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Last reviewed: 18 Mar 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

Other diagnostic factors

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

Risk factors

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

Diagnostic investigations

1st investigations to order

  • CT scan of primary tumour
  • MRI of primary tumour
  • CT scan chest
  • biopsy for histology

Investigations to consider

  • abdominal/pelvic computed tomography
  • magnetic Resonance Imaging (MRI) of total spine
  • fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT)
  • endoscopy
  • full blood count (FBC)
  • coagulation studies
  • urea
  • creatinine
  • liver function tests (LFTs)
  • echocardiogram or multi-gated acquisition (MUGA) scan
  • ancillary techniques

Treatment algorithm

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.

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