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ORPHA:104075

Adenocarcinoma of the small intestine

high confidenceDisorder

Also known as: Adenocarcinoma of the small bowel

Publications

2,987

89.7th percentile

Trials

26

Interventional, condition-specific

Researchers

1,263

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

Small bowel adenocarcinoma (SBA) is a rare small intestinal malignancy, most commonly located in the duodenum (55% of cases) but also rarely in the jejunum and ileum, which is usually discovered at an advanced stage in the 6th to 7th decade of life due to non-specific symptoms at presentation such as nausea, abdominal pain and weight loss. In some cases it is asymptomatic, and therefore usually has a poor prognosis.

How rare: 1-9 / 1 000 000 — roughly one to nine people per million. In a city the size of Kolkata, perhaps a few dozen.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (8)

adenocarcinoma - small intest. · adenocarcinoma of small bowel · adenocarcinoma of small intestine · adenocarcinoma of the small bowel · adenocarcinoma of the small intestine · small bowel adenocarcinoma · small intestinal adenocarcinoma · small intestine adenocarcinoma

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

    2,987 matched papers (1,629 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

    26 matched on ClinicalTrials.gov (9 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 2026-07-27

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

23

Drugs / clinical candidates · MONDO_0003198

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

2,987

2,987 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.

2,987 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).

1,629 in the last 10 years · high confidence · 89.7th percentile (publications denominator)

Phrase hits: 2,987 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,263

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
    Vanoli A9 papers · 2026

    Department of Molecular Medicine, Unit of Anatomic Pathology, University of Pavia, Pavia 27100, Italy; Unit of Anatomic Pathology, Fondazione IRCCS San Matteo Hospital, Pavia 27100, Italy.

    Papers in Europe PMC
  2. 02
    Di Sabatino A8 papers · 2026

    Department of Internal Medicine, University of Pavia, San Matteo Hospital Foundation, Pavia 27100, Italy. Electronic address: a.disabatino@smatteo.pv.it.

    Papers in Europe PMC
  3. 03
    Arpa G7 papers · 2025

    Department of Molecular Medicine, Unit of Anatomic Pathology, University of Pavia, Pavia 27100, Italy.

    Papers in Europe PMC
  4. 04
    Lenti MV7 papers · 2026

    Department of Internal Medicine, University of Pavia, San Matteo Hospital Foundation, Pavia 27100, Italy.

    Papers in Europe PMC
  5. 05
    Quaquarini E6 papers · 2026

    Medical Oncology Unit, ICS Maugeri-IRCCS SpA SB, Pavia 27100, Italy.

    Papers in Europe PMC
  6. 06
    Guerini C5 papers · 2026

    Department of Molecular Medicine, Unit of Anatomic Pathology, University of Pavia, Pavia 27100, Italy.

    Papers in Europe PMC
  7. 07
    Paulli M5 papers · 2026

    Department of Molecular Medicine, Unit of Anatomic Pathology, University of Pavia, Pavia 27100, Italy; Unit of Anatomic Pathology, Fondazione IRCCS San Matteo Hospital, Pavia 27100, Italy.

    Papers in Europe PMC
  8. 08
    Wang H5 papers · 2026

    Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai 200032, China.

    Papers in Europe PMC
  9. 09
    Antoci F4 papers · 2026

    Unit of Anatomic Pathology, IRCCS San Matteo Hospital Foundation, Pavia, Italy.

    Papers in Europe PMC
  10. 10
    De Lisi G4 papers · 2026

    Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia, Pavia, Italy.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

26

interventional trials for this specific condition

26 interventional trials matched this specific condition name; 9 currently recruiting in our sample. 3,666 trials are registered for adenocarcinoma, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 9 September 2026

26 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 95.6th percentile).

high confidence · 95.6th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Broader category: adenocarcinoma

3,666

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

6 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

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 38 · after dedupe 38 · already on CT.gov 0 · kept 1 · parent 0 · uncertain 3 · dropped 34 · fetched 2026-07-27

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

Uncertain / not reviewed (3)

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 Adenocarcinoma of the small intestine — 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.

"Adenocarcinoma of the small intestine" OR "Adenocarcinoma of the small bowel" OR "adenocarcinoma - small intest." OR "adenocarcinoma of small bowel" OR "adenocarcinoma of small intestine" OR "small bowel adenocarcinoma" OR "small intestinal adenocarcinoma" OR "small intestine adenocarcinoma"

Run this search on Europe PMC

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

"Adenocarcinoma of the small intestine" OR "Adenocarcinoma of small intestine" OR "Adenocarcinoma of the small bowel" OR "Adenocarcinoma of small bowel" OR "adenocarcinoma - small intest." OR "small bowel adenocarcinoma" OR "small intestinal adenocarcinoma" OR "small intestine adenocarcinoma"

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: 26 interventional · 6 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"adenocarcinoma"

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

Ingested 2026-07-26T02:34:40.002Z