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Protocol Details

Natural History of Familial Carcinoid Tumor

This study is NOT currently recruiting participants.

Summary | Eligibility | Citations | Contacts




Sponsoring Institute

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

Recruitment Detail

Type: Clinical hold/Recruitment or enrollment suspended
Gender: Male & Female
Min Age: 18 Years
Max Age: N/A

Referral Letter Required


Population Exclusion(s)

Pregnant Women;

Special Instructions

Currently Not Provided



Recruitment Keyword(s)

Carcinoid Tumor;
Gastrointestinal Carcinoid Tumor;
Familial Cancer Tumor



Investigational Drug(s)

(18-F) DOPA
[68Ga] Dotatate

Investigational Device(s)




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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1. Adult patients who a) have a present diagnosis of small intestinal carcinoid tumor and have at least one blood relation with an immediate or distant family member with a documented diagnosis of either small intestinal, pulmonary, kidney or gastropancreatic neuroendocrine tumor or metastatic neuroendocrine tumor of unknown primary b) do not have a diagnosis of small intestinal carcinoid tumor and have at least two immediate or distant family members with documented diagnoses of either small intestinal, pulmonary kidney, gastropancreatic neuroendocrine tumor or metastatic neuroendocrine tumor of unknown primary or c) patients who have multiple synchronous primary small intestinal tumors without a known relative with a neuroendocrine tumor of any type. This includes family members of patients with carcinoid tumors even if the patients with tumors are unwilling to participate so long as we have appropriate documentation confirming the diagnosis of the carcinoid tumors in the affected family members. Adult patients who are unable to provide informed consent but whose wishes suggest they are willing to donate samples for research purposes will be considered for study enrollment.

2. Unaffected spouses of patients with a carcinoid tumor and who have children.


1. Families with multiple endocrine neoplasia (MEN) I, MEN II or other familial tumor syndromes such as Von Hippel Lindau Syndrome and Neurofibomatosis for which there is a known genetic predisposition to non-carcinoid tumors as well as carcinoid tumors will be excluded from the study.

2. Pregnancy, breastfeeding.

3. Anticoagulation, seizures, severe systemic disease of any sort, advanced metastatic carcinoid may be relative exclusion criteria prohibiting a full evaluation as described above under protocol design. However, these medical conditions should not absolutely exclude participation in the protocol. Participation in each protocol delineated evaluation procedure will be judged on a case by case basis with patient safety as the paramount consideration.

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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)
BG 10 RM 9C101
(301) 496-4202

Joanne Forbes, C.R.N.P.
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
BG 10-CRC 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: 1-866-411-1010

Clinical Trials Number:


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