RARE DISEASERESEARCH ATLAS
Open navigation menu

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 checked

    Not yet enriched from Monarch / HPO

  4. Animal modelNot checked

    Not yet enriched from Monarch / Alliance

  5. Orphan designationNot checked

    FDA/EMA orphan-drug designation not enriched yet

  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)

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?

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=5449) is 41.

2,319 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).

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 27 July 2026

3 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 85.1th percentile).

low confidence · 85.1th 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.

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 2026-07-26

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