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
Pruebas diagnósticas
Primeras pruebas diagnósticas para solicitar
- CT scan of primary tumor
- MRI of primary tumor
- CT scan chest
- biopsy for histology
Pruebas diagnósticas que deben considerarse
- abdominal/pelvic computed tomography
- MRI of total spine
- fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT)
- MRI of the central nervous system (CNS)
- endoscopy
- complete blood count (CBC)
- coagulation studies
- blood urea nitrogen (BUN)
- creatinine
- liver function tests (LFTs)
- echocardiogram or multigated acquisition (MUGA) scan
- ancillary techniques
Algoritmo de tratamiento
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 (primary or recurrent)
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
Colaboradores
Autores
James S. Hu, MD, FACP
Associate Professor of Clinical Medicine
USC Norris Comprehensive Cancer Center
Los Angeles
CA
Divulgaciones
JSH has been reimbursed for speaking on desmoid tumors and the drug Ogsiveo (nirogacestat).
Sant P. Chawla, MD, FRACP
Director
Sarcoma Oncology Center
Santa Monica
CA
Divulgaciones
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.
Agradecimientos
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.
Divulgaciones
SS, JCT and SH each declare that they have no competing interests. DT has received research support from Pfizer, Amgen, and Novartis.
Revisores por pares
Richard F. Riedel, MD
Professor of Medicine
Duke University
Durham
NC
Divulgaciones
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.
Agradecimiento de los revisores por pares
Los temas de BMJ Best Practice se actualizan de forma continua de acuerdo con los desarrollos en la evidencia y en las guías. Los revisores por pares listados aquí han revisado el contenido al menos una vez durante la historia del tema.
Divulgaciones
Las afiliaciones y divulgaciones de los revisores por pares se refieren al momento de la revisión.
Referencias
Artículos principales
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].Texto completo
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.Texto completo Resumen
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. Resumen
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: gastrointestinal stromal tumors (GIST) [internet publication].Texto completo
Artículos de referencia
Una lista completa de las fuentes a las que se hace referencia en este tema está disponible para los usuarios con acceso a todo BMJ Best Practice.
Diferenciales
- Lipoma
- Gastrointestinal ulcer
- Lymphoma
Más DiferencialesGuías de práctica clínica
- NCCN clinical practice guidelines in oncology: gastrointestinal stromal tumors (GIST)
- NCCN clinical practice guidelines in oncology: soft tissue sarcoma
Más Guías de práctica clínica
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