Adenocarcinoma of the oesophagus and oesophagogastric junction

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ORPHA:99976OMIM:614266C15.2C15.5
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5FDA treatments17Active trials30Specialists8Treatment centers2Financial resources

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Adenocarcinoma of the oesophagus and oesophagogastric junction is a malignant epithelial tumour arising from the glandular cells lining the lower oesophagus or the junction between the oesophagus and stomach. It is one of the two main histological types of oesophageal cancer (the other being squamous cell carcinoma) and has been increasing in incidence in Western countries over recent decades. The disease primarily affects the digestive system, and its development is strongly associated with chronic gastro-oesophageal reflux disease (GORD) and Barrett's oesophagus, a condition in which the normal squamous epithelium of the lower oesophagus is replaced by intestinal-type columnar epithelium. Other risk factors include obesity, smoking, male sex, and a diet low in fruits and vegetables. Key symptoms include progressive difficulty swallowing (dysphagia), initially for solids and later for liquids, unintentional weight loss, chest or epigastric pain, heartburn, regurgitation, and occasionally gastrointestinal bleeding leading to anaemia. Because early-stage disease is often asymptomatic, diagnosis is frequently made at an advanced stage, which contributes to a generally poor prognosis. Diagnosis is established through upper gastrointestinal endoscopy with biopsy, and staging involves imaging studies such as CT, PET-CT, and endoscopic ultrasound. Treatment depends on the stage at diagnosis. Early-stage tumours confined to the mucosa may be treated with endoscopic resection techniques. Locally advanced disease is typically managed with neoadjuvant chemotherapy or chemoradiotherapy followed by surgical resection (oesophagectomy). Advanced or metastatic disease is treated with palliative systemic therapy, which may include chemotherapy, targeted therapies (such as trastuzumab for HER2-positive tumours and ramucirumab), and immune checkpoint inhibitors (such as nivolumab and pembrolizumab). Multidisciplinary care is essential for optimising outcomes.

Also known as:

Clinical phenotype terms— hover any for plain English:

Clinodactyly of the 5th toeHP:0001864Esophageal carcinomaHP:0011459Barrett esophagusHP:0100580
Inheritance

Multifactorial

Caused by a mix of several genes and environmental factors

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

10 events
Jun 2026A Study of Barrett's Esophagus Patients: Optimization of a Risk Model to Better Predict the Development of Cancer Recurrence and the Effect of Risk Profile Disclosure on Patient Quality of Life and Fear of Cancer

University Hospital, Antwerp — NA

TrialNOT YET RECRUITING
Apr 2026Neoadjuvant Intra-tumoral RP2 and FLOT in Gastroesophageal Adenocarcinoma

Abramson Cancer Center at Penn Medicine — PHASE2

TrialNOT YET RECRUITING
Mar 2026A Study to Evaluate the Safety and Efficacy of Pumitamig in Combination With Chemotherapy Versus Nivolumab in Combination With Chemotherapy in Participants With Previously Untreated Advanced or Metastatic Gastric, Gastroesophageal Junction, or Esophageal Adenocarcinoma (ROSETTA Gastric-204)

Bristol-Myers Squibb — PHASE2, PHASE3

TrialRECRUITING
Feb 2026Givastomig Combined With Nivolumab and Chemotherapy in Adults With CLDN18.2 Positive Metastatic Gastric Cancer (GIVA-2)

I-Mab Biopharma US Limited — PHASE2

TrialRECRUITING
Dec 2025A Phase Ib Trial Evaluating the Safety and Activity of Neoadjuvant Combination of Tislelizumab Plus FLOT for Resectable Esophagogastric Adenocarcinoma

Institut für Klinische Krebsforschung IKF GmbH at Krankenhaus Nordwest — PHASE1

TrialRECRUITING
Dec 2025Phase II RAINSPOT: Zolbetuximab-Paclitaxel-Ramucirumab for CLDN18.2 Positive Gastro-esophageal Cancer

Universitaire Ziekenhuizen KU Leuven — PHASE2

TrialRECRUITING
Dec 2025First-in-Human Study of PLX-61639 in Locally Advanced or Metastatic Solid Tumors

Plexium, Inc. — PHASE1

TrialRECRUITING
Sep 2025Clinical Study of Chemotherapy and Immunotherapy Combined With Lactobacillus Johnsonii in Patients With Multiple Irresectable Solid Tumors

Second Affiliated Hospital, School of Medicine, Zhejiang University — NA

TrialNOT YET RECRUITING
Sep 2025Total Neoadjuvant Therapy for Lymph Node-positive Adenocarcinoma of the OESophagus and Oesophagogastric Junction

Erasmus Medical Center — PHASE2

TrialRECRUITING
Sep 2025Evaluation of Efficacy and Safety De-escalation Versus Standard Adjuvant Chemotherapy in Patients With Low Risk Localized Gastroesophageal Adenocarcinoma

Centre Leon Berard — PHASE2

TrialNOT YET RECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

5 available

Vyloy

zolbetuximab-clzb· Astellas Pharma Global Development Inc.
in combination with fluoropyrimidine- and platinum-containing chemotherapy, is indicated for the first-line treatment of adults with locally advanced unresectable or metastatic human epidermal growth

in combination with fluoropyrimidine- and platinum-containing chemotherapy, is indicated for the first-line treatment of adults with locally advanced unresectable or metastatic human epidermal growth factor receptor 2 (HER2)-negative gastroesophageal junction (GEJ) adenocarcinoma whose tumors are claudin (CLDN) 18.2 positive as determined by an FDA-approved test

Tevimbra

tislelizumab-jsgr· BeOne Medicines USA, Inc.Orphan Drug
in combination with platinum and fluoropyrimidine-based chemotherapy, for the first-line treatment of adults with unresectable or metastatic HER2-negative gastric or gastroesophageal junction adenocar

in combination with platinum and fluoropyrimidine-based chemotherapy, for the first-line treatment of adults with unresectable or metastatic HER2-negative gastric or gastroesophageal junction adenocarcinoma (G/GEJ) whose tumors express PD-L1 (greater than or equal to 1)

Enhertu

fam-trastuzumab deruxtecan-nxki· Daiichi Sankyo, Inc.■ Boxed WarningOrphan Drug

treatment of adult patients with locally advanced or metastatic HER2-positive gastric or gastroesophageal junction (GEJ) adenocarcinoma who have received a prior trastuzumab-based regimen

LONSURF

trifluridine/tipiracil· Taiho Oncology, Inc.Orphan Drug
LONSURF is indicated for the treatment of adult patients with metastatic gastric or gastroesophageal junction adenocarcinoma previously treated with at least two prior lines of chemotherapy that inclu

LONSURF is indicated for the treatment of adult patients with metastatic gastric or gastroesophageal junction adenocarcinoma previously treated with at least two prior lines of chemotherapy that included a fluoropyrimidine, a platinum, either a taxane or irinotecan, and if appropriate, HER2/neu-targeted therapy.

Herceptin

trastuzumab· Genentech, Inc.■ Boxed WarningOrphan Drug

Treatment of patients with HER2 overexpressing metastatic gastric or gastroesophageal junction adenocarcinoma, who have not received prior treatment for metastatic disease

Clinical Trials

17 recruitingView all trials with filters →
Phase 27 trials
Givastomig Combined With Nivolumab and Chemotherapy in Adults With CLDN18.2 Positive Metastatic Gastric Cancer (GIVA-2)
Phase 2
Actively Recruiting
· Sites: Goodyear, Arizona; Duarte, California +1 more · Age: 1899 yrs
Adjuvant Trastuzumab Deruxtecan (Enhertu) & Nivolumab For Patients Who Are Disease Free After Completion of Trimodality Treatment For HER-2+ Cancers of Esophagus & Gastroesophageal Junction
Phase 2
Active
PI: Howard Safran, MD (Lifespan Cancer Institute) · Sites: New York, New York; Providence, Rhode Island · Age: 1899 yrs
A Phase II Trial to Evaluate the Effect of Itraconazole on Pathologic Complete Response Rates in Resectable Esophageal Cancer
Phase 2
Actively Recruiting
PI: David H Wang, MD PhD (VA Ann Arbor Healthcare System, Ann Arbor, MI) · Sites: Palo Alto, California; Ann Arbor, Michigan +5 more · Age: 1899 yrs
Total Neoadjuvant Therapy for Lymph Node-positive Adenocarcinoma of the OESophagus and Oesophagogastric Junction
Phase 2
Actively Recruiting
PI: Bianca Mostert, MD, PhD (Erasmus Medical Centre) · Sites: Eindhoven, North Brabant; Leeuwarden, North Brabant +8 more · Age: 1899 yrs
Chemoradiation and Pembrolizumab Followed by Pembrolizumab and Lenvatinib Before Surgery for the Treatment of Non-metastatic Esophageal or Esophageal/Gastroesophageal Junction Cancer
Phase 2
Active
PI: Vincent Chung, MD (City of Hope Medical Center) · Sites: Duarte, California · Age: 1899 yrs
Perioperative mFOLFOX Plus Pembrolizumab in Gastroesophageal Junction (GEJ) and Stomach Adenocarcinoma
Phase 2
Active
PI: Weijing Sun, MD, FACP (The University of Kansas - Cancer Center) · Sites: Westwood, Kansas · Age: 1875 yrs
A Study to Evaluate the Adverse Events, Efficacy, and Optimal Dose of Intravenous (IV) ABBV-400 in Combination With IV Fluorouracil, Leucovorin, and Budigalimab in Adult Participants With Locally Advanced Unresectable or Metastatic Gastric, Gastroesophageal Junction, or Esophageal Adenocarcinoma
Phase 2
Actively Recruiting
PI: ABBVIE INC. (AbbVie) · Sites: Duarte, California; Irvine, California +47 more · Age: 1899 yrs
Phase 13 trials
A Phase Ib Trial Evaluating the Safety and Activity of Neoadjuvant Combination of Tislelizumab Plus FLOT for Resectable Esophagogastric Adenocarcinoma
Phase 1
Actively Recruiting
PI: Florian Lordick, Prof. Dr. (Universitätsklinikum Leipzig) · Sites: Frankfurt; Hamburg +2 more · Age: 1899 yrs
First-in-Human Study of PLX-61639 in Locally Advanced or Metastatic Solid Tumors
Phase 1
Actively Recruiting
PI: Chief Medical Officer (Plexium, Inc.) · Sites: Scottsdale, Arizona; Duarte, California +8 more · Age: 1899 yrs
A Study of KK2269 in Adult Participants With Solid Tumors
Phase 1
Actively Recruiting
· Sites: Scottsdale, Arizona; Duarte, California +9 more · Age: 1899 yrs
N/A1 trial
Evaluation, in Humans, of the Correlation Between Hepatotoxicity, Neurotoxicity Induced by Oxaliplatin, and Blood Levels of HMGB1
N/A
Actively Recruiting
PI: Marine JARY, MD (CHU Estaing de Clermont Ferrand/FRANCE) · Sites: Clermont-Ferrand · Age: 1899 yrs
Other1 trial
Registry Platform Gastric/Esophageal Cancer (SAPHIR)
Actively Recruiting
PI: Tobias Dechow, Prof. (Ravensburg) · Sites: Multiple Locations · Age: 1899 yrs

Specialists

Showing 25 of 30View all specialists →
GL
Global Clinical Leader
Specialist
PI on 44 active trials4 Adenocarcinoma of the oesophagus and oesophagogastric junction publications
SM
Syma Iqbal, MD
LOS ANGELES, CA
Specialist
PI on 3 active trials
ED
Executive Director
Specialist
PI on 2 active trials
VM
Vincent Chung, MD
DUARTE, CA
Specialist
PI on 4 active trials
MC
Michael Cecchini
BEL AIR, MD
Specialist
PI on 2 active trials10 Adenocarcinoma of the oesophagus and oesophagogastric junction publications
AK
Andreas Kaubisch
BRONX, NY
Specialist
PI on 2 active trials
RR
Ruddy RICHARD
Specialist
PI on 1 active trial
HS
Howard P Safran
PROVIDENCE, RI
Specialist
PI on 1 active trial
KS
Kohei Shitara
Specialist
PI on 1 active trial
JM
JoAl Mayor
SAN FRANCISCO, CA
Specialist
PI on 1 active trial
DW
David H Wang
Specialist
PI on 1 active trial
TD
Tobias Dechow
Specialist
PI on 1 active trial
BM
Bianca Mostert
Specialist
PI on 1 active trial
JA
Jaffer Ajani
HOUSTON, TX
Specialist
PI on 3 active trials
DA
David Adelstein
CLEVELAND, OH
Specialist
PI on 1 active trial
JE
Jennifer R Eads
PHILADELPHIA, PA
Specialist
PI on 1 active trial
WS
Weijing Sun
WESTWOOD, KS
Specialist
PI on 1 active trial
GK
Geoffrey Ku
NEW YORK, NY
Specialist
PI on 1 active trial
FL
Florian Lordick
Specialist
PI on 2 active trials
MM
Michael McNamara
Specialist
PI on 2 active trials
WC
William Chapin
PHILADELPHIA, PA
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Financial Resources

2 resources
Enhertu(fam-trastuzumab deruxtecan-nxki)Daiichi Sankyo, Inc.
Herceptin(trastuzumab)Genentech, Inc.

Travel Grants

No travel grants are currently matched to Adenocarcinoma of the oesophagus and oesophagogastric junction.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Adenocarcinoma of the oesophagus and oesophagogastric junction

Disease timeline:

New recruiting trial: Folfox+Irinotecan+Chemort In Esophageal Cancer

A new clinical trial is recruiting patients for Adenocarcinoma of the oesophagus and oesophagogastric junction

New recruiting trial: Adding Nivolumab to Usual Treatment for People With Advanced Stomach or Esophageal Cancer, PARAMUNE Trial

A new clinical trial is recruiting patients for Adenocarcinoma of the oesophagus and oesophagogastric junction

New recruiting trial: A Multicenter Cancer Biospecimen Collection Study

A new clinical trial is recruiting patients for Adenocarcinoma of the oesophagus and oesophagogastric junction

New recruiting trial: Substudy 06D: Combination Therapies in Second Line (2L) Gastroesophageal Adenocarcinoma (MK-3475-06D/Keymaker-U06)

A new clinical trial is recruiting patients for Adenocarcinoma of the oesophagus and oesophagogastric junction

New recruiting trial: Relationship Between Perioperative Treatment Efficacy and The Tumor Microenvironment for Locally Advanced Resectable Gastric Cancer.

A new clinical trial is recruiting patients for Adenocarcinoma of the oesophagus and oesophagogastric junction

New recruiting trial: A Randomized, Multicenter Phase II Basket Study of Hypofractionated Radiotherapy/Stereotactic Body Radiotherapy Followed by Immunotherapy-Based Systemic Therapy +/- L. Rhamnosus M9 for the First-Line Treatment of Advanced Digestive System Malignancies.

A new clinical trial is recruiting patients for Adenocarcinoma of the oesophagus and oesophagogastric junction

New recruiting trial: Collecting Blood Samples From Patients With and Without Cancer to Evaluate Tests for Early Cancer Detection

A new clinical trial is recruiting patients for Adenocarcinoma of the oesophagus and oesophagogastric junction

New recruiting trial: Phase II RAINSPOT: Zolbetuximab-Paclitaxel-Ramucirumab for CLDN18.2 Positive Gastro-esophageal Cancer

A new clinical trial is recruiting patients for Adenocarcinoma of the oesophagus and oesophagogastric junction

New recruiting trial: Camrelizumab Combined With Chemotherapy and Radiotherapy Perioperative Treatment of Esophageal Gastric Junction Adenocarcinoma

A new clinical trial is recruiting patients for Adenocarcinoma of the oesophagus and oesophagogastric junction

New recruiting trial: Registry Platform Gastric/Esophageal Cancer (SAPHIR)

A new clinical trial is recruiting patients for Adenocarcinoma of the oesophagus and oesophagogastric junction

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Common questions about Adenocarcinoma of the oesophagus and oesophagogastric junction

What is Adenocarcinoma of the oesophagus and oesophagogastric junction?

Adenocarcinoma of the oesophagus and oesophagogastric junction is a malignant epithelial tumour arising from the glandular cells lining the lower oesophagus or the junction between the oesophagus and stomach. It is one of the two main histological types of oesophageal cancer (the other being squamous cell carcinoma) and has been increasing in incidence in Western countries over recent decades. The disease primarily affects the digestive system, and its development is strongly associated with chronic gastro-oesophageal reflux disease (GORD) and Barrett's oesophagus, a condition in which the nor

How is Adenocarcinoma of the oesophagus and oesophagogastric junction inherited?

Adenocarcinoma of the oesophagus and oesophagogastric junction follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Adenocarcinoma of the oesophagus and oesophagogastric junction typically begin?

Typical onset of Adenocarcinoma of the oesophagus and oesophagogastric junction is adult. Age of onset can vary across affected individuals.

Are there clinical trials for Adenocarcinoma of the oesophagus and oesophagogastric junction?

Yes — 17 recruiting clinical trials are currently listed for Adenocarcinoma of the oesophagus and oesophagogastric junction on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Adenocarcinoma of the oesophagus and oesophagogastric junction?

25 specialists and care centers treating Adenocarcinoma of the oesophagus and oesophagogastric junction are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.

What treatment and support options exist for Adenocarcinoma of the oesophagus and oesophagogastric junction?

4 patient support programs are currently tracked on UniteRare for Adenocarcinoma of the oesophagus and oesophagogastric junction. See the treatments and support programs sections for copay assistance, eligibility, and contact details.