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Protocol Details

Natural History of Familial Carcinoid Tumor

This study is currently recruiting participants.

Summary | Eligibility | Citations | Contacts




Sponsoring Institute

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

Recruitment Detail

Type: Participants currently recruited/enrolled
Gender: Male & Female
Min Age: 18 Years
Max Age: N/A

Referral Letter Required


Population Exclusion(s)

Pregnant Women;


Natural History

Recruitment Keyword(s)

Carcinoid Tumor;
Gastrointestinal Carcinoid Tumor;
Familial Cancer Tumor



Investigational Drug(s)

(18-F) DOPA
[68Ga] Dotatate

Investigational Device(s)



Drug: [18F]-DOPA

Supporting Site

National Institute of Diabetes and Digestive and Kidney Diseases

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.

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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.


An individual who meets any of the following criteria will be excluded from participation in this


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

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Caplin ME, Buscombe JR, Hilson AJ, Jones AL, Watkinson AF, Burroughs AK. Carcinoid tumour. Lancet. 1998 Sep 5;352 (9130):799-805.

Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer. 2003 Feb 15;97 (4):934-59.

Capella C, Heitz PU, H(SqrRoot)(Delta)fler H, Solcia E, Kl(SqrRoot)(Delta)ppel G. Revised classification of neuroendocrine tumours of the lung, pancreas and gut. Virchows Arch. 1995;425(6):547-60

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Principal Investigator

Referral Contact

For more information:

Stephen A. Wank, M.D.
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
(301) 496-4202

Joanne Forbes, C.R.N.P.
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
NIHBC 10 - CRC BG RM 5-2740
(301) 443-9557

Office of Patient Recruitment
National Institutes of Health Clinical Center (CC)
Building 61, 10 Cloister Court
Bethesda, Maryland 20892
Toll Free: 1-800-411-1222
Local Phone: 301-451-4383
TTY: TTY Users Dial 7-1-1

Clinical Trials Number:


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