ORPHA:313906
Congenital pancreatic cyst
Also known as: Neonatal congenital pancreatic cyst · True congenital pancreatic cyst
Clinical definition (Orphanet)
A rare pancreatic disease characterized by a most commonly single, unilocular, thin-walled cystic lesion which may be located anywhere within the pancreas (but is more frequently found in the body and tail) and does not communicate with the pancreatic ductal system. Patients may be asymptomatic or present with signs and symptoms of gastrointestinal or biliary obstruction, or pancreatitis. The condition can be isolated or occur in association with other anomalies (such as von Hippel-Lindau disease or polycystic kidney disease).
How rare: <1 / 1 000 000 — fewer than one in a million. In a city the size of Kolkata, that might mean on the order of fifteen people.
Is anyone studying this?
42
42 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=183) is 38.
42 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 38 (publications denominator n=183).
8 in the last 10 years · high confidence · 27.9th percentile (publications denominator)
Is a treatment being tested?
0
trials for this specific condition
No interventional trial testing a treatment matched this specific condition name on ClinicalTrials.gov (observational studies and pan-disease registries are listed separately when present).
Data as of 26 July 2026
No matched interventional trials. This is true for 59.2% of diseases in the trials denominator (151 of 255). Here are the researchers publishing on it.
high confidence · 29.6th percentile (trials denominator)
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.
Who's working on it?
199
Distinct author names in 42 sampled papers — named people below.
Who's working on it?
No interventional trial matched this name; these authors publish on it in the sampled literature — a practical starting point for contact.
- 01Al-Salem AH2 papers · 2014
Department of Pediatric Surgery, Maternity and Children Hospital, Dammam, Saudi Arabia.
Papers in Europe PMC - 02Boulanger SC2 papers · 2007
Division of Pediatric Surgical Services, Department of Surgery, The State University of New York at Buffalo, School of Medicine and Biomedical Sciences, Buffalo, NY, USA.
Papers in Europe PMC - 03Matta H2 papers · 2014
Department of Surgery, Tawam Hospital, Al-Ain, Abu Dhabi, United Arab Emirates.
Papers in Europe PMC - 04Singh S2 papers · 2023
Division of Gastroenterology and Hepatology, University of Nebraska Medical Center, Omaha, NE, USA.
Papers in Europe PMC - 05Achugatla S1 paper · 2023
Department of Paediatric Surgery, Lilavati Hospital and Research Centre, Mumbai, Maharashtra, India.
Papers in Europe PMC - 06Adler DG1 paper · 2022
Huntsman Cancer Center, University of Utah School of Medicine, Salt Lake City, UT, USA.
Papers in Europe PMC - 07Aloui S1 paper · 2012Papers in Europe PMC
- 08Angioni A1 paper · 2014Papers in Europe PMC
- 09Aslan O1 paper · 2000Papers in Europe PMC
- 10
Recruiting interventional trials
Trials testing a treatment from the matched ClinicalTrials.gov set
No interventional trial testing a treatment was found for this specific condition name on ClinicalTrials.gov.
See who's working on it above — people publishing on this disease are often the practical next contact when no trial is listed.
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 below but are not added to the query string.
"Congenital pancreatic cyst" OR "Neonatal congenital pancreatic cyst" OR "True congenital pancreatic cyst"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Congenital pancreatic cyst" OR "Neonatal congenital pancreatic cyst" OR "True congenital pancreatic cyst"
Study-type breakdown: 0 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.
Cross-references (from Mondo): UMLS:C0341480
Query health: ok — strategies attempted: phrase; with hits: phrase
Run this search on ClinicalTrials.gov
Confidence reasoning
- Preferred label is multi-word and distinctive
- No synonyms dropped by stoplist
- No label/synonym collisions with other diseases in this corpus