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.
References
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 here.
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