ORPHA:104075
Adenocarcinoma of the small intestine
Also known as: Adenocarcinoma of the small bowel
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.
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=183) is 38.
2,987 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).
1,629 in the last 10 years · high confidence · 96.4th percentile (publications denominator)
Is a treatment being tested?
101
trials for this specific condition
101 interventional trials matched this specific condition name; 12 currently recruiting in our sample. 3,665 trials are registered for adenocarcinoma, the broader category — shown separately because they may or may not enrol this specific subtype.
Data as of 26 July 2026
3,665
trials for adenocarcinoma, the broader category this belongs to
Trials registered for a broader category may or may not enrol people with this specific subtype — eligibility criteria vary, and the trial record often doesn't say. Worth raising with a clinician. How we count trials.
101 interventional trials — more than 59.2% of diseases in the trials denominator have none at all (151 of 255; this disease is at the 97.1th percentile).
high confidence · 97.1th 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?
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.
- 01Vanoli 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 - 02Di 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 - 03Arpa G7 papers · 2025
Department of Molecular Medicine, Unit of Anatomic Pathology, University of Pavia, Pavia 27100, Italy.
Papers in Europe PMC - 04Lenti MV7 papers · 2026
Department of Internal Medicine, University of Pavia, San Matteo Hospital Foundation, Pavia 27100, Italy.
Papers in Europe PMC - 05Quaquarini E6 papers · 2026
Medical Oncology Unit, ICS Maugeri-IRCCS SpA SB, Pavia 27100, Italy.
Papers in Europe PMC - 06Guerini C5 papers · 2026
Department of Molecular Medicine, Unit of Anatomic Pathology, University of Pavia, Pavia 27100, Italy.
Papers in Europe PMC - 07Paulli 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 - 08Wang H5 papers · 2026
Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai 200032, China.
Papers in Europe PMC - 09Antoci F4 papers · 2026
Unit of Anatomic Pathology, IRCCS San Matteo Hospital Foundation, Pavia, Italy.
Papers in Europe PMC - 10De Lisi G4 papers · 2026
Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia, Pavia, Italy.
Papers in Europe PMC
Recruiting interventional trials
Trials testing a treatment from the matched ClinicalTrials.gov set
101 interventional trials matched after quoted-phrase search and title/condition post-filter.
- NCT04907643·Virtual Reality for GI Cancer Pain to Improve Patient Reported Outcomes
- NCT07720258·Sacituzumab Tirumotecan (MK-2870) in Patients With Advanced Small Bowel Adenocarcinoma Refractory or Intolerant to Platinum-Based Combination Therapy
- NCT07347314·Enfortumab Vedotin in Patients With Advanced Small Bowel Adenocarcinoma Refractory or Intolerant to Platinum-based Combination Therapy
- NCT06835387·Study of NALIRIFOX in Advanced Unresectable Small Bowel Tumors
- NCT07417488·GUCY2C Prime-Boost Vaccination for Advanced Colorectal and Small Bowel Adenocarcinomas
- NCT06333314·Dostarlimab for Locally Advanced or Metastatic Cancer (Non-colorectal/Non-endometrial) With Tumor dMMR/MSI
- NCT05472948·Surufatinib and Sintilimab in Combination With Capecitabine for Metastatic Adenocarcinoma of Small Intestine or Appendix Carcinoma
- NCT06278545·Trial Evaluating Modified FOLFIRINOX and Modified FOLFOX in the Treatment of Locally Advanced or Metastatic Small Bowel Adenocarcinoma
- NCT04205968·Ramucirumab and Paclitaxel or FOLFIRI in Advanced Small Bowel Cancers
- NCT07060014·NALIRIFOX (Nal-IRI Plus 5-FU/LV Plus Oxaliplatin) as First-Line Treatment for Patients With Advanced Small Intestine and Appendiceal Cancers
- NCT05704010·Videocapsule Endoscopy in Lynch Syndrome
- NCT06715839·Target-specific immunoPET Imaging of Digestive System Carcinoma
Observational and natural-history studies
15 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
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.
"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"
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" OR "small intestine carcinoma" OR "small intestine cancer"
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: 101 interventional · 15 observational · 0 expanded access. Only interventional studies enter the trial headline.
Cross-references (from Mondo): UMLS:C0278803 NCIT:C7888
Query health: ok — strategies attempted: phrase, recall-expansion; with hits: phrase, recall-expansion
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