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Leiomyosarcoma

Cancer and Oncology · NLM MeSH · updated 2026-10-29 · version 1

Citations verified against PubMedNot yet clinician-reviewed10 referencesApproximately 4 min read

A sarcoma containing large spindle cells of smooth muscle.

Table of Contents

1. Definition2. Research landscape3. Reading the evidence4. Open-access studies5. Key reviews6. References7. About this article

Definition and research guide

This page combines a MeSH definition, publication statistics and selected research abstracts. It is a starting point for reading the literature, not a medical textbook or a systematic evidence review.

Content available: 4 complete abstracts (651 words), 10 references. Full article text is not stored or reproduced here; follow the PMID, DOI or PMC links to read the original sources and their access terms.

Definition

A sarcoma containing large spindle cells of smooth muscle. Although it rarely occurs in soft tissue, it is common in the viscera. It is the most common soft tissue sarcoma of the gastrointestinal tract and uterus. The median age of patients is 60 years. (From Dorland, 27th ed; Holland et al., Cancer Medicine, 3d ed, p1865)

Definition: NLM Medical Subject Headings (MeSH) scope note (Public domain (U.S. National Library of Medicine); attribution requested). View the MeSH record.

Also known as: Leiomyosarcomas; Leiomyosarcoma, Epithelioid; Epithelioid Leiomyosarcoma; Epithelioid Leiomyosarcomas; Leiomyosarcomas, Epithelioid; Leiomyosarcoma, Myxoid; Leiomyosarcomas, Myxoid; Myxoid Leiomyosarcoma.

Where it fits in the MeSH classification

Broader topics
Neoplasms, Muscle Tissue; Sarcoma

Research landscape

PubMed indexes 9,796 records tagged with the MeSH heading “Leiomyosarcoma” (counts retrieved 2026-10-29). 1,047 of them (10.7%) were published from 2020 onward, and the busiest period so far is through 1999 with 4,999 records.

Reviews account for 1,241 records (12.7%) and meta-analyses for 15. Randomized controlled trials number 36 (0.4%) as a share of all records on the topic. Randomized trials make up only a small share of this literature, so much of what is known comes from laboratory, observational or descriptive work.

PubMed records by publication periodthrough 1999: 4,999 records; 2000-2009: 1,826 records; 2010-2019: 1,924 records; 2020 onward: 1,047 records4,999through 19991,8262000-20091,9242010-20191,0472020 onward
Records in PubMed indexed with this heading, by publication period. Real counts from NCBI E-utilities; the final period covers only part of the current year. Volume reflects how much has been published, not how strong the evidence is.
Show the data
PeriodRecords
through 19994,999
2000-20091,826
2010-20191,924
2020 onward1,047
Share of records by publication typeReviews: 12.7 percent; Meta-analyses: 0.2 percent; Randomized controlled trials: 0.4 percentReviews12.7% (1,241)Meta-analyses0.2% (15)Randomized controlled trials0.4% (36)
Share of all PubMed records on this heading that carry each publication type. Types overlap with other records, so the shares do not sum to 100%.
Publication-type chart data
Publication types among 9,796 PubMed records
TypeRecordsShare
Reviews1,24112.7%
Meta-analyses150.2%
Randomized controlled trials360.4%

Method: PubMed query "Leiomyosarcoma"[MeSH Terms] with publication-type and date filters, retrieved 2026-10-29.

Reading the evidence in cancer and oncology

Cancer research spans very different kinds of evidence. Laboratory studies in cell lines and animal models reveal mechanisms but often do not translate to patients. Early-phase trials mainly test safety and dosing, while phase 3 randomized trials and their meta-analyses are the usual basis for treatment conclusions. Outcomes also differ in meaning: tumour shrinkage, progression-free survival and overall survival are not interchangeable, and screening studies can be distorted by lead-time and overdiagnosis. When reading a result, check the cancer type and stage studied, the comparison treatment, and which outcome was measured.

Evidence from open-access studies

These open-access studies come from PubMed’s best-match results for this heading (papers whose main topic it is), ranked here by citation count, with one recent addition. Each abstract is reproduced in full under its recorded open licence so that you can read the authors’ own words. They illustrate the literature; they are not a systematic summary of it. Citation counts measure attention, not reliability or clinical benefit.

Study comparison — selected abstracts, not a systematic review
Study / authorsYearSource label / designEurope PMC citationsLicence / source
Lineage-defined leiomyosarcoma subtypes emerge years before diagnosis and determine patient survival
Anderson ND, Babichev Y, Fuligni F, Comitani F, Layeghifard M, Venier RE, et al.
2021Research article
Design not recorded in structured metadata
66cc by
PMID 34301934 · DOI · PMC8302638
Comprehensive Review of Uterine Leiomyosarcoma: Pathogenesis, Diagnosis, Prognosis, and Targeted Therapy
Yang Q, Madueke-Laveaux OS, Cun H, Wlodarczyk M, Garcia N, Carvalho KC, et al.
2024Review article
Design not recorded in structured metadata
40cc by
PMID 38994959 · DOI · PMC11240800
The proteomic landscape of soft tissue sarcomas
Burns J, Wilding CP, Krasny L, Zhu X, Chadha M, Tam YB, et al.
2023Research article
Design not recorded in structured metadata
36cc by
PMID 37386008 · DOI · PMC10310735
Immunotherapy for Leiomyosarcoma: Current Status and Perspectives
Qi H, Zhang H, Wang Q, Wang X, Min L, Zhou Y, et al.
2026Review article
Design not recorded in structured metadata
1cc by
PMID 42105786 · DOI · PMC13157314

Lineage-defined leiomyosarcoma subtypes emerge years before diagnosis and determine patient survival [1]

Research article Anderson ND, Babichev Y, Fuligni F, Comitani F, Layeghifard M, Venier RE, et al. · · 2021 · cited 66 times (Europe PMC)

Abstract reading summary — source excerpts
Opening excerpt
Leiomyosarcomas (LMS) are genetically heterogeneous tumors differentiating along smooth muscle lines.
Closing excerpt
Finally, by phylogenetic reconstruction, we present evidence that clones seeding lethal metastases arise decades prior to LMS diagnosis.
Interpretation limits
Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (173 words)

Leiomyosarcomas (LMS) are genetically heterogeneous tumors differentiating along smooth muscle lines. Currently, LMS treatment is not informed by molecular subtyping and is associated with highly variable survival. While disease site continues to dictate clinical management, the contribution of genetic factors to LMS subtype, origins, and timing are unknown. Here we analyze 70 genomes and 130 transcriptomes of LMS, including multiple tumor regions and paired metastases. Molecular profiling highlight the very early origins of LMS. We uncover three specific subtypes of LMS that likely develop from distinct lineages of smooth muscle cells. Of these, dedifferentiated LMS with high immune infiltration and tumors primarily of gynecological origin harbor genomic dystrophin deletions and/or loss of dystrophin expression, acquire the highest burden of genomic mutation, and are associated with worse survival. Homologous recombination defects lead to genome-wide mutational signatures, and a corresponding sensitivity to PARP trappers and other DNA damage response inhibitors, suggesting a promising therapeutic strategy for LMS. Finally, by phylogenetic reconstruction, we present evidence that clones seeding lethal metastases arise decades prior to LMS diagnosis.

Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 34301934 · DOI · PMC8302638

Comprehensive Review of Uterine Leiomyosarcoma: Pathogenesis, Diagnosis, Prognosis, and Targeted Therapy [2]

Review article Yang Q, Madueke-Laveaux OS, Cun H, Wlodarczyk M, Garcia N, Carvalho KC, et al. · · 2024 · cited 40 times (Europe PMC)

Abstract reading summary — source excerpts
Opening excerpt
Uterine leiomyosarcoma (uLMS) is the most common subtype of uterine sarcomas.
Closing excerpt
Comprehensive, integrated, and deeper insights into the pathobiology and underlying molecular mechanisms of uLMS will help develop novel strategies to treat patients with this aggressive tumor.
Interpretation limits
Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (208 words)

Uterine leiomyosarcoma (uLMS) is the most common subtype of uterine sarcomas. They have a poor prognosis with high rates of recurrence and metastasis. The five-year survival for uLMS patients is between 25 and 76%, with survival rates approaching 10-15% for patients with metastatic disease at the initial diagnosis. Accumulating evidence suggests that several biological pathways are involved in uLMS pathogenesis. Notably, drugs that block abnormal functions of these pathways remarkably improve survival in uLMS patients. However, due to chemotherapy resistance, there remains a need for novel drugs that can target these pathways effectively. In this review article, we provide an overview of the recent progress in ascertaining the biological functions and regulatory mechanisms in uLMS from the perspective of aberrant biological pathways, including DNA repair, immune checkpoint blockade, protein kinase and intracellular signaling pathways, and the hedgehog pathway. We review the emerging role of epigenetics and epitranscriptome in the pathogenesis of uLMS. In addition, we discuss serum markers, artificial intelligence (AI) combined with machine learning, shear wave elastography, current management and medical treatment options, and ongoing clinical trials for patients with uLMS. Comprehensive, integrated, and deeper insights into the pathobiology and underlying molecular mechanisms of uLMS will help develop novel strategies to treat patients with this aggressive tumor.

Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 38994959 · DOI · PMC11240800

The proteomic landscape of soft tissue sarcomas [3]

Research article Burns J, Wilding CP, Krasny L, Zhu X, Chadha M, Tam YB, et al. · · 2023 · cited 36 times (Europe PMC)

Abstract reading summary — source excerpts
Opening excerpt
Soft tissue sarcomas (STS) are rare and diverse mesenchymal cancers with limited treatment options.
Closing excerpt
Our study highlights the utility of proteomics for identifying molecular subgroups with implications for risk stratification and therapy selection and provides a rich resource for future sarcoma research.
Interpretation limits
Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (149 words)

Soft tissue sarcomas (STS) are rare and diverse mesenchymal cancers with limited treatment options. Here we undertake comprehensive proteomic profiling of tumour specimens from 321 STS patients representing 11 histological subtypes. Within leiomyosarcomas, we identify three proteomic subtypes with distinct myogenesis and immune features, anatomical site distribution and survival outcomes. Characterisation of undifferentiated pleomorphic sarcomas and dedifferentiated liposarcomas with low infiltrating CD3 + T-lymphocyte levels nominates the complement cascade as a candidate immunotherapeutic target. Comparative analysis of proteomic and transcriptomic profiles highlights the proteomic-specific features for optimal risk stratification in angiosarcomas. Finally, we define functional signatures termed Sarcoma Proteomic Modules which transcend histological subtype classification and show that a vesicle transport protein signature is an independent prognostic factor for distant metastasis. Our study highlights the utility of proteomics for identifying molecular subgroups with implications for risk stratification and therapy selection and provides a rich resource for future sarcoma research.

Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 37386008 · DOI · PMC10310735

Immunotherapy for Leiomyosarcoma: Current Status and Perspectives [4]

Review article Qi H, Zhang H, Wang Q, Wang X, Min L, Zhou Y, et al. · · 2026 · cited 1 times (Europe PMC)

Abstract reading summary — source excerpts
Opening excerpt
Leiomyosarcoma (LMS), one of the soft-tissue sarcomas (STSs), has a relatively higher risk of distant metastasis.
Closing excerpt
We also analyzed the classical immunotherapy led by immune checkpoint inhibitors (ICIs), individualized immunotherapy such as cell therapy, and gene editing technologies, which bring better effect mechanisms and survival benefits, as well as opportunities and challenges for large-scale clinical applications.
Interpretation limits
Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (121 words)

Leiomyosarcoma (LMS), one of the soft-tissue sarcomas (STSs), has a relatively higher risk of distant metastasis. This has resulted in current first-line treatments still failing to deliver satisfactory outcomes, and the current treatment of LMS remains controversial and challenging. Therefore, it is worth thinking about how to guide and decide on the direction of treatment for LMS. In this review, we analyzed the immune microenvironment, gene expression, and molecular landscape of LMS, hinting at future research directions and prospects for LMS. We also analyzed the classical immunotherapy led by immune checkpoint inhibitors (ICIs), individualized immunotherapy such as cell therapy, and gene editing technologies, which bring better effect mechanisms and survival benefits, as well as opportunities and challenges for large-scale clinical applications.

Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 42105786 · DOI · PMC13157314

Key reviews and meta-analyses

The most cited review-type papers among PubMed’s best-match results for this heading, listed with bibliographic details only. Follow the links for the full text and access terms.

  • Research article Provisional matrix: A role for versican and hyaluronan [5]
    Wight TN · Matrix Biol · 2017 · cited 194 times (Europe PMC)
  • Research article Soft Tissue and Uterine Leiomyosarcoma [6]
    George S, Serrano C, Hensley ML, Ray-Coquard I · J Clin Oncol · 2018 · cited 188 times (Europe PMC)
  • Research article Uterine leiomyosarcoma: Epidemiology, contemporary treatment strategies and the impact of uterine morcellation [7]
    Ricci S, Stone RL, Fader AN · Gynecol Oncol · 2017 · cited 137 times (Europe PMC)
  • Research article Uterine leiomyosarcoma: A review of the literature and update on management options [8]
    Roberts ME, Aynardi JT, Chu CS · Gynecol Oncol · 2018 · cited 128 times (Europe PMC)
  • Research article Impact of morcellation on survival outcomes of patients with unexpected uterine leiomyosarcoma: a systematic review and meta-analysis [9]
    Bogani G, Cliby WA, Aletti GD · Gynecol Oncol · 2015 · cited 127 times (Europe PMC)
  • Research article Leiomyosarcoma [10]
    Serrano C, George S · Hematol Oncol Clin North Am · 2013 · cited 118 times (Europe PMC)

References

  1. Anderson ND, Babichev Y, Fuligni F, Comitani F, Layeghifard M, Venier RE, et al.. Lineage-defined leiomyosarcoma subtypes emerge years before diagnosis and determine patient survival. . 2021. PMID 34301934 · DOI · PMC8302638
  2. Yang Q, Madueke-Laveaux OS, Cun H, Wlodarczyk M, Garcia N, Carvalho KC, et al.. Comprehensive Review of Uterine Leiomyosarcoma: Pathogenesis, Diagnosis, Prognosis, and Targeted Therapy. . 2024. PMID 38994959 · DOI · PMC11240800
  3. Burns J, Wilding CP, Krasny L, Zhu X, Chadha M, Tam YB, et al.. The proteomic landscape of soft tissue sarcomas. . 2023. PMID 37386008 · DOI · PMC10310735
  4. Qi H, Zhang H, Wang Q, Wang X, Min L, Zhou Y, et al.. Immunotherapy for Leiomyosarcoma: Current Status and Perspectives. . 2026. PMID 42105786 · DOI · PMC13157314
  5. Wight TN. Provisional matrix: A role for versican and hyaluronan. Matrix Biol. 2017. PMID 27932299 · DOI · PMC5438907
  6. George S, Serrano C, Hensley ML, Ray-Coquard I. Soft Tissue and Uterine Leiomyosarcoma. J Clin Oncol. 2018. PMID 29220301 · DOI · PMC5759317
  7. Ricci S, Stone RL, Fader AN. Uterine leiomyosarcoma: Epidemiology, contemporary treatment strategies and the impact of uterine morcellation. Gynecol Oncol. 2017. PMID 28209496 · DOI
  8. Roberts ME, Aynardi JT, Chu CS. Uterine leiomyosarcoma: A review of the literature and update on management options. Gynecol Oncol. 2018. PMID 30244960 · DOI
  9. Bogani G, Cliby WA, Aletti GD. Impact of morcellation on survival outcomes of patients with unexpected uterine leiomyosarcoma: a systematic review and meta-analysis. Gynecol Oncol. 2015. PMID 25462199 · DOI
  10. Serrano C, George S. Leiomyosarcoma. Hematol Oncol Clin North Am. 2013. PMID 24093170 · DOI

About this article

Sources
NLM Medical Subject Headings (definition, synonyms); NCBI PubMed (counts, identifiers); Europe PMC (open-access abstracts and citation counts).
Verification
Every reference was resolved against PubMed on 2026-10-31; title, year and DOI matched.
Review state
automated source verified; no human expert review has been recorded yet.
Version
1, last updated 2026-10-29.

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At a glance

9,796
PubMed records on this heading
11%
published since 2020
1,241
review articles
36
randomized trials
10
verified references below

Counts come from PubMed on 2026-10-29; they show how much has been written, not how strong the evidence is.

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  • CarcinosarcomaCancer and Oncology
  • ChondroblastomaCancer and Oncology

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