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Soft-tissue sarcoma

  • Overview
  • Theory
  • Diagnosis
  • Management
  • Follow up
  • Resources
Last reviewed: 1 Apr 2025
Last updated: 06 Dec 2024

Summary

Definition

History and exam

Key diagnostic factors

  • mass
  • upper/lower gastrointestinal bleed
  • neuropathic pain
  • increased abdominal girth
  • features of acute abdomen
Full details

Other diagnostic factors

  • unilateral extremity swelling
  • weight loss
  • fatigue
  • anorexia
  • abdominal bloating, discomfort, pain
Full details

Risk factors

  • genetically inherited syndromes
  • radiation
  • human herpesvirus-8 (HHV-8) infection
  • congenital disorders
  • male sex
  • lymphedema
  • history of exposure to chemical carcinogens
Full details

Diagnostic tests

1st tests to order

  • CT scan of primary tumor
  • MRI of primary tumor
  • CT scan chest
  • biopsy for histology
Full details

Tests to consider

  • abdominal/pelvic computed tomography
  • magnetic Resonance Imaging (MRI) of total spine
  • fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT)
  • endoscopy
  • complete blood count (CBC)
  • coagulation studies
  • blood urea nitrogen (BUN)
  • creatinine
  • liver function tests (LFTs)
  • echocardiogram or multigated acquisition (MUGA) scan
  • ancillary techniques
Full details

Treatment algorithm

ACUTE

extremity: stage I

extremity: stage II resectable

extremity: stage III or select stage IV (any T, N1, M0), resectable

extremity: stage III or select stage IV (any T, N1, M0), unresectable

extremity: stage IV, oligometastases with limited tumor bulk or regional nodes

extremity: stage IV, disseminated metastases

extremity: recurrent

retroperitoneal/intra-abdominal: resectable (primary or recurrent)

retroperitoneal/intra-abdominal: unresectable

retroperitoneal/intra-abdominal: metastatic disease, single organ and limited tumor bulk amenable to local therapy

retroperitoneal/intra-abdominal: disseminated metastases

gastrointestinal stromal tumors: resectable with minimal morbidity

gastrointestinal stromal tumors: resectable with significant morbidity

gastrointestinal stromal tumors: unresectable

gastrointestinal stromal tumors: progressive

gastrointestinal stromal tumors: recurrent or metastatic

Contributors

Authors

James S. Hu, MD, FACP

Associate Professor of Clinical Medicine

USC Norris Comprehensive Cancer Center

Los Angeles

CA

Disclosures

JSH declares that he has no competing interests.

Sant P. Chawla, MD, FRACP

Director

Sarcoma Oncology Center

Santa Monica

CA

Disclosures

SPC owns stock in AADi, Cellestia Biotech, CounterPoint, and Immix BioPharma, and has received grants, consulting fees, payments of honoraria, and payment from speakers bureau for Amgen, Roche, GSK, Threshold Pharmaceuticals, CytRx Corporation, Ignyta, Immune Design, TRACON Pharma, Karyopharm Therapeutics, Sarcoma Alliance for Research through Collaboration (SARC), Janssen, Advenchen Laboratories, Bayer, Inhibrx, NKMax, and Thyme.

Acknowledgements

Dr James S. Hu and Dr Sant P. Chawla would like to gratefully acknowledge Dr Swati Sikaria, Dr Jonathan C. Trent, Dr Saira Hassan, and Dr David Thomas, previous contributors to this topic.

Disclosures

SS, JCT and SH each declare that they have no competing interests. DT has received research support from Pfizer, Amgen, and Novartis.

Peer reviewers

Richard F. Riedel, MD

Professor of Medicine

Duke University

Durham

NC

Disclosures

RFR owns shares in Limbguard, has received institutional clinical research support from AADi, Adaptimmune, AROG, Ayala, BioAtla, Blueprint, Cogent, Daiichi-Sankyo, Deciphera, GlaxoSmithKline, InhibRx, NanoCarrier, Oncternal, PTC Therapeutics, SARC, SpringWorks, Tracon, and Trillium. He has been a consultant/advisor for AADi, Adaptimmune, Bayer, Blueprint, Boehringer Ingelheim, Daiichi-Sankyo, Deciphera, GlaxoSmithKline, NanoCarrier, SpringWorks and has received funding for travel from Deciphera.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

World Health Organization. WHO classification of tumours: soft tissue and bone tumours. 5th ed (Vol 3). Lyon, France: IARC Press; 2020.

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: soft tissue sarcoma [internet publication].Full text

Gronchi A, Miah AB, Dei Tos AP, et al. Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS Clinical practice guidelines for diagnosis, treatment and follow-up*. Ann Oncol. 2021 Nov;32(11):1348-65.Full text  Abstract

Casali PG, Blay JY, Abecassis N, et al. Gastrointestinal stromal tumours: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2022 Jan;33(1):20-33. Abstract

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: gastrointestinal stromal tumors (GIST) [internet publication].Full text

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

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