AACE/ACE disease state clinical review: diagnosis and management of midgut carcinoids

Endocr Pract. 2015 May;21(5):534-545. doi: 10.4158/EP14464.DSC.


Objective: Neuroendocrine tumors (NETs) are a collection of complex tumors that arise from the diffuse endocrine system, primarily from the digestive tract. Carcinoid tumors most commonly originate from the small intestine. These tumors are either referred to as small intestinal neuroendocrine tumors or midgut carcinoids (MGCs). The purpose of this review article is to survey the diagnostic and therapeutic pathways for patients with MGC and provide an overview of the complex multidisciplinary care involved in improving their quality of life, treatment outcomes, and survival.

Methods: The current literature regarding the diagnosis and management of MGCs was reviewed.

Results: Dry flushing and secretory diarrhea are the hallmarks of the clinical syndrome of MGC. Managing MGC requires attention to the overall symptom complex, including the physical effects of the tumor and biomarker levels. The somatostatin analogs (SAs) octreotide and lanreotide are highly efficacious for symptomatic improvement. MGCs require resection to encompass the primary tumor and mesenteric lymph node metastases and should include cholecystectomy if the patient is likely to receive SA therapy. Debulking of liver metastasis by resection in combination with ablative therapies and other liver-directed modalities may help palliate symptoms and hormonal overproduction in carefully selected patients. Quality of life is an important measure of patients' perception of the burden of their disease and impact of treatment modalities and may be a useful guide in deciding changes in therapy to alter apparent health status.

Conclusion: MGC is a challenging malignancy that requires the input of a multidisciplinary team to develop the best treatment plan. Consultation with expert centers that specialize in NETs may also be indicated for complex cases. With expert care, patients can be cured or live with the disease and enjoy good quality of life.

Publication types

  • Review

MeSH terms

  • Abdominal Pain
  • Biomarkers, Tumor / blood
  • Biomarkers, Tumor / urine
  • Carcinoid Heart Disease
  • Carcinoid Tumor / diagnosis*
  • Carcinoid Tumor / surgery
  • Carcinoid Tumor / therapy*
  • Cholecystectomy
  • Diagnostic Imaging
  • Diarrhea
  • Flushing
  • Humans
  • Interdisciplinary Communication
  • Intestinal Neoplasms / diagnosis*
  • Intestinal Neoplasms / surgery
  • Intestinal Neoplasms / therapy*
  • Intestine, Small*
  • Liver Neoplasms / secondary
  • Liver Neoplasms / surgery
  • Liver Neoplasms / therapy
  • Lymph Node Excision
  • Lymphatic Metastasis
  • Mesentery
  • Octreotide / therapeutic use
  • Peptides, Cyclic / therapeutic use
  • Quality of Life
  • Somatostatin / analogs & derivatives
  • Somatostatin / therapeutic use


  • Biomarkers, Tumor
  • Peptides, Cyclic
  • lanreotide
  • Somatostatin
  • Octreotide

Supplementary concepts

  • Carcinoid Tumors, Intestinal