PPoma
Synonyms: Pancreatic polypeptidoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
PPoma is a type of pancreatic endocrine tumor that hypersecretes pancreatic polypeptide (PP) but that does not cause a hypersecretion syndrome (is non-functioning) and instead presents with only non-specific symptoms such as weight loss, abdominal pain, jaundice, diarrhea and/or an abdominal mass, hence leading to a late diagnosis. PPoma can be associated with multiple endocrine neoplasia 1 (MEN-1).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | PPoma | Pancreatic polypeptidoma |
| fr | PPome | Polypeptidome pancréatique · Tumeur à polypeptide pancréatique |
| de | PPoma | Pankreatisches Polypeptidom |
| es | PPoma | |
| it | PPoma | Polipeptidoma pancreatico |
| nl | PPoom | Pancreatisch polypeptidoom |
| pt | PPoma | |
| pl | Nowotwór wydzielający polipeptyd trzustkowy | Wyspiak wydzielający polipeptyd trzustkowy |
| cs | PPom | Pankreatický polypeptidom |
| uk | PPoma | Поліпептидома підшлункової залози |
| zh | 胰多肽瘤 | 胰多肽瘤 |
| tr | PPom | Pankreas polipeptidoması |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D37.7 | NTBT | attributed | validated |
| ICD-10 | E16.8 | ND | attributed | validated |
| ICD-11 | 2C10.1 | NTBT | index | validated |
| MONDO | 0019957 | E | validated | |
| UMLS | C0346407 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:506060Functioning neuroendocrine tumor of pancreas
rare gastroenterological diseases; rare endocrine diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
32 annotations · validated 2017-02-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002894 | Neoplasm of the pancreas | |
| HP:0100833 | Neoplasm of the small intestine | |
| Frequent (79-30%) | ||
| HP:0001438 | Abnormality of abdomen morphology | |
| HP:0002039 | Anorexia | |
| HP:0002019 | Constipation | |
| HP:0002014 | Diarrhea | |
| HP:0002574 | Episodic abdominal pain | |
| HP:0002240 | Hepatomegaly | |
| HP:0030144 | Hypoactive bowel sounds | |
| HP:0002017 | Nausea and vomiting | |
| HP:0004396 | Poor appetite | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0001541 | Ascites | |
| HP:0001081 | Cholelithiasis | |
| HP:0012334 | Extrahepatic cholestasis | |
| HP:0002239 | Gastrointestinal hemorrhage | |
| HP:0001046 | Intermittent jaundice | |
| HP:0006723 | Intestinal carcinoid | |
| HP:0005214 | Intestinal obstruction | |
| HP:0001406 | Intrahepatic cholestasis | |
| HP:0030145 | Lack of bowel sounds | |
| Very rare (<4-1%) | ||
| HP:0000820 | Abnormality of the thyroid gland | |
| HP:0008256 | Adrenocortical adenoma | |
| HP:0000845 | Elevated circulating growth hormone concentration | |
| HP:0003072 | Hypercalcemia | |
| HP:0003118 | Increased circulating cortisol level | |
| HP:0000837 | Increased circulating gonadotropin level | |
| HP:0000870 | Increased circulating prolactin concentration | |
| HP:0002897 | Parathyroid adenoma | |
| HP:0002893 | Pituitary adenoma | |
| HP:0008200 | Primary hyperparathyroidism | |
| HP:0001031 | Subcutaneous lipoma | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 26742109[PMID]_22997445[PMID]_22261872[PMID]_23582916[PMID]_18798544[PMID]_10940685[PMID]_2440390[PMID]_18941127[PMID]_22052063[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/97278
- This record as JSON: /data/d/97278.json