The Human Phenotype Ontology (HPO) provides the following list of features that have been reported in people with this condition. Much of the information in the HPO comes from Orphanet, a European rare disease database. If available, the list includes a rough estimate of how common a feature is (its frequency). Frequencies are based on a specific study and may not be representative of all studies. You can use the MedlinePlus Medical Dictionary for definitions of the terms below.
| Signs and Symptoms | Approximate number of patients (when available) |
| Hypokalemia | Very frequent (present in 80%-99% of cases) |
| Very frequent (present in 80%-99% of cases) |
|
| Secretory diarrhea | Very frequent (present in 80%-99% of cases) |
| Anorexia | Frequent (present in 30%-79% of cases) |
| Chronic fatigue | Frequent (present in 30%-79% of cases) |
| Dehydration | Frequent (present in 30%-79% of cases) |
| Frequent (present in 30%-79% of cases) |
|
| Episodic abdominal pain | Frequent (present in 30%-79% of cases) |
| Erythema | Frequent (present in 30%-79% of cases) |
| Generalized muscle weakness | Frequent (present in 30%-79% of cases) |
| Hepatomegaly | Frequent (present in 30%-79% of cases) |
| Hypercalcemia | Frequent (present in 30%-79% of cases) |
| Malabsorption | Frequent (present in 30%-79% of cases) |
| Muscle cramps | Frequent (present in 30%-79% of cases) |
| Nausea and vomiting | Frequent (present in 30%-79% of cases) |
| Normochromic |
Frequent (present in 30%-79% of cases) |
| Poor appetite | Frequent (present in 30%-79% of cases) |
| Weight loss | Frequent (present in 30%-79% of cases) |
| Abnormal gastrointestinal motility | Occasional (present in 5%-29% of cases) |
| Ascites | Occasional (present in 5%-29% of cases) |
| Extrahepatic cholestasis | Occasional (present in 5%-29% of cases) |
| Intermittent |
Occasional (present in 5%-29% of cases) |
| Intrahepatic cholestasis | Occasional (present in 5%-29% of cases) |
| Adrenocortical adenoma | Very rare (present in 1%-4% of cases) |
| Benign gastrointestinal tract tumors | Very rare (present in 1%-4% of cases) |
| Elevated calcitonin | Very rare (present in 1%-4% of cases) |
| Follicular thyroid carcinoma | Very rare (present in 1%-4% of cases) |
| Ganglioneuroma | Very rare (present in 1%-4% of cases) |
| Growth hormone excess | Very rare (present in 1%-4% of cases) |
| Increased circulating cortisol level | Very rare (present in 1%-4% of cases) |
| Increased circulating gonadotropin level | Very rare (present in 1%-4% of cases) |
| Neoplasm of the liver | Very rare (present in 1%-4% of cases) |
| Parathyroid adenoma | Very rare (present in 1%-4% of cases) |
| Pituitary adenoma | Very rare (present in 1%-4% of cases) |
| Primary hyperparathyroidism | Very rare (present in 1%-4% of cases) |
| Prolactin excess | Very rare (present in 1%-4% of cases) |
| Respiratory insufficiency due to muscle weakness | Very rare (present in 1%-4% of cases) |
| Subcutaneous lipoma | Very rare (present in 1%-4% of cases) |
Making a diagnosis for a genetic or rare disease can often be challenging. Healthcare professionals typically look at a person’s medical history, symptoms, physical exam, and laboratory test results in order to make a diagnosis. The following resources provide information relating to diagnosis and testing for this condition. If you have questions about getting a diagnosis, you should contact a healthcare professional.
Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
Don’t Miss the NCATS Toolkit for Patient-Focused Therapy Development Event
August 31, 2017
Funding Opportunities Now Available for the Undiagnosed Diseases Network (UDN) Phase II
August 30, 2017
IRDiRC Goals 2017-2027: New Rare Disease Research Goals for the Next Decade
August 10, 2017
Overcoming Barriers to International Clinical Trials for Rare Cancers
Friday, December 10, 2010
Location:
Bethesda,
Maryland
Description: The goals of this conference were to introduce key institutional players to the topic of international clinical trials in rare cancers and to establish an ongoing dialogue. Participants left the meeting with a set of specific priorities that need to be enacted to promote these trials. The meeting promoted consensus on the way that resources are prioritized to address rare cancers. Participants were asked to convey the content of the meeting to their constituencies and to follow up with pilot concepts.
Contact: Jack Welch, M.D., Ph.D., jack.welch@nih.govjack.welch@nih.gov
Co-funding Institute(s): National Cancer Institute, Office of Rare Diseases Research
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My husband was diagnosed in 1998 with VIPoma. He uses octreotide to help control the symptoms. Is there any other known treatment (other than surgery) for this rare cancer? See answer
