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Soft-tissue sarcoma

Last reviewed: 3 Nov 2023
Last updated: 22 Aug 2023



History and exam

Key diagnostic factors

  • mass
  • upper/lower gastrointestinal bleed
  • rash
  • purplish macular-papular lesions
  • abnormal uterine bleeding
  • increased abdominal girth
  • history of HIV infection
  • features of acute abdomen
  • neuropathic pain
More key diagnostic factors

Other diagnostic factors

  • weight loss
  • fatigue
  • anorexia
  • abdominal bloating, discomfort, pain
  • unilateral extremity swelling
Other diagnostic factors

Risk factors

  • genetically inherited syndromes
  • radiation
  • human herpesvirus-8 (HHV-8) infection
  • congenital disorders
  • lymphedema
  • history of exposure to chemical carcinogens
More risk factors

Diagnostic investigations

1st investigations to order

  • CT scan of primary tumor
  • MRI of primary tumor
  • CT scan chest
  • biopsy for histology
More 1st investigations to order

Investigations to consider

  • HIV test
  • ultrasound of primary tumor
  • chest x-ray
  • positron emission tomography (PET) scan
  • endoscopy
  • complete blood count (CBC)
  • blood urea nitrogen (BUN)
  • creatinine
  • liver function tests (LFTs)
  • echocardiogram or multigated acquisition (MUGA) scan
  • gene rearrangement testing
More investigations to consider

Treatment algorithm


extremity: stage I

extremity: stage II-III, resectable

extremity: stage II-III, unresectable

extremity: stage IV, single organ + limited tumor bulk or regional nodes

extremity: stage IV, disseminated metastases

retroperitoneal/intra-abdominal: resectable

retroperitoneal/intra-abdominal: unresectable or stage IV

gastrointestinal stromal tumors: resectable with minimal morbidity

gastrointestinal stromal tumors: resectable with significant morbidity

gastrointestinal stromal tumors: unresectable, progressive, or metastatic with imatinib-sensitive mutation

gastrointestinal stromal tumors: unresectable, progressive, or metastatic with imatinib-insensitive mutation





James S. Hu, MD, FACP

Associate Professor of Clinical Medicine

USC Norris Comprehensive Cancer Center

Los Angeles



JSH declares that he has no competing interests.

Sant P. Chawla, MD, FRACP


Sarcoma Oncology Center

Santa Monica



SPC declares that he has no competing interests.


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.


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

Peer reviewers

Alfio Ferlito, MD, DLO, DPath, FRCSEd


Department of Surgical Sciences

Professor and Chairman

ENT Clinic

University of Udine




AF declares that he has no competing interests.

Adrienne Flanagan, MB BCh FRCPath PhD

Professor of Musculoskeletal Pathology

Institute of Orthopaedics and Musculoskeletal Science

University College London




AF declares that she has no competing interests.

Robert Maki, MB, PhD

Medical Oncologist

Memorial Sloan-Kettering Cancer Center

New York



RM declares that he has no competing interests.

  • Differentials

    • Lipoma
    • Gastrointestinal ulcer
    • Lymphoma
    More Differentials
  • Guidelines

    • NCCN clinical practice guidelines in oncology: gastrointestinal stromal tumors (GIST)
    • NCCN clinical practice guidelines in oncology: soft tissue sarcoma
    More Guidelines
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