PPoma
diseaseOn this page
Also known as pancreatic polypeptide neoplasmpancreatic polypeptide tumorpancreatic polypeptide tumourpancreatic polypeptidoma
Summary
PPoma (MONDO:0019957) is a disease and 16 clinical trials. Top therapeutic interventions include gefitinib, octreotide acetate, and pazopanib hydrochloride. A subtype of pancreatic neuroendocrine tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: Unknown (Worldwide) [Orphanet-validated]
- Phenotypes (HPO): 32
- Clinical trials: 16
Clinical features
Signs & symptoms
Clinical features (HPO)
32 HPO clinical features (Orphanet curated; top 32 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0002894 | Neoplasm of the pancreas | Very frequent (80-99%) |
| HP:0100833 | Neoplasm of the small intestine | Very frequent (80-99%) |
| HP:0001438 | Abnormality of abdomen morphology | Frequent (30-79%) |
| HP:0001824 | Weight loss | Frequent (30-79%) |
| HP:0002014 | Diarrhea | Frequent (30-79%) |
| HP:0002017 | Nausea and vomiting | Frequent (30-79%) |
| HP:0002019 | Constipation | Frequent (30-79%) |
| HP:0002039 | Anorexia | Frequent (30-79%) |
| HP:0002240 | Hepatomegaly | Frequent (30-79%) |
| HP:0002574 | Episodic abdominal pain | Frequent (30-79%) |
| HP:0004396 | Poor appetite | Frequent (30-79%) |
| HP:0030144 | Hypoactive bowel sounds | Frequent (30-79%) |
| HP:0001046 | Intermittent jaundice | Occasional (5-29%) |
| HP:0001081 | Cholelithiasis | Occasional (5-29%) |
| HP:0001406 | Intrahepatic cholestasis | Occasional (5-29%) |
| HP:0001541 | Ascites | Occasional (5-29%) |
| HP:0002239 | Gastrointestinal hemorrhage | Occasional (5-29%) |
| HP:0005214 | Intestinal obstruction | Occasional (5-29%) |
| HP:0006723 | Intestinal carcinoid | Occasional (5-29%) |
| HP:0012334 | Extrahepatic cholestasis | Occasional (5-29%) |
| HP:0030145 | Lack of bowel sounds | Occasional (5-29%) |
| HP:0000820 | Abnormality of the thyroid gland | Very rare (<1-4%) |
| HP:0000837 | Increased circulating gonadotropin level | Very rare (<1-4%) |
| HP:0000845 | Elevated circulating growth hormone concentration | Very rare (<1-4%) |
| HP:0000870 | Increased circulating prolactin concentration | Very rare (<1-4%) |
| HP:0001031 | Subcutaneous lipoma | Very rare (<1-4%) |
| HP:0002893 | Pituitary adenoma | Very rare (<1-4%) |
| HP:0002897 | Parathyroid adenoma | Very rare (<1-4%) |
| HP:0003072 | Hypercalcemia | Very rare (<1-4%) |
| HP:0003118 | Increased circulating cortisol level | Very rare (<1-4%) |
| HP:0008200 | Primary hyperparathyroidism | Very rare (<1-4%) |
| HP:0008256 | Adrenocortical adenoma | Very rare (<1-4%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | PPoma |
| Mondo ID | MONDO:0019957 |
| Orphanet | 97278 |
| ICD-11 | 1180851464 |
| NCIT | C67453 |
| SNOMED CT | 255039001 |
| UMLS | C0346407 |
| MedGen | 577392 |
| GARD | 0019358 |
| Is cancer (heuristic) | no |
Also known as: pancreatic polypeptide neoplasm · pancreatic polypeptide tumor · pancreatic polypeptide tumour · pancreatic polypeptidoma
Disease family
This is a subtype of pancreatic neuroendocrine tumor. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by body system or component › digestive system disorder › digestive system neuroendocrine neoplasm › digestive system neuroendocrine tumor, grade 1/2 › pancreatic neuroendocrine tumor › PPoma
Related subtypes (10): pancreatic delta cell neuroendocrine tumor, pancreatic gastrin-producing neuroendocrine tumor, non-functional pancreatic neuroendocrine tumor, pancreatic insulin-producing neuroendocrine tumor, somatostatinoma, GRFoma, glucagonoma, VIPoma, pancreatic neuroendocrine tumor G1, functional pancreatic neuroendocrine tumor
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 16.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 11 |
| PHASE1 | 5 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT01824875 | PHASE2 | ACTIVE_NOT_RECRUITING | Temozolomide With or Without Capecitabine in Treating Patients With Advanced Pancreatic Neuroendocrine Tumors |
| NCT00075439 | PHASE2 | COMPLETED | Gefitinib in Treating Patients With Progressive Metastatic Neuroendocrine Tumors |
| NCT00084461 | PHASE2 | TERMINATED | Romidepsin in Treating Patients With Locally Advanced or Metastatic Neuroendocrine Tumors |
| NCT00131911 | PHASE2 | COMPLETED | Sorafenib Tosylate in Treating Patients With Progressive Metastatic Neuroendocrine Tumors |
| NCT00454363 | PHASE2 | COMPLETED | Pazopanib Hydrochloride in Treating Patients With Advanced Neuroendocrine Cancer |
| NCT01010126 | PHASE2 | COMPLETED | Temsirolimus and Bevacizumab in Treating Patients With Advanced Endometrial, Ovarian, Liver, Carcinoid, or Islet Cell Cancer |
| NCT01525082 | PHASE2 | COMPLETED | Capecitabine, Temozolomide, and Bevacizumab for Metastatic or Unresectable Pancreatic Neuroendocrine Tumors |
| NCT02031536 | PHASE2 | TERMINATED | Everolimus in Patients With Pancreatic Neuroendocrine Tumors Metastatic to the Liver Previously Treated With Surgery |
| NCT02108782 | PHASE2 | WITHDRAWN | Dovitinib Lactate in Treating Patients With Pancreatic Neuroendocrine Tumors |
| NCT02259725 | PHASE2 | COMPLETED | Regorafenib in Treating Patients With Advanced or Metastatic Neuroendocrine Tumors |
| NCT02273752 | PHASE2 | TERMINATED | Pharmacokinetically Guided Everolimus in Patients With Breast Cancer, Pancreatic Neuroendocrine Tumors, or Kidney Cancer |
| NCT00004074 | PHASE1 | COMPLETED | Interleukin-12 and Trastuzumab in Treating Patients With Cancer That Has High Levels of HER2/Neu |
| NCT00655655 | PHASE1 | COMPLETED | Everolimus and Vatalanib in Treating Patients With Advanced Solid Tumors |
| NCT01155258 | PHASE1 | COMPLETED | Temsirolimus and Vinorelbine Ditartrate in Treating Patients With Unresectable or Metastatic Solid Tumors |
| NCT01204476 | PHASE1 | COMPLETED | Cixutumumab, Everolimus, and Octreotide Acetate in Treating Patients With Advanced Low to Intermediate Grade Neuroendocrine Carcinoma |
| NCT03147768 | PHASE1 | COMPLETED | Laser Tissue Welding - Distal Pancreatectomy Sealing Study |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| GEFITINIB | 4 | 1 |
| OCTREOTIDE ACETATE | 4 | 1 |
| PAZOPANIB HYDROCHLORIDE | 4 | 1 |
| REGORAFENIB | 4 | 1 |
| ROMIDEPSIN | 4 | 1 |
| SORAFENIB | 4 | 1 |
| TEMSIROLIMUS | 4 | 1 |
| VATALANIB | 3 | 2 |
| CIXUTUMUMAB | 2 | 1 |
| DOVITINIB LACTATE | 2 | 1 |
| EDODEKIN ALFA | 2 | 1 |
| CHEMBL4066465 | 0 | 1 |