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
- lymphedema
- history of exposure to chemical carcinogens
Diagnostic tests
1st tests to order
- CT scan of primary tumor
- MRI of primary tumor
- CT scan chest
- biopsy for histology
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
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 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.
Differentials
- Lipoma
- Gastrointestinal ulcer
- Lymphoma
More DifferentialsGuidelines
- Management of soft tissue sarcoma
- Management of gastrointestinal stromal tumors
More Guidelines
Log in or subscribe to access all of BMJ Best Practice
Use of this content is subject to our disclaimer