ORPHA:97278
PPoma
Also known as: Pancreatic polypeptidoma
Publications
149
56.2th percentile
Trials
16
Interventional, condition-specific
Researchers
662
Distinct authors in sample
Gene link
—
Readiness
2/6
Stages with a signal
Clinical definition (Orphanet)
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).
How rare: How common this is has not been clearly measured.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0019957
- UMLS:C0346407
- NCIT:C67453
Additional Mondo synonyms (4)
pancreatic polypeptide neoplasm · pancreatic polypeptide tumor · pancreatic polypeptide tumour · pancreatic polypeptidoma
Research stages
Trial readiness signals
Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”
2/6 stages with a signal
An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
149 matched papers (58 in last 10 years) Source
- Phenotype characterisedNot checked
Not yet enriched from Monarch / HPO
- Animal modelNot checked
Not yet enriched from Monarch / Alliance
- Orphan designationNot checked
FDA/EMA orphan-drug designation not enriched yet
- Interventional trialPresent
16 matched on ClinicalTrials.gov
Biology
Genes and phenotypes
Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.
Do we know what causes it?
Not yet — the cause hasn't been pinned down in GenCC.
No strong gene–disease assertion joined for this Orphanet entity.
Phenotypes (Monarch / HPO)
Not enriched in this build — Monarch phenotype joins were not run for this record.
Animal models (Monarch / Alliance)
Not enriched in this build.
Literature
Is anyone studying this?
149
149 papers have ever been published on this condition (under this name). For scale, breast cancer has over 700,000. Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.
149 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).
58 in the last 10 years · medium confidence · 56.2th percentile (publications denominator)
Phrase hits: 149 · MeSH hits: 0
Who's working on it?
662
Distinct author names in 149 sampled papers — named people below.
Who's working on it?
People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.
- 01Tomita T7 papers · 2021
Department of Integrative Biosciences, Oregon Health and Science University, Portland, OR 97239-3097, USA. tomitat@ohsu.edu
Papers in Europe PMC - 02Thakker RV5 papers · 2020
Radcliffe Department of Medicine, Oxford Centre for Diabetes, Endocrinology and Metabolism (OCDEM), University of Oxford, Churchill Hospital, Headington, Oxford OX3 7LJ, UK. Electronic address: rajesh.thakker@ndm.ox.ac.uk.
Papers in Europe PMC - 03Bloom SR4 papers · 1989Papers in Europe PMC
- 04Jensen RT4 papers · 2012
Digestive Diseases Branch, National Institutes of Diabetes, Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA. roberj@bdg10.niddk.nih.gov
Papers in Europe PMC - 05Poma PA4 papers · 2000
Department of Obstetrics and Gynecology, Loyola University and Ravenswood Hospital Medical Center, Chicago, Illinois 60640-5205, USA. ppoma@rhmc.com
Papers in Europe PMC - 06Adrian TE3 papers · 1988Papers in Europe PMC
- 07Bastiat G3 papers · 2006
Laboratoire de Physico-Chimie des Polymères (L.P.C.P.), C.N.R.S./U.P.P.A. UMR 5067, Helioparc PAU-PYRENEES, 2 Av. du Président Angot, 64053 Pau 9, France.
Papers in Europe PMC - 08François J3 papers · 2006Papers in Europe PMC
- 09Grassl B3 papers · 2006Papers in Europe PMC
- 10Ito T3 papers · 2025
Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
16
interventional trials for this specific condition
16 interventional trials matched this specific condition name; none in our sample are currently recruiting.
Data as of 27 July 2026
16 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 93.7th percentile).
medium confidence · 93.7th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
16 interventional trials matched after quoted-phrase search and title/condition post-filter.
No currently recruiting studies in the matched set. Open the same search on ClinicalTrials.gov.
Where to find support
We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:
India — NPRD
Last verified 2026-07-26This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.
Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.
How we counted this
Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.
"PPoma" OR "Pancreatic polypeptidoma" OR "pancreatic polypeptide neoplasm" OR "pancreatic polypeptide tumor" OR "pancreatic polypeptide tumour"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"PPoma" OR "Pancreatic polypeptidoma" OR "pancreatic polypeptide neoplasm" OR "pancreatic polypeptide tumor" OR "pancreatic polypeptide tumour"
Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).
Study-type breakdown: 16 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.
Query health: ok — strategies attempted: phrase; with hits: phrase
Run this search on ClinicalTrials.gov
Confidence reasoning
- Preferred label is short or not clearly distinctive
- No synonyms dropped by stoplist
- No label/synonym collisions with other diseases in this corpus
Ingested 2026-07-27T05:04:42.388Z