CarcinoidNCT00646022
Natural History of Familial Carcinoid Tumor
An observational study of Carcinoid, run by National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) at 1 site in United States.
Trial summary
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… Read more about this study
- Who is it for?
- Ages 18 to 100 years
- Conditions the study is for
- Carcinoid
- What is being tested?
- [18F]-DOPA
- How many people
- 1,600 participants
- Where
- 1 siteUnited States
- Study dates
- 25 Aug 2008to not given
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 to 100 years
- All sexes
- No healthy volunteers
- Adult, Older adult
Study population: Community sample
Who may be able to join
- 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
Show all 18Show fewer
- 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.
Who cannot join
- 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.
- Any condition which, in the opinion of the investigator, would make it unsafe to participate or would prohibit completion of the protocol.
- Inability to provide informed consent (Arm 1 only)
- Pregnant or breastfeeding (Arm 1 only)
Trial locations and contacts
1 site in 1 country. Contact the study team or a site near you to ask about taking part.
United States1 site, 1 recruiting
- Recruiting
National Institutes of Health Clinical Center
Bethesda, Maryland, 20892
For more information at the NIH Clinical Center contact Office of Patient Recruitment (OPR), 800-411-1222 ext. TTY dial 711, ccopr@nih.gov
About this study
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.
Read more
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.
Read the detailed description
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 Breeza, 18F-DOPA 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.
- Conditions
- Carcinoid
- Being tested
- [18F]-DOPA
- Keywords
- Neuroendocrine, PET, Gastrointestinal, Serotonin, Natural History, Carcinoid Tumor, Gastrointestinal Carcinoid Tumor, Familial Cancer Tumor
- Medical subject headings
- Carcinoid Tumor
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
[18F]-DOPADrug
18F-DOPA PET/CT Scan.
| Study group | Role | What they receive |
|---|---|---|
| Arm 1 | Participants who undergo extended evaluation for disease at NIH Receives: Drug: [18F]-DOPA | |
| Arm 2 | Participants who do not undergo extended screening or evaluation for disease at NIH Receives: Drug: [18F]-DOPA |
What is being measured
The primary outcome is the main question the study is designed to answer.
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
Measured: End of study
Secondary outcomes (4)
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
Measured: 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
Measured: 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
Measured: End of study
Screen for occult disease and determine whether early detection affects the natural history of the disease
Change in survival
Measured: End of study
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted28 Mar 2008 (estimated)
- Study start25 Aug 2008
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (4)Papers the sponsor has linked to this study.
- Other trials for CarcinoidStudies of the same condition, open and under way.
- More trials run by National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)The sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Family based
- Time perspective
- Prospective
- Enrolment
- 1,600 participants (estimated)
Sponsor and investigators
- Sponsor type
- US National Institutes of Health
- Responsible party
- Sponsor
- Investigators
- Stephen A Wank, M.D., Principal investigator, National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT00646022
- Sponsor's protocol ID
- 080098
- Other IDs
- 08-DK-0098
- Status last verified
- 22 Oct 2026
- First submitted
- 26 Mar 2008
- Last updated
- 5 Nov 2026
- Results posted
- No
- FDA-regulated drug
- Yes
- FDA-regulated device
- No
- Individual data sharing
- No
Publications
Caplin ME, Buscombe JR, Hilson AJ, Jones AL, Watkinson AF, Burroughs AK. Carcinoid tumour. Lancet. 1998 Sep 5;352(9130):799-805. doi: 10.1016/S0140-6736(98)02286-7.
PubMed 9737302Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer. 2003 Feb 15;97(4):934-59. doi: 10.1002/cncr.11105.
PubMed 12569593Capella C, Heitz PU, Hofler H, Solcia E, Kloppel G. Revised classification of neuroendocrine tumours of the lung, pancreas and gut. Virchows Arch. 1995;425(6):547-60. doi: 10.1007/BF00199342.
PubMed 7697211Tang D, Lim R, Korman L, Forbes J, Ellsbury K, Auh S, Trivedi A, Chen CC, Hughes M, Wank S. Performance of capsule endoscopy for the detection of small intestinal neuroendocrine tumors in familial carcinoid: a prospective single-site study. Gastrointest Endosc. 2024 Feb;99(2):227-236. doi: 10.1016/j.gie.2023.08.024. Epub 2023 Oct 13.
PubMed 37838323
Study at a glance
From the registry record, last updated by the sponsor on 5 Nov 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Natural History of Familial Carcinoid Tumor
- ClinicalTrials.gov ID
- NCT00646022
- Acronym / sponsor ID
- 080098
- Conditions
- Carcinoid
- Keywords
- Neuroendocrine, PET, Gastrointestinal, Serotonin, Natural History, Carcinoid Tumor, Gastrointestinal Carcinoid Tumor, Familial Cancer Tumor
- Study type
- Observational
- Recruitment status
- Recruiting
- Enrolment
- 1,600 participants (estimated)
- Lead sponsor
- National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)US National Institutes of Health
- Study start
- 25 Aug 2008 (actual)
- First posted
- 28 Mar 2008 (estimated)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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.
Detailed description
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 Breeza, 18F-DOPA 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.
Study design
- Observational model
- Family based
- Time perspective
- Prospective
- Number of arms
- 2
| Arm | Type | Receives |
|---|---|---|
| Arm 1 | – | Drug: [18F]-DOPAParticipants who undergo extended evaluation for disease at NIH |
| Arm 2 | – | Drug: [18F]-DOPAParticipants who do not undergo extended screening or evaluation for disease at NIH |
Who may qualify
- Ages
- 18 to 100 years
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
- Study population
- Community sample
Key inclusion criteria (18 listed)
- 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
Key exclusion criteria (7 listed)
- An individual who meets any of the following criteria will be excluded from participation in this
- 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.
- Any condition which, in the opinion of the investigator, would make it unsafe to participate or would prohibit completion of the protocol.
- Inability to provide informed consent (Arm 1 only)
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| [18F]-DOPA18F-DOPA PET/CT Scan. | Drug | Arm 1, Arm 2 |
Primary outcome
Study the natural history of familial carcinoid tumors
Time frame: End of study
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
4 secondary outcomes
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 ...
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
Time frame: End of study
Collect tumor specimens for histologic evaluation, culturing of intestinal organoids, and genotyping (including DNA and RNA sequencing)
Time frame: End of study
Screen for occult disease and determine whether early detection affects the natural history of the disease
Time frame: End of study
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 5 Nov 2026.
- Background: PubMed 9737302Caplin ME, Buscombe JR, Hilson AJ, Jones AL, Watkinson AF, Burroughs AK. Carcinoid tumour. Lancet. 1998 Sep 5;352(9130):799-805. doi: 10.1016/S0140-6736(98)02286-7.
- Background: PubMed 12569593Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer. 2003 Feb 15;97(4):934-59. doi: 10.1002/cncr.11105.
- Background: PubMed 7697211Capella C, Heitz PU, Hofler H, Solcia E, Kloppel G. Revised classification of neuroendocrine tumours of the lung, pancreas and gut. Virchows Arch. 1995;425(6):547-60. doi: 10.1007/BF00199342.
- Derived from this study: PubMed 37838323Tang D, Lim R, Korman L, Forbes J, Ellsbury K, Auh S, Trivedi A, Chen CC, Hughes M, Wank S. Performance of capsule endoscopy for the detection of small intestinal neuroendocrine tumors in familial carcinoid: a prospective single-site study. Gastrointest Endosc. 2024 Feb;99(2):227-236. doi: 10.1016/j.gie.2023.08.024. Epub 2023 Oct 13.
- NIH Clinical Center Detailed Web PageLink provided by the sponsor.
- Individual participant dataSharing planned: No
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
Stephen A Wank, M.D. principal investigator
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Carcinoid, run by National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) at 1 site in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 18 inclusion and 7 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: looking for participants now, as of the sponsor's last update on 5 Nov 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact Joanne Forbes, C.R.N.P. ((301) 443-9557, forbesjo@mail.nih.gov), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Study the natural history of familial carcinoid tumors (time frame: End of study). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT00646022, which also lists 4 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 5 Nov 2026.