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Sarcoma

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

Citations verified against PubMedNot yet clinician-reviewed9 referencesApproximately 4 min read

A connective tissue neoplasm formed by proliferation of mesodermal cells; it is usually highly malignant.

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: 3 complete abstracts (730 words), 9 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 connective tissue neoplasm formed by proliferation of mesodermal cells; it is usually highly malignant.

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: Sarcomas; Sarcoma, Soft Tissue; Sarcomas, Soft Tissue; Soft Tissue Sarcoma; Soft Tissue Sarcomas; Sarcoma, Epithelioid; Epithelioid Sarcoma; Epithelioid Sarcomas.

Where it fits in the MeSH classification

Broader topics
Neoplasms, Connective and Soft Tissue
Narrower topics
Adenosarcoma; Carcinosarcoma; Chondrosarcoma; Desmoplastic Small Round Cell Tumor; Endometrial Stromal Tumors; Fibrosarcoma; Hemangiosarcoma; Histiocytoma, Malignant Fibrous; Leiomyosarcoma; Liposarcoma; Lymphangiosarcoma; Mixed Tumor, Mesodermal; Myosarcoma; Myxosarcoma
See also
Avian Sarcoma Viruses

Research landscape

PubMed indexes 160,507 records tagged with the MeSH heading “Sarcoma” (counts retrieved 2026-10-29). 22,755 of them (14.2%) were published from 2020 onward, and the busiest period so far is through 1999 with 77,721 records.

Reviews account for 16,566 records (10.3%) and meta-analyses for 450. Randomized controlled trials number 747 (0.5%) 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: 77,721 records; 2000-2009: 26,912 records; 2010-2019: 33,119 records; 2020 onward: 22,755 records77,721through 199926,9122000-200933,1192010-201922,7552020 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 199977,721
2000-200926,912
2010-201933,119
2020 onward22,755
Share of records by publication typeReviews: 10.3 percent; Meta-analyses: 0.3 percent; Randomized controlled trials: 0.5 percentReviews10.3% (16,566)Meta-analyses0.3% (450)Randomized controlled trials0.5% (747)
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 160,507 PubMed records
TypeRecordsShare
Reviews16,56610.3%
Meta-analyses4500.3%
Randomized controlled trials7470.5%

Method: PubMed query "Sarcoma"[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
New biological insights into osteosarcoma-lessons from single cell sequencing studies
Zehenter JI, Kager L, Eder SK, Taschner-Mandl S, Hinić S
2026Review article
Design not recorded in structured metadata
2cc by
PMID 42091739 · DOI · PMC13149624
Detection of Gene Fusions in Soft Tissue Sarcoma Using Next-Generation Sequencing
Remiszewski P, Bobak K, Piątkowski J, Golik P, Tysarowski A, Seliga K, et al.
2026Research article
Design not recorded in structured metadata
2cc by
PMID 42194972 · DOI · PMC13206013
Immunotherapy in Soft Tissue Sarcomas-An Ongoing Quest
Cobeña S, Esperança-Martins M, Syder Queiroz A, Melo-Alvim C, Costa L
2026Review article
Design not recorded in structured metadata
1cc by
PMID 42505406 · DOI · PMC13406894

New biological insights into osteosarcoma-lessons from single cell sequencing studies [1]

Review article Zehenter JI, Kager L, Eder SK, Taschner-Mandl S, Hinić S · · 2026 · cited 2 times (Europe PMC)

Abstract reading summary — source excerpts
Opening excerpt
Osteosarcoma (OS), the most frequent primary malignant bone tumor in children and adolescents, is characterized by substantial inter- and intra-tumoral heterogeneity and an immunosuppressive tumor microenvironment (TME), constraining the efficacy of both standard chemotherapy and emerging immunotherapies.
Closing excerpt
This review summarizes the current landscape of single-cell transcriptomics in OS and highlights methodological challenges in single-cell studies in OS tumors, recent biological insights, and their implication for immunotherapies.
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 (247 words)

Osteosarcoma (OS), the most frequent primary malignant bone tumor in children and adolescents, is characterized by substantial inter- and intra-tumoral heterogeneity and an immunosuppressive tumor microenvironment (TME), constraining the efficacy of both standard chemotherapy and emerging immunotherapies. Recent advances in single-cell and single-nucleus RNA sequencing (scRNA-seq and snRNA-seq) have enabled high-resolution profiling of OS tumors, revealing diverse malignant, immune, and stromal cell populations. These studies have identified proliferative, inflammatory, and angiogenic tumor states, immunosuppressive myeloid subsets, exhausted T cells, and complex cell-cell communication networks that contribute to tumor progression and immune evasion. However, several challenges constrain the broader application of single-cell approaches in OS. The mineralized structure of bone tissue complicates dissociation into viable single cells, and the rarity of OS limits access to fresh specimens. Most existing datasets are based on small, heterogeneous cohorts and are generated using diverse protocols, which complicate data integration and comparison. Emerging methods are beginning to overcome these barriers. These include snRNA-seq for frozen and archival tissue, improved dissociation protocols for mineralized tumors, and integration with spatial transcriptomics to retain spatial context. Moving forward, combining single-cell transcriptomics with complementary modalities, such as immune repertoire analysis, chromatin accessibility profiling, and spatial proteo-genomics, combined with functional validation, will provide deeper insights into immune dynamics, regulatory mechanisms, and the cellular architecture of the OS TME. This review summarizes the current landscape of single-cell transcriptomics in OS and highlights methodological challenges in single-cell studies in OS tumors, recent biological insights, and their implication for immunotherapies.

Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 42091739 · DOI · PMC13149624

Detection of Gene Fusions in Soft Tissue Sarcoma Using Next-Generation Sequencing [2]

Research article Remiszewski P, Bobak K, Piątkowski J, Golik P, Tysarowski A, Seliga K, et al. · · 2026 · cited 2 times (Europe PMC)

Abstract reading summary — source excerpts
Opening excerpt
IntroductionSoft tissue sarcomas (STS) exhibit profound molecular heterogeneity.
Closing excerpt
In the present series, however, the detected rearrangements did not alter systemic treatment, and the data do not support claims of prognostic, predictive, or therapeutic actionability.
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 (291 words)

IntroductionSoft tissue sarcomas (STS) exhibit profound molecular heterogeneity. While recurrent gene fusions hold significant diagnostic and therapeutic value-guiding treatment selection and identifying novel molecular targets-our understanding of their broader clinical implications remains limited.Materials and methodsWe performed next-generation sequencing (NGS; FusionPlex Sarcoma v2, Archer™) and bioinformatic analysis (STAR v.2.7, Arriba) on formalin-fixed paraffin-embedded (FFPE) core needle biopsy specimens. The cohort consisted of patients enrolled in a phase II clinical trial (NCT03651375) who received preoperative chemoradiotherapy according to the UNRESARC protocol.ResultsThe analysed cohort comprised nine adult patients (median age 66 years; range 44-73) diagnosed with undifferentiated pleomorphic sarcoma (UPS; n = 3), malignant peripheral nerve sheath tumour (MPNST; n = 3), myxofibrosarcoma (MFS; n = 2), and leiomyosarcoma (LMS; n = 1), predominantly high-grade (G3; 5/9) and extremity-localised (6/9). Gene fusions were detected in one-third of patients (3/9), exclusively in G3 tumours. Specifically, we identified an SGSH-PRKCA fusion in MFS (thigh), a LINC01133-OGA fusion in MPNST (thorax), and a concurrent JAZF1-MYH7B (chr7:27995037 intronic-chr20:33563203 exon/splice-site, out-of-frame but preserving myosin domains) with a PRKCA-associated intergenic rearrangement (chr1, retaining C1/kinase domains) in UPS (upper back). Notably, the SGSH-PRKCA and JAZF1-MYH7B pairs have not been previously described in the literature for these STS subtypes. Fusion-positive (F1) cases showed stable radiological disease (RECIST 1.1 SD) and EORTC C/D pathological responses with 5-20% residual viable tumour, whereas fusion-negative (F0) cases showed a wider range of radiological and pathological outcomes, including partial response, progression, and stable disease.ConclusionsOur analysis suggests that broad genomic profiling may provide complementary molecular information in diagnostically challenging cases managed at specialised sarcoma centres, particularly when morphology and immunohistochemistry are insufficient. In the present series, however, the detected rearrangements did not alter systemic treatment, and the data do not support claims of prognostic, predictive, or therapeutic actionability.

Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 42194972 · DOI · PMC13206013

Immunotherapy in Soft Tissue Sarcomas-An Ongoing Quest [3]

Review article Cobeña S, Esperança-Martins M, Syder Queiroz A, Melo-Alvim C, Costa L · · 2026 · cited 1 times (Europe PMC)

Abstract reading summary — source excerpts
Opening excerpt
Soft tissue sarcomas (STSs) are rare and heterogeneous mesenchymal malignancies characterized by diverse molecular profiles and immune landscapes.
Closing excerpt
Collectively, the available data support a paradigm shift from a sarcoma cell-centered approach toward a multi-compartment TME-including strategy, providing a framework for the development of more effective and personalized immunotherapeutic strategies in STS.
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 (192 words)

Soft tissue sarcomas (STSs) are rare and heterogeneous mesenchymal malignancies characterized by diverse molecular profiles and immune landscapes. Although immunotherapy has revolutionized the treatment of specific solid tumors, its efficacy in STSs remains limited and variable across histotypes. This review explores the panorama of biomarkers of immunotherapy sensitiveness in STSs, with particular emphasis on tumor-intrinsic features and on tumor microenvironment (TME) signatures. Current evidence highlights low tumor mutational burden, rare microsatellite instability, heterogeneous antigen expression, and epigenetic suppression of antigen presentation as hallmarks of the immune resistance that is characteristic of many STSs. However, growing evidence underlines TME composition as a major determinant of response to different types of immunotherapy. Indeed, the presence of B-cell-rich tertiary lymphoid structures and certain traits of adaptive immune responses are provenly associated with enhanced sensitivity to immunotherapy and enhanced outcomes. We further discuss emerging strategies aimed at enhancing STS immunogenicity, either by increasing intrinsic tumor immunogenicity or remodeling TME composition and functional profile. Collectively, the available data support a paradigm shift from a sarcoma cell-centered approach toward a multi-compartment TME-including strategy, providing a framework for the development of more effective and personalized immunotherapeutic strategies in STS.

Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 42505406 · DOI · PMC13406894

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 Progression-free survival in gastrointestinal stromal tumours with high-dose imatinib: randomised trial [4]
    Verweij J, Casali PG, Zalcberg J, LeCesne A, Reichardt P, Blay JY, et al. · Lancet · 2004 · cited 1,170 times (Europe PMC)
  • Research article A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma [5]
    Pervaiz N, Colterjohn N, Farrokhyar F, Tozer R, Figueredo A, Ghert M · Cancer · 2008 · cited 660 times (Europe PMC)
  • Research article Fusion gene-negative alveolar rhabdomyosarcoma is clinically and molecularly indistinguishable from embryonal rhabdomyosarcoma [6]
    Williamson D, Missiaglia E, de Reyniès A, Pierron G, Thuille B, Palenzuela G, et al. · J Clin Oncol · 2010 · cited 378 times (Europe PMC)
  • Research article High-dose methotrexate-induced nephrotoxicity in patients with osteosarcoma [7]
    Widemann BC, Balis FM, Kempf-Bielack B, Bielack S, Pratt CB, Ferrari S, et al. · Cancer · 2004 · cited 208 times (Europe PMC)
  • Research article Prognostic and predictive factors for outcome to first-line ifosfamide-containing chemotherapy for adult patients with advanced soft tissue sarcomas: an exploratory, retrospective analysis on large series from the European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group (EORTC-STBSG) [8]
    Sleijfer S, Ouali M, van Glabbeke M, Krarup-Hansen A, Rodenhuis S, Le Cesne A, et al. · Eur J Cancer · 2010 · cited 199 times (Europe PMC)
  • Research article Osteosarcoma: A Meta-Analysis and Review of the Literature [9]
    Friebele JC, Peck J, Pan X, Abdel-Rasoul M, Mayerson JL · Am J Orthop (Belle Mead NJ) · 2015 · cited 145 times (Europe PMC)

References

  1. Zehenter JI, Kager L, Eder SK, Taschner-Mandl S, Hinić S. New biological insights into osteosarcoma-lessons from single cell sequencing studies. . 2026. PMID 42091739 · DOI · PMC13149624
  2. Remiszewski P, Bobak K, Piątkowski J, Golik P, Tysarowski A, Seliga K, et al.. Detection of Gene Fusions in Soft Tissue Sarcoma Using Next-Generation Sequencing. . 2026. PMID 42194972 · DOI · PMC13206013
  3. Cobeña S, Esperança-Martins M, Syder Queiroz A, Melo-Alvim C, Costa L. Immunotherapy in Soft Tissue Sarcomas-An Ongoing Quest. . 2026. PMID 42505406 · DOI · PMC13406894
  4. Verweij J, Casali PG, Zalcberg J, LeCesne A, Reichardt P, Blay JY, et al.. Progression-free survival in gastrointestinal stromal tumours with high-dose imatinib: randomised trial. Lancet. 2004. PMID 15451219 · DOI
  5. Pervaiz N, Colterjohn N, Farrokhyar F, Tozer R, Figueredo A, Ghert M. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer. 2008. PMID 18521899 · DOI
  6. Williamson D, Missiaglia E, de Reyniès A, Pierron G, Thuille B, Palenzuela G, et al.. Fusion gene-negative alveolar rhabdomyosarcoma is clinically and molecularly indistinguishable from embryonal rhabdomyosarcoma. J Clin Oncol. 2010. PMID 20351326 · DOI
  7. Widemann BC, Balis FM, Kempf-Bielack B, Bielack S, Pratt CB, Ferrari S, et al.. High-dose methotrexate-induced nephrotoxicity in patients with osteosarcoma. Cancer. 2004. PMID 15139068 · DOI
  8. Sleijfer S, Ouali M, van Glabbeke M, Krarup-Hansen A, Rodenhuis S, Le Cesne A, et al.. Prognostic and predictive factors for outcome to first-line ifosfamide-containing chemotherapy for adult patients with advanced soft tissue sarcomas: an exploratory, retrospective analysis on large series from the European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group (EORTC-STBSG). Eur J Cancer. 2010. PMID 19853437 · DOI
  9. Friebele JC, Peck J, Pan X, Abdel-Rasoul M, Mayerson JL. Osteosarcoma: A Meta-Analysis and Review of the Literature. Am J Orthop (Belle Mead NJ). 2015. PMID 26665241

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

160,507
PubMed records on this heading
14%
published since 2020
16,566
review articles
747
randomized trials
9
verified references below

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

Related topics See all →

  • OsteosarcomaCancer and Oncology
  • Giant Cell Tumor of BoneCancer and Oncology
  • Giant Cell TumorsCancer and Oncology
  • LeiomyosarcomaCancer and Oncology
  • Granular Cell TumorCancer and Oncology

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