Rare Gastroenterology News

Spotlight On

Carcinoid tumor

A carcinoid is a slow-growing type of neuroendocrine tumor originating in the cells of the neuroendocrine system




US Estimated


Europe Estimated

Age of Onset





Autosomal dominant


Autosomal recessive




X-linked dominant


X-linked recessive


Rare View

Carcinoid syndrome is a paraneoplastic syndrome comprising the signs and symptoms that occur secondary to carcinoid tumors. The syndrome includes flushing and diarrhea, and less frequently, heart failure, vomiting and bronchoconstriction.The overall incidence rates for carcinoid tumors have increased significantly over the past 25 years.


5 Facts you should know



Carcinoid tumors are of neuroendocrine origin and derived from primitive stem cells in the gut wall, especially the appendix



The average age of people diagnosed with digestive or lung carcinoids is about 60



In the intestinal tract, these tumors develop deep in the mucosa, growing slowly and extending into the underlying submucosa and mucosal surface



The tumor may produce hormone-like substances that spread to the body and cause symptoms of carcinoid syndrome, such as flushing of the face and chest, diarrhea



The primary treatment is surgical removal

Carcinoid tumor is also known as...

Carcinoid tumor

Carcinoid apudoma; Carcinoid cancer; Carcinoid disease; Functioning argentaffinoma; Functioning carcinoid; Malignant carcinoid syndrome; Neuroendocrine tumor carcinoid type

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What percentage of people with a carcinoid tumor go on to develop carcinoid syndrome?

Current treatments


(Brand name: Afinitor) Manufactured by Novartis Pharmaceuticals Corporation
FDA-approved indication: Treatment of adult patients with progressive, well differentiated, nonfunctional,neuroendocrine tumors(NET) of gastrointestinal (GI) or lung origin, (excluding pancreatic) with unresectable, locally advanced or metastatic disease.

Lutetium Lu 177 dotatate

(Brand name: Lutathera) Manufactured by Advanced Accelerator Applications USA Inc
FDA-approved indication: Treatment of somatostatin receptor-positive gastroenteropancreatic neuroendocrine tumors (GEP-NETs), including foregut, midgut, and hindgut tumors in adults.

Gallium [Ga-68]

(Brand name: Netspot) Manufactured by Advanced Accelerator Applications, USA
FDA-approved indication: For use after radiolabeling with Ga 68, with positron emission tomography (PET) for localization of somatostatin receptor positive neuroendocrine tumors (NETs) in adult and pediatric patients.


(Brand name: Sandostatin LAR) Manufactured by Novartis Pharmaceuticals Corporation
FDA-approved indication: Reduction of growth hormone and IGF-1 (somatomedin C) in acromegaly.


(Brand name: Somatuline Depot (injection)) Manufactured by Ipsen, Inc
FDA-approved indication: Treatment of patients with unresectable,wellor moderately-differentiated locally advanced or metastatic gastroenteropancreatic neuroendocrine tumors to improve progression-free survival.

Top Clinical Trials

TitleDescriptionPhasesStatusInterventionsMore Information
Cabozantinib and Nivolumab for Carcinoid TumorsThis research study, is studying the combination of cabozantinib and nivolumab in treating advanced carcinoid tumors.Phase 2RecruitingDrug: Nivolumab|Drug: CabozantinibLearn more
EMB-01 in Patients With Advanced/Metastatic Gastrointestinal CancersThis study is to evaluate the safety and antitumor activity of EMB-01 in advanced/metastatic gastrointestinal cancers, including gastric cancer, hepatocellular cancer, cholangiocarcinoma and colorectal cancer.Phase 1|Phase 2RecruitingDrug: EMB-01Learn more
Abemaciclib in Treating Patients With Advanced, Refractory, and Unresectable Digestive System Neuroendocrine TumorsThis phase II trial studies how well abemaciclib works in treating patients with digestive system neuroendocrine tumors that have spread to other places in the body, do not respond to treatment, and cannot be removed by surgery. Abemaciclib may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth.Phase 2RecruitingDrug: AbemaciclibLearn more
Study of Pembrolizumab (MK-3475) in Participants With Advanced Solid Tumors (MK-3475-158/KEYNOTE-158)In this study, participants with multiple types of advanced (unresectable and/or metastatic) solid tumors who have progressed on standard of care therapy will be treated with pembrolizumab (MK-3475).Phase 2RecruitingBiological: pembrolizumabLearn more

Top Treatments in Research

AgentClass/Mechanism of ActionDevelopment StatusCompanyClinical StudiesMore Information
NivolumabNivolumab is a human immunoglobulin G4 (IgG4) monoclonal antibody that binds to the PD-1 receptor and blocks its interaction with PD-L1 and PD-L2, releasing PD-1 pathway-mediated inhibition of the immune response, including the anti-tumor immune response, resulting in decreased tumor growth.Phase 2Bristol-Myers Squibb|ExelixisLearn moreMore information
EMB-01Bispecific antibody targeting EGFR and cMet on tumor cells mediates a novel mechanism to improve anti-tumor efficacy.Phase 1|Phase 2Shanghai EpimAb Biotherapeutics Co., Ltd.Learn moreMore information
AbemaciclibAbemaciclib selectively inhibits CDK4 and CDK6 with low nanomolar potency, inhibits Rb phosphorylation resulting in a G1 arrest and inhibition of proliferation, and its activity is specific for Rb-proficient cells. Unlike other CDK inhibitors such as Palbociclib and Ribociclib, abemaciclib exhibits greater selectivity for CDK4 compared to CDK6.Phase 2Eli Lilly and CompanyLearn moreMore information
PembrolizumabPembrolizumab is a highly selective humanized monoclonal IgG4 antibody directed against the PD-1 receptor on the cell surface. The drug blocks the PD-1 receptor, preventing binding and activation of PD-L1 and PD-L2. This mechanism causes the activation of T-cell mediated immune responses against tumor cells.Phase 2Merck Sharp & Dohme Corp.Learn moreMore information