of a large vein. Surgery is the only cure; for spread disease, doxorubicin-based chemotherapy, trabectedin and gemcitabine-docetaxel are the standards, with no targeted drug yet.

## Summary

Leiomyosarcoma arises from smooth muscle and is genomically complex, with TP53, RB1 and ATRX loss and no recurrent fusion. Uterine leiomyosarcoma is usually found unexpectedly after surgery for presumed fibroids, which is why morcellation of unsuspected tumours is avoided; retroperitoneal and vascular tumours present late. Complete surgical resection, with radiotherapy for limb tumours, is the only curative treatment and adjuvant chemotherapy has not proven benefit. Metastatic disease is treated with doxorubicin, alone or with trabectedin (LMS-04) or dacarbazine, gemcitabine-docetaxel, trabectedin and pazopanib; responses are modest and median survival is about two years. Immunotherapy has little activity, and trials target the disease's DNA repair and cell-cycle vulnerabilities.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: LMS; Uterine leiomyosarcoma; Smooth muscle sarcoma
- Tags: subtype-page
- Group: sarcoma
- Burden: One of the two commonest soft-tissue sarcomas in adults, arising in the uterus, the retroperitoneum, large veins and limbs; about half of patients with deep tumours eventually develop metastases, most often to the lungs.
- Subtypes: Uterine leiomyosarcoma; Retroperitoneal and intra-abdominal leiomyosarcoma; Vascular leiomyosarcoma (inferior vena cava, large veins); Limb and cutaneous leiomyosarcoma (better outlook)
- Biomarkers: TP53, RB1 and ATRX alterations; Grade (FNCLCC) and size; Smooth muscle markers (desmin, h-caldesmon, SMA); Oestrogen and progesterone receptor in uterine tumours (hormone therapy in low-grade disease)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/leiomyosarcoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/leiomyosarcoma/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/leiomyosarcoma/#what-it-is [4 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/leiomyosarcoma/#finding-it [4 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/leiomyosarcoma/#treating-it [3 settings, 2 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/leiomyosarcoma/#evidence [5 trials, 5 key papers, 4 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/leiomyosarcoma/#science [8 targets]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/leiomyosarcoma/where-you-are/
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/leiomyosarcoma/#living-with-it [14 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/leiomyosarcoma/coming/ [7 medicines, 5 trials, 3 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/leiomyosarcoma/data/ [40 connected records]

## Standard of care

- Localised: Wide surgical resection; radiotherapy for limb and trunk tumours; no morcellation of uterine masses that could be sarcoma; adjuvant chemotherapy not standard. ([IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/))
- Metastatic, first line: Doxorubicin plus trabectedin (LMS-04) or doxorubicin alone; gemcitabine-docetaxel as an alternative, especially in uterine disease. ([Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Trabectedin](https://onco.cc/drugs/trabectedin/), [Gemcitabine](https://onco.cc/drugs/gemcitabine/), [Docetaxel](https://onco.cc/drugs/docetaxel/))
- Later lines: Trabectedin, pazopanib, dacarbazine-based regimens, aromatase inhibitors in hormone receptor-positive low-grade uterine disease; metastasectomy for limited lung spread. ([Trabectedin](https://onco.cc/drugs/trabectedin/), [Pazopanib](https://onco.cc/drugs/pazopanib/), [Letrozole (and other aromatase inhibitors)](https://onco.cc/drugs/letrozole/))

## State of the art

- LMS-04 gave the first randomised progression-free survival gain in a decade by combining doxorubicin with trabectedin.
- Lung metastasectomy in selected patients produces long survival that chemotherapy alone does not.
- Genomic profiling has defined the disease but not yet yielded a targeted drug.

## Open problems

- No targeted or immune therapy works in most patients.
- Uterine tumours are still discovered by accident after fibroid surgery.
- Adjuvant chemotherapy remains unproven.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Leiomyosarcoma
- Wikipedia: https://en.wikipedia.org/wiki/Leiomyosarcoma

## Connected records

- cancers: [Gastrointestinal stromal tumour (GIST)](https://onco.cc/cancers/gist/), [Liposarcoma](https://onco.cc/cancers/liposarcoma/), [Myxofibrosarcoma](https://onco.cc/cancers/myxofibrosarcoma/), [Retroperitoneal sarcoma](https://onco.cc/cancers/retroperitoneal-sarcoma/), [Sarcomas (soft tissue, bone, GIST)](https://onco.cc/cancers/sarcoma/), [Soft tissue sarcoma of the extremity (localised and advanced)](https://onco.cc/cancers/extremity-soft-tissue-sarcoma/), [Synovial sarcoma](https://onco.cc/cancers/synovial-sarcoma/), [Undifferentiated pleomorphic sarcoma (UPS)](https://onco.cc/cancers/undifferentiated-pleomorphic-sarcoma/), [Uterine sarcoma](https://onco.cc/cancers/uterine-sarcoma/), [Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma)](https://onco.cc/cancers/vascular-tumours/)
- key papers: [EORTC 62012: doxorubicin alone versus intensified doxorubicin plus ifosfamide for first-line treatment of advanced soft tissue sarcoma](https://onco.cc/key-papers/paper-eortc-62012-doxorubicin-ifosfamide-judson-lancet-oncol-2014/), [Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma: results of a phase 2 trial](https://onco.cc/key-papers/paper-hensley-gemcitabine-docetaxel-leiomyosarcoma-jco-2002/), [LMS-04: doxorubicin plus trabectedin followed by trabectedin maintenance versus doxorubicin alone in metastatic leiomyosarcoma](https://onco.cc/key-papers/paper-lms-04-doxorubicin-trabectedin-lancet-oncol-2022/), [Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS clinical practice guideline](https://onco.cc/key-papers/paper-esmo-sts-guideline-gronchi-ann-oncol-2021/), [Trabectedin versus dacarbazine for metastatic liposarcoma or leiomyosarcoma after anthracycline failure](https://onco.cc/key-papers/paper-trabectedin-vs-dacarbazine-demetri-jco-2016/)
- drugs: [Docetaxel](https://onco.cc/drugs/docetaxel/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Gemcitabine](https://onco.cc/drugs/gemcitabine/), [Ifosfamide](https://onco.cc/drugs/ifosfamide/), [Letrozole (and other aromatase inhibitors)](https://onco.cc/drugs/letrozole/), [Pazopanib](https://onco.cc/drugs/pazopanib/), [Trabectedin](https://onco.cc/drugs/trabectedin/)
- technologies: [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Methylation classifier for sarcomas](https://onco.cc/technologies/sarcoma-methylation-classifier/)
- people: [Patricia Pautier](https://onco.cc/people/patricia-pautier/), [Patrick Schöffski](https://onco.cc/people/patrick-schoffski/)
- trials: [Depsipeptide (Romidepsin) in Treating Patients With Metastatic or Unresectable Soft Tissue Sarcoma](https://onco.cc/trials/nct00112463/), [LMS-04](https://onco.cc/trials/lms-04/), [Phase III Trial of Anlotinib, Catequentinib in Advanced Alveolar Soft Part Sarcoma, Leiomyosarcoma, Synovial Sarcoma (APROMISS)](https://onco.cc/trials/nct03016819/), [STRASS (EORTC 62092)](https://onco.cc/trials/strass/), [Ziv-aflibercept in Treating Patients With Locally Advanced, Unresectable, or Metastatic Gynecologic Soft Tissue Sarcoma](https://onco.cc/trials/nct00390234/)
- companies: [French Sarcoma Group](https://onco.cc/companies/french-sarcoma-group/)
- targets: [CAMTA1](https://onco.cc/targets/camta1/), [DDX6](https://onco.cc/targets/ddx6/), [GLI1](https://onco.cc/targets/gli1/), [USP9X](https://onco.cc/targets/usp9x/), [WNK2](https://onco.cc/targets/wnk2/)

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JSON: https://onco.cc/api/v1/entities/leiomyosarcoma.json