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
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 tumour 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 tumour bulk amenable to local therapy
retroperitoneal/intra-abdominal: disseminated metastases
gastrointestinal stromal tumours: resectable with minimal morbidity
gastrointestinal stromal tumours: resectable with significant morbidity
gastrointestinal stromal tumours: unresectable
gastrointestinal stromal tumours: progressive
gastrointestinal stromal tumours: 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.
Differentials
- Lipoma
- Gastrointestinal ulcer
- Lymphoma
More DifferentialsGuidelines
- 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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