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Natural History of Familial Carcinoid Tumor

Study Purpose

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:

  • - Video Capsule Endoscopy: Visualization of the gastrointestinal tract by ingesting a disposable, "vitamin-pill sized" video capsule that has its own camera and light source.
  • - CT of the chest abdomen and pelvis with oral and IV contrast : X-ray examination of the chest, abdominal and pelvis organs.
  • - 18 FDOPA Positron emission tomography (PET) with CT for localization: Nuclear imaging scan to look at tumor activity.
  • - MRI Liver with contrast - to determine if disease has spread to liver.
  • - Gallium 68 PET/CT-limited to individuals that have residual tumor.
  • - Clinical and research blood work.
Should mid gut carcinoid tumors be found every participant will be assisted in determine what the best course of treatment will be for them.

Recruitment Criteria

Accepts Healthy Volunteers

Healthy volunteers are participants who do not have a disease or condition, or related conditions or symptoms

 No
Study Type

An interventional clinical study is where participants are assigned to receive one or more interventions (or no intervention) so that researchers can evaluate the effects of the interventions on biomedical or health-related outcomes.


An observational clinical study is where participants identified as belonging to study groups are assessed for biomedical or health outcomes.


Searching Both is inclusive of interventional and observational studies.

 Observational
Eligible Ages 18 Years and Over
Gender All
More Inclusion & Exclusion Criteria

  • -

    INCLUSION CRITERIA:

    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)
  • - Male and female subjects >= 18 years of age.
  • - Have a diagnosis of small intestinal carcinoid tumor.
  • - Have at least one blood relation with a diagnosis of either small intestinal, pulmonary, kidney or gastropancreatic neuroendocrine tumor or metastatic neuroendocrine tumor of unknown primary.
Group 2 (Arm 1 or Arm 2)
  • - Male and female subjects >= 18 years of age.
  • - Has multiple synchronous primary small intestinal tumors.
Group 3 (Arm 1 or Arm 2)
  • - Male and female subjects >=18 years of age.
  • - Does not have a diagnosis of carcinoid tumor.
  • - Has one of the following: - at least two blood relatives with any combination of diagnoses of small intestinal carcinoid tumor, a pulmonary, kidney, gastropancreatic neuroendocrine tumor or metastatic neuroendocrine tumor of unknown primary OR.
  • - has at least one blood relative with multiple, synchronous primary small bowel tumors.
Group 4 (Arm 2 only)
  • - Male and female subjects >= 18 years of age.
  • - Not biologically related to the participating family but has offspring who is/are blood relative(s) of a participating subject.

EXCLUSION CRITERIA:

An individual who meets any of the following criteria will be excluded from participation in this. study: 1. 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. 2. Any condition which, in the opinion of the investigator, would make it unsafe to participate or would prohibit completion of the protocol. 3. Inability to provide informed consent. 4. Pregnant or breastfeeding

Trial Details

Trial ID:

This trial id was obtained from ClinicalTrials.gov, a service of the U.S. National Institutes of Health, providing information on publicly and privately supported clinical studies of human participants with locations in all 50 States and in 196 countries.

 NCT00646022
Phase

Phase 1: Studies that emphasize safety and how the drug is metabolized and excreted in humans.

Phase 2: Studies that gather preliminary data on effectiveness (whether the drug works in people who have a certain disease or condition) and additional safety data.

Phase 3: Studies that gather more information about safety and effectiveness by studying different populations and different dosages and by using the drug in combination with other drugs.

Phase 4: Studies occurring after FDA has approved a drug for marketing, efficacy, or optimal use.
Lead Sponsor

The sponsor is the organization or person who oversees the clinical study and is responsible for analyzing the study data.

 National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Principal Investigator

The person who is responsible for the scientific and technical direction of the entire clinical study.

 Stephen A Wank, M.D.
Principal Investigator Affiliation National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Agency Class

Category of organization(s) involved as sponsor (and collaborator) supporting the trial.

 NIH
Overall Status Recruiting
Countries United States
Conditions

The disease, disorder, syndrome, illness, or injury that is being studied.

 Carcinoid
Study Website: View Trial Website
Additional Details

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:

  • - Screen for occult disease and determine whether early detection affects the natural history of the disease.
  • - Compare the sensitivity and specificity of various imaging tomography (CT) with IV contrast and oral Volumen, 18FDOPA PET/CT scan, [68Ga] DOTATATE PET/CT scan and endoscopic modalities for diagnosing and following carcinoid tumors.
  • - Collect tumor specimens for histologic evaluation, culturing of intestinal organoids, and genotyping (including DNA and RNA sequencing).
  • - 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.

Arms & Interventions

Arms

: Arm 1

Participants who undergo extended evaluation for disease at NIH

: Arm 2

Participants who do not undergo extended screening or evaluation for disease at NIH

Interventions

Drug: - [18F]-DOPA

Contact a Trial Team

If you are interested in learning more about this trial, find the trial site nearest to your location and contact the site coordinator via email or phone. We also strongly recommend that you consult with your healthcare provider about the trials that may interest you and refer to our terms of service below.

Bethesda, Maryland

Status

Recruiting

Address

National Institutes of Health Clinical Center

Bethesda, Maryland, 20892

Site Contact

For more information at the NIH Clinical Center contact Office of Patient Recruitment (OPR)

[email protected]

800-411-1222 #TTY dial 711

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