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ORPHA:506098

Neuroendocrine carcinoma of pancreas

low confidenceDisorder

Also known as: Pancreatic NEC · Pancreatic neuroendocrine carcinoma · Poorly-differentiated NEN of pancreas · Poorly-differentiated neuroendocrine neoplasm of pancreas · Poorly-differentiated pancreatic NEN · Poorly-differentiated pancreatic neuroendocrine neoplasm

Publications

3,095

Trials

39

Interventional, condition-specific

Researchers

1,265

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare neuroendocrine neoplasm of pancreas characterized by a high-grade malignant epithelial tumor with neuroendocrine differentiation. Based on histopathologic appearance, a small cell (composed of diffuse sheets of cells) and a large cell type (showing a nesting/trabecular pattern) are distinguished. Synaptophysin and chromogranin are positive on immunohistochemistry. The Ki-67 proliferation index is typically very high (>60 - 80%). Patients present with back pain, jaundice, and/or non-specific abdominal symptoms. Serum hormone activity is unusual. The tumor is highly aggressive with poor prognosis.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (24)

Pancreatic Neuroendocrine cancer · carcinoma of endocrine pancreas · carcinoma, islet cell, malignant · endocrine pancreas carcinoma · high grade pancreatic neuroendocrine carcinoma · high-grade pancreatic neuroendocrine carcinoma · islet cell cancer · islet cell carcinoma · islet cell carcinoma (morphologic abnormality) · malignant islet cell tumor · malignant islet cell tumour · malignant neoplasm of islets of Langerhans · malignant pancreatic endocrine tumor · malignant pancreatic endocrine tumour · pancreatic NEC · pancreatic NEC G3 · pancreatic endocrine cancer · pancreatic endocrine carcinoma · pancreatic neuroendocrine carcinoma · poorly differentiated pancreatic endocrine carcinoma · poorly-differentiated NEN of pancreas · poorly-differentiated neuroendocrine neoplasm of pancreas · poorly-differentiated pancreatic NEN · poorly-differentiated pancreatic neuroendocrine neoplasm

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

    3,095 matched papers (1,427 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

    39 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?

3,095

3,095 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.

3,095 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).

1,427 in the last 10 years · low confidence

Phrase hits: 3,091 · MeSH hits: 10

Open Europe PMC search

Who's working on it?

1,265

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
    Zhang J7 papers · 2026

    Thoracic Medical Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas, UK.

    Papers in Europe PMC
  2. 02
    Wang Y6 papers · 2026

    Institute of Future Biophysics, Moscow Center for Advanced Studies, Moscow, 123592, Russia.

    Papers in Europe PMC
  3. 03
    Li J5 papers · 2026

    Department of Radiology, Changhai Hospital, Shanghai, China.

    Papers in Europe PMC
  4. 04
    Li Y5 papers · 2026

    Department of Radiology, Changhai Hospital, Shanghai, China.

    Papers in Europe PMC
  5. 05
    Wang Z5 papers · 2026

    Department of Radiology, The Affiliated Hospital of Nanjing University of Chinese Medicine, 155 Hanzhong Road, Nanjing, 2100029, China.

    Papers in Europe PMC
  6. 06
    Chen X4 papers · 2025

    Department of Radiology, The Affiliated Hospital of Nanjing University of Chinese Medicine, 155 Hanzhong Road, Nanjing, 2100029, China. chxwin@163.com.

    Papers in Europe PMC
  7. 07
    Chen Y4 papers · 2024

    Department of Oncology, Xuhui Central Hospital, Shanghai.

    Papers in Europe PMC
  8. 08
    Guo C4 papers · 2025

    Department of Radiology, The First Affiliated Hospital, College of Medicine, Zhejiang University, 79 Qingchun Road, Hangzhou, 310003, China.

    Papers in Europe PMC
  9. 09
    Li H4 papers · 2026

    Department of Thyroid Surgery, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, 325000, P.R. China.

    Papers in Europe PMC
  10. 10
    Liu J4 papers · 2026

    West China Hospital, Sichuan University, Chengdu, China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

39

interventional trials for this specific condition

39 interventional trials matched this specific condition name; 2 currently recruiting in our sample. 148 trials are registered for neuroendocrine carcinoma, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 27 July 2026

39 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 96.5th percentile).

low confidence · 96.5th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Broader category: neuroendocrine carcinoma

148

Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.

Worth raising with a clinician. How we count trials.

Recruiting under the broader category

Observational and natural-history studies

5 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.

Recruiting or not-yet-recruiting

Open the complete matched 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 2026-07-26

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.

"Neuroendocrine carcinoma of pancreas" OR "Neuroendocrine carcinoma of the pancreas" OR "Pancreatic NEC" OR "Pancreatic neuroendocrine carcinoma" OR "Poorly-differentiated NEN of pancreas" OR "Poorly-differentiated NEN of the pancreas" OR "Poorly-differentiated neuroendocrine neoplasm of pancreas" OR "Poorly-differentiated neuroendocrine neoplasm of the pancreas" OR "Poorly-differentiated pancreatic NEN" OR "Poorly-differentiated pancreatic neuroendocrine neoplasm" OR "Pancreatic Neuroendocrine cancer" OR "carcinoma of endocrine pancreas" OR "carcinoma of the endocrine pancreas" OR "carcinoma, islet cell, malignant" OR "endocrine pancreas carcinoma" OR "high grade pancreatic neuroendocrine carcinoma" OR "high-grade pancreatic neuroendocrine carcinoma" OR "islet cell cancer" OR "islet cell carcinoma" OR "islet cell carcinoma (morphologic abnormality)" OR "malignant islet cell tumor" OR "malignant islet cell tumour" OR "malignant neoplasm of islets of Langerhans" OR "malignant neoplasm of the islets of the Langerhans" OR "malignant pancreatic endocrine tumor" OR "malignant pancreatic endocrine tumour" OR "pancreatic NEC G3" OR "pancreatic endocrine cancer" OR "pancreatic endocrine carcinoma" OR "poorly differentiated pancreatic endocrine carcinoma"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Carcinoma, Islet Cell

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

"Neuroendocrine carcinoma of pancreas" OR "Neuroendocrine carcinoma of the pancreas" OR "Pancreatic NEC" OR "Pancreatic neuroendocrine carcinoma" OR "Poorly-differentiated NEN of pancreas" OR "Poorly-differentiated NEN of the pancreas" OR "Poorly-differentiated neuroendocrine neoplasm of pancreas" OR "Poorly-differentiated neuroendocrine neoplasm of the pancreas" OR "Poorly-differentiated pancreatic NEN" OR "Poorly-differentiated pancreatic neuroendocrine neoplasm" OR "Pancreatic Neuroendocrine cancer" OR "carcinoma of endocrine pancreas" OR "carcinoma of the endocrine pancreas" OR "carcinoma, islet cell, malignant" OR "endocrine pancreas carcinoma" OR "high grade pancreatic neuroendocrine carcinoma" OR "high-grade pancreatic neuroendocrine carcinoma" OR "islet cell cancer" OR "islet cell carcinoma" OR "islet cell carcinoma (morphologic abnormality)" OR "malignant islet cell tumor" OR "malignant islet cell tumour" OR "malignant neoplasm of islets of Langerhans" OR "malignant neoplasm of the islets of the Langerhans" OR "malignant pancreatic endocrine tumor" OR "malignant pancreatic endocrine tumour" OR "pancreatic NEC G3" OR "pancreatic endocrine cancer" OR "pancreatic endocrine carcinoma" OR "poorly differentiated pancreatic endocrine carcinoma" OR "Carcinoma, Islet Cell"

Interventional trials matched via: phrase, mesh (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 39 interventional · 5 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"neuroendocrine carcinoma"

Query health: ok — strategies attempted: phrase, mesh; with hits: phrase, mesh

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 (3095) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity

Ingested 2026-07-27T17:52:19.804Z