Summary
Definition
History and exam
Key diagnostic factors
- profuse watery diarrhea
Other diagnostic factors
- age around 50 years
- headache
- weight loss
- poor skin turgor
- dry mucus membranes
- muscle weakness
- muscle cramps
- gastrointestinal symptoms
- hyperglycemia symptoms
- flushing
- hepatomegaly
- history of multiple endocrine neoplasia type 1 (MEN1)
Risk factors
- multiple endocrine neoplasia type 1 (MEN1)
- age around 50 years
Diagnostic tests
1st tests to order
- serum potassium
- serum bicarbonate
- serum calcium
- serum glucose
- vasoactive intestinal peptide (VIP) radioimmunoassay
- liver function tests
Tests to consider
- arterial blood gas analysis
- gastric pH monitoring
- chromogranin A
- pancreatic polypeptide
- contrast-enhanced CT scan of abdomen
- contrast-enhanced MRI of abdomen
- somatostatin receptor PET-CT/MRI
- somatostatin receptor scintigraphy
- endoscopic ultrasound
- operative exploration
- intraoperative ultrasound
- biopsy
Treatment algorithm
initial presentation of symptomatic patient
localized disease
metastatic disease: surgical candidate
metastatic disease: nonsurgical candidate
refractory disease
Contributors
Authors
Harish Lavu, MD, FACS
Professor of Surgery
Sidney Kimmel Medical College
Thomas Jefferson University
Philadelphia
PA
Disclosures
HL declares that he has no competing interests.
Charles J. Yeo, MD, FACS
The Samuel D. Gross Professor and Chair
Department of Surgery
Sidney Kimmel Medical College
Thomas Jefferson University
Philadelphia
PA
Disclosures
CJY declares that he has no competing interests.
Peer reviewers
Alexandria Phan, MD
Professor of Medicine
Froedtert Hospital and the Medical College of Wisconsin
Milwaukee
WI
Declarações
AP declares that she has no competing interests.
Jonathan Strosberg, MD
Professor
Moffitt Cancer Center
Tampa
FL
Declarações
JS has consulted for Novartis and serves on the Speakers’ Bureau for Ipsen.
Jaime Ruiz-Tovar, MD
Fellow of the Spanish Association of Surgeons (AEC)
Department of Surgery
University Hospital Ramon y Cajal
Madrid
Spain
Declarações
JRT declares that he has no competing interests.
Créditos aos pareceristas
Os tópicos do BMJ Best Practice são constantemente atualizados, seguindo os desenvolvimentos das evidências e das diretrizes. Os pareceristas aqui listados revisaram o conteúdo pelo menos uma vez durante a história do tópico.
Declarações
As afiliações e declarações dos pareceristas referem--se ao momento da revisão.
Referências
Principais artigos
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].Texto completo
Hofland J, Falconi M, Christ E, et al. European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes. J Neuroendocrinol. 2023 Aug;35(8):e13318.Texto completo Resumo
Pavel M, Öberg K, Falconi M, et al. Gastroenteropancreatic neuroendocrine neoplasms: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2020 Jul;31(7):844-60.Texto completo
Del Rivero J, Perez K, Kennedy EB, et al. Systemic therapy for tumor control in metastatic well-differentiated gastroenteropancreatic neuroendocrine tumors: ASCO guideline. J Clin Oncol. 2023 Nov 10;41(32):5049-67.Texto completo Resumo
Artigos de referência
Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.
Diagnósticos diferenciais
- Infectious gastroenteritis
- Osmotic diarrhea
- Ileal bile acid malabsorption
Mais Diagnósticos diferenciaisDiretrizes
- NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors
- Symptom management for well-differentiated gastroenteropancreatic neuroendocrine tumors: ASCO guideline
Mais DiretrizesFolhetos informativos para os pacientes
Diarrhea in children
Mais Folhetos informativos para os pacientes
Conectar-se ou assinar para acessar todo o BMJ Best Practice
O uso deste conteúdo está sujeito ao nosso aviso legal