An observational study in Carcinoid, sponsored by National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). Recruiting at 1 site in United States. Open to participants aged 18 Years to 100 Years. Per ClinicalTrials.gov, last updated 2026-10-02.
Sponsored by National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) · Observational
This study will evaluate members in families with a history of small bowel carcinoid cancer to study the natural history of those family members that have the disease, determine ways to improve early detection by performing surveillance on those at risk but without disease and to identify the gene(s) that may cause the tumors. Familial carcinoid tumors usually originate in hormone-producing cells that line the small intestine or other cells of the digestive tract. The tumors are slow-growing and usually take many years before they cause symptoms. It is known that these tumors occur more often in some families and are then passed from one generation to the next by inherited genes.
Members of families, including all siblings and offspring in which two or more immediate blood relatives have had small bowel carcinoid tumors are eligible for this study. In some cases unaffected spouses of family members diagnosed with carcinoid cancer are also requested to participate by donating a sample of blood only.
Participants undergo a medical evaluation every 3 years during a 3- to 5-day hospital stay at the NIH Clinical Center. All participants have a personal and family medical history obtained and undergo a physical examination, blood and urine tests.
People who already have a small bowel carcinoid tumor or are at risk of developing a carcinoid tumor have some or all of the following procedures to determine the presence of carcinoid tumor and its (omit next two words- location or) spread to other areas of the body:
Should mid gut carcinoid tumors be found every participant will be assisted in determine what the best course of treatment will be for them.
Study Description:
This study is designed as a prospective evaluation for diagnostic screening, genotyping and natural history of participants belonging to kindreds with familial carcinoid tumor.
Objectives:
Primary Objective:
Study the natural history of familial carcinoid tumors: incidence, age of onset, symptoms, the appropriate diagnostic (biochemical and imaging) modalities, location, histology and metastatic potential of the tumors, metabolic sequelae of the tumor, and clinical and biochemical prognostic factors.
Secondary Objectives:
152 studies on the registry are indexed under Carcinoid Tumor; 16 are open to participants now.
This study's planned enrollment of 1,600 is above the median of 370 across 24 observational studies indexed under Carcinoid Tumor.
Browse Carcinoid Tumor studies →National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) is the lead sponsor of 529 studies on the registry; 54 are open to participants now.
Of its 79 completed or terminated interventional studies of FDA-regulated products, 50 (63%) have results posted.
Counted across the registry records on this site, refreshed daily.
Community sample
There are four types of participants who will be included in this protocol as outlined below.
In order to be eligible to participate in this study, an individual must meet all of the following criteria for their group:
Group 1 (Arm 1 or Arm 2)
Group 2 (Arm 1 or Arm 2)
Group 3 (Arm 1 or Arm 2)
Has one of the following:
Group 4 (Arm 2 only)
EXCLUSION CRITERIA:
An individual who meets any of the following criteria will be excluded from participation in this
study:
Members of families with multiple endocrine neoplasia (MEN) I, MEN II or other familial tumor syndromes such as Von Hippel Lindau Syndrome and Neurofibromatosis type I and type II for which there is a known genetic predisposition to non-carcinoid tumors as well as
carcinoid tumors will be excluded from the study.
Participants who undergo extended evaluation for disease at NIH
Drug: [18F]-DOPA
Participants who do not undergo extended screening or evaluation for disease at NIH
Drug: [18F]-DOPA
18F-DOPA PET/CT Scan.
Study the natural history of familial carcinoid tumors
Incidence, age of onset, symptoms, the appropriate diagnostic (biochemical and imaging) modalities, location, histology and metastatic potential of the tumors, metabolic sequelae of the tumor, and clinical and biochemical prognostic factors
Time frame: End of study
Compare the sensitivity and specificity of various imaging modalities: computed tomography (CT) with IV contrast and oral Volumen, 18F-DOPA PET/CT scan, [68Ga]DOTATATE PET/CT scan and endoscopic modalities for diagnosing and following carcinoid ...
Enrollment of a sufficient number of subjects to allow for the determination of sensitivity and specificity for each imaging and endoscopic modality to show statistically significant superiority for one modality relative to the others
Time frame: End of study
Sequester DNA from peripheral blood for genotyping (including sequencing) with the intention of localizing a susceptibility gene/s responsible for the familial occurrence of the disease
Determination of the gene mutation responsible for the disease in each family
Time frame: End of study
Collect tumor specimens for histologic evaluation, culturing of intestinal organoids, and genotyping (including DNA and RNA sequencing)
Sufficient number of tumors collected to correlate disease grade with natural history of disease and assess the variability in disease grade
Time frame: End of study
Screen for occult disease and determine whether early detection affects the natural history of the disease
Change in survival
Time frame: End of study
Plan to share: No
From the registry record's own update history. This site started tracking changes on Sep 25, 2026; for anything earlier, see the record history on ClinicalTrials.gov ↗
Eligibility is decided by the study team. Share this record with your doctor or contact the team directly.
Contact study teamGet an email when the registry record changes — status, dates, results — or when someone posts here.
Sign in to followQuestions and observations about this study, from anyone following it. Not medical advice, and not a channel to the study team — their contact details are on the registry record.
Sign in to join the discussion. Reading takes no account; posting does. You choose a display name, and a pseudonym is the default.
Nothing here yet. If you are running this trial, taking part in it, or weighing whether to, this is the place to say so.
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)