RARE DISEASERESEARCH ATLAS

ORPHA:506090

Serotonin-producing neuroendocrine tumor of pancreas

low confidenceDisorder

Also known as: Serotonin-producing PNET · Serotonin-producing pancreatic NET · Serotonin-producing pancreatic neuroendocrine tumor

Publications

2,319

Trials

3

Interventional, condition-specific

Researchers

1,248

Distinct authors in sample

Gene link

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare functioning neuroendocrine tumor of pancreas characterized by a typically well-differentiated neoplasm composed of cells expressing serotonin. Patients may present with atypical carcinoid syndrome with abdominal pain, diarrhea, weight loss, and/or flushing. Carcinoid syndrome is usually present only when there are liver metastases. The tumors tend to be larger than non-functioning tumors and are associated with a poorer prognosis because they are almost always metastatic.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (23)

EC cell, serotonin producing pancreatic NET · EC cell, serotonin producing pancreatic neuroendocrine tumor · EC cell, serotonin producing pancreatic neuroendocrine tumour · carcinoid neoplasm of pancreas · carcinoid neoplasm of the pancreas · carcinoid tumor of pancreas · carcinoid tumor of the pancreas · carcinoid tumour of pancreas · carcinoid tumour of the pancreas · enterochromaffin cell serotonin-producing pancreatic neuroendocrine tumor · pancreatic carcinoid tumor · pancreatic carcinoid tumour · pancreatic serotonin producing neoplasm · pancreatic serotonin producing tumor · pancreatic serotonin producing tumour · serotonin-producing PNET · serotonin-producing pancreatic NET · serotonin-producing pancreatic neuroendocrine tumor · serotonin-producing pancreatic neuroendocrine tumour · serotonin-producing tumor of pancreas · serotonin-producing tumor of the pancreas · serotonin-producing tumour of pancreas · serotonin-producing tumour of the pancreas

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.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    2,319 matched papers (914 in last 10 years) Source

  3. Phenotype characterisedNot found

    No HPO disease–phenotype associations via Monarch for these Mondo IDs

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  6. Interventional trialPresent

    3 matched on ClinicalTrials.gov (2 recruiting in sample)

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)

None returned for this Mondo ID. That often means “not linked under this ID,” not “no clinical features.”

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.

Open Targets candidates

No drugs or clinical candidates returned for this Mondo ID on Open Targets.

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

2,319

2,319 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

2,319 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

914 in the last 10 years · low confidence

Phrase hits: 2,319 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,248

Distinct author names in 200 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.

  1. 01
    Wang Y6 papers · 2026

    Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China.

    Papers in Europe PMC
  2. 02
    Zhang Y4 papers · 2026

    Department of Endocrinology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China.

    Papers in Europe PMC
  3. 03
    Chiang CH3 papers · 2022

    Division of General Medicine, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Foundation, New Taipei City, Taiwan.

    Papers in Europe PMC
  4. 04
    Liu Y3 papers · 2026

    Department of Radiology, Zibo Central Hospital, Zibo, China.

    Papers in Europe PMC
  5. 05
    Yang Y3 papers · 2026

    The First College of Clinical Medical Science, China Three Gorges University, Hubei Yichang, China.

    Papers in Europe PMC
  6. 06
    Zhang L3 papers · 2026

    School of Marxism, Anhui Medical University, Hefei, Anhui, 230032, China. zhangliping@ahmu.edu.cn.

    Papers in Europe PMC
  7. 07
    Ammori JB2 papers · 2022

    Department of Surgery, Division of Surgical Oncology, University Hospitals Cleveland Medical Center, Cleveland, OH, USA.

    Papers in Europe PMC
  8. 08
    Bara T2 papers · 2025

    2 Maros Megyei Sürgősségi Klinikai Kórház, II. Sebészeti Klinika Marosvásárhely Románia.

    Papers in Europe PMC
  9. 09
    Bösch F2 papers · 2026

    Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, LMU, Klinikum der Universität München, Deutschland.

    Papers in Europe PMC
  10. 10
    Chen Y2 papers · 2026

    School of Health Care Management, Anhui Medical University, Hefei, 230032, China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

3

interventional trials for this specific condition

3 interventional trials matched this specific condition name; 2 currently recruiting in our sample.

Data as of 11 September 2026

3 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 86.7th percentile).

low confidence · 86.7th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

3 interventional trials matched after quoted-phrase search and title/condition post-filter.

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 0 · after dedupe 0 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 0 · dropped 0 · fetched 2026-07-30

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Serotonin-producing neuroendocrine tumor of pancreas — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This 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.

"Serotonin-producing neuroendocrine tumor of pancreas" OR "Serotonin-producing neuroendocrine tumor of the pancreas" OR "Serotonin-producing PNET" OR "Serotonin-producing pancreatic NET" OR "Serotonin-producing pancreatic neuroendocrine tumor" OR "EC cell, serotonin producing pancreatic NET" OR "EC cell, serotonin producing pancreatic neuroendocrine tumor" OR "EC cell, serotonin producing pancreatic neuroendocrine tumour" OR "carcinoid neoplasm of pancreas" OR "carcinoid neoplasm of the pancreas" OR "carcinoid tumor of pancreas" OR "carcinoid tumor of the pancreas" OR "carcinoid tumour of pancreas" OR "carcinoid tumour of the pancreas" OR "enterochromaffin cell serotonin-producing pancreatic neuroendocrine tumor" OR "pancreatic carcinoid tumor" OR "pancreatic carcinoid tumour" OR "pancreatic serotonin producing neoplasm" OR "pancreatic serotonin producing tumor" OR "pancreatic serotonin producing tumour" OR "serotonin-producing pancreatic neuroendocrine tumour" OR "serotonin-producing tumor of pancreas" OR "serotonin-producing tumor of the pancreas" OR "serotonin-producing tumour of pancreas" OR "serotonin-producing tumour of the pancreas"

Run this search on Europe PMC

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Serotonin-producing neuroendocrine tumor of pancreas" OR "Serotonin-producing neuroendocrine tumor of the pancreas" OR "Serotonin-producing PNET" OR "Serotonin-producing pancreatic NET" OR "Serotonin-producing pancreatic neuroendocrine tumor" OR "EC cell, serotonin producing pancreatic NET" OR "EC cell, serotonin producing pancreatic neuroendocrine tumor" OR "EC cell, serotonin producing pancreatic neuroendocrine tumour" OR "carcinoid neoplasm of pancreas" OR "carcinoid neoplasm of the pancreas" OR "carcinoid tumor of pancreas" OR "carcinoid tumor of the pancreas" OR "carcinoid tumour of pancreas" OR "carcinoid tumour of the pancreas" OR "enterochromaffin cell serotonin-producing pancreatic neuroendocrine tumor" OR "pancreatic carcinoid tumor" OR "pancreatic carcinoid tumour" OR "pancreatic serotonin producing neoplasm" OR "pancreatic serotonin producing tumor" OR "pancreatic serotonin producing tumour" OR "serotonin-producing pancreatic neuroendocrine tumour" OR "serotonin-producing tumor of pancreas" OR "serotonin-producing tumor of the pancreas" OR "serotonin-producing tumour of pancreas" OR "serotonin-producing tumour of the pancreas"

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: 3 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 multi-word and distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus
  • Publication count (2319) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity

Ingested 2026-07-27T17:51:13.580Z