Disease Directory Carcinoid Tumor
Oncology

Carcinoid Tumor

Also known as: well-differentiated neuroendocrine tumor, NET, carcinoid NET, serotonin-secreting tumor

Prevalence

2–5 in 100,000 (rising with improved detection)

Onset

Adult (median age 50s–60s); varies by primary site

Type

Sporadic; rarely MEN1-associated

About Carcinoid Tumor

Carcinoid tumours are well-differentiated (grade 1–2) neuroendocrine neoplasms most commonly arising in the small intestine, appendix, rectum, and bronchus, characterised by expression of neuroendocrine markers (synaptophysin, chromogranin A) and, in functional tumours, excessive production of serotonin and other vasoactive peptides. The carcinoid syndrome — episodic flushing, diarrhoea, and bronchospasm — occurs when vasoactive mediators bypass hepatic metabolism, typically indicating hepatic metastases or a primary bronchial carcinoid with direct systemic venous drainage. Long-acting somatostatin analogues (octreotide LAR, lanreotide) are the cornerstone of symptom control and have demonstrated antiproliferative activity in midgut NETs.

Common Clinical Features

Carcinoid syndrome: episodic cutaneous flushing (often triggered by alcohol, stress, or food), profuse watery diarrhoea, and abdominal cramping Bronchospasm and wheezing from bronchoconstrictor mediator release Carcinoid heart disease: right-sided endocardial fibrosis causing tricuspid and pulmonary valve abnormalities from chronic serotonin exposure Abdominal pain from primary intestinal tumour, mesenteric fibrosis, or intestinal obstruction Pellagra-like dermatitis from tryptophan diversion to serotonin synthesis causing niacin deficiency Weight loss and malnutrition from diarrhoea and malabsorption Incidental finding of a pulmonary or intestinal mass on imaging

Clinical Trial Eligibility Tips

What to know before applying to Carcinoid Tumor trials.

Biochemical documentation with plasma chromogranin A and 24-hour urinary 5-HIAA (or plasma 5-HIAA) is required for most functional NET trials; ensure values are obtained while off proton pump inhibitors (which falsely elevate chromogranin A).

Somatostatin receptor expression confirmed by 68Ga-DOTATATE PET-CT is required for PRRT (peptide receptor radionuclide therapy) trials and for somatostatin analogue-related protocols — recent functional imaging within the protocol-specified window is essential.

Ki-67 proliferation index and WHO grade (G1: Ki-67 <3%, G2: 3–20%, G3: >20%) must be documented from the most recent biopsy as they determine eligibility for graded therapy protocols and may change over time with tumour progression.

Patient Resources

Patient Organization

Carcinoid Cancer Foundation

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Natural History Registry

NANETS Registry

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Orphanet

European reference resource for rare diseases (ORPHA:100091)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

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Find recruiting Carcinoid Tumor trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Carcinoid Tumor. Updated daily.

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