Overview
Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) is a very rare inherited condition that causes hundreds of polyps (small growths) to develop in the upper part of the stomach, specifically in the fundic gland area. These polyps are found in the body and fundus of the stomach but spare the lower part (antrum) and do not typically involve the colon or rectum, which helps distinguish GAPPS from other polyposis syndromes. Over time, some of these stomach polyps can become cancerous, leading to gastric adenocarcinoma — a type of stomach cancer. GAPPS was first described in 2012 and is caused by specific mutations in the promoter region of the APC gene, which is well known for its role in other polyposis conditions like familial adenomatous polyposis (FAP). Because of the significant risk of developing stomach cancer, people diagnosed with GAPPS require close monitoring through regular upper endoscopy (a camera examination of the stomach). In many cases, doctors may recommend a prophylactic total gastrectomy, which is the surgical removal of the entire stomach, to prevent cancer from developing. This is currently the most effective way to eliminate the cancer risk. After surgery, patients can still eat but must make significant dietary adjustments. Early detection through genetic testing of at-risk family members is very important, as catching the condition before cancer develops greatly improves outcomes. There are currently no medications that can prevent the polyps from forming or progressing to cancer.
Also known as:
Key symptoms:
Hundreds of polyps in the upper part of the stomachStomach pain or discomfortNauseaFeeling full quickly after eatingUnexplained weight lossAnemia (low red blood cell count)Fatigue or tirednessVomitingLoss of appetiteBleeding in the stomach (which may cause dark stools)Stomach cancer if polyps are not monitored or removed
Autosomal dominant
Passed on from just one parent; each child has about a 50% chance of inheriting it
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Gastric adenocarcinoma and proximal polyposis of the stomach.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Financial Resources
1 resourcesVYLOY
Astellas
Stomach Cancer
Travel Grants
No travel grants are currently matched to Gastric adenocarcinoma and proximal polyposis of the stomach.
Community
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1 articlesCaregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
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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.
Questions for your doctor
Bring these to your next appointment
- Q1.What is my personal risk of developing stomach cancer based on my specific mutation and family history?,How often should I have upper endoscopy surveillance, and what are you looking for during each exam?,At what point would you recommend preventive removal of my stomach, and what does that surgery involve?,Should my family members be tested for this genetic mutation, and at what age should testing begin?,What nutritional supplements and dietary changes will I need if I have my stomach removed?,Are there any clinical trials or new treatments being studied for GAPPS?,How will this condition affect my ability to work, exercise, and live a normal life long-term?
Common questions about Gastric adenocarcinoma and proximal polyposis of the stomach
What is Gastric adenocarcinoma and proximal polyposis of the stomach?
Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) is a very rare inherited condition that causes hundreds of polyps (small growths) to develop in the upper part of the stomach, specifically in the fundic gland area. These polyps are found in the body and fundus of the stomach but spare the lower part (antrum) and do not typically involve the colon or rectum, which helps distinguish GAPPS from other polyposis syndromes. Over time, some of these stomach polyps can become cancerous, leading to gastric adenocarcinoma — a type of stomach cancer. GAPPS was first described in 2012
How is Gastric adenocarcinoma and proximal polyposis of the stomach inherited?
Gastric adenocarcinoma and proximal polyposis of the stomach follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Gastric adenocarcinoma and proximal polyposis of the stomach typically begin?
Typical onset of Gastric adenocarcinoma and proximal polyposis of the stomach is adult. Age of onset can vary across affected individuals.
Which specialists treat Gastric adenocarcinoma and proximal polyposis of the stomach?
16 specialists and care centers treating Gastric adenocarcinoma and proximal polyposis of the stomach are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.
What treatment and support options exist for Gastric adenocarcinoma and proximal polyposis of the stomach?
1 patient support program are currently tracked on UniteRare for Gastric adenocarcinoma and proximal polyposis of the stomach. See the treatments and support programs sections for copay assistance, eligibility, and contact details.