Overview
Epithelial tumors of the appendix are a group of rare growths that start in the lining cells of the appendix — a small, finger-shaped pouch attached to the large intestine in the lower right side of the belly. These tumors are sometimes called appendiceal neoplasms or appendix cancers. They include several types, such as low-grade and high-grade mucinous neoplasms (also called LAMN and HAMN), mucinous adenocarcinoma, and non-mucinous adenocarcinoma. Some types produce a jelly-like substance called mucin, which can build up inside the belly if the tumor ruptures — a serious condition known as pseudomyxoma peritonei (PMP). Many people with an epithelial tumor of the appendix have no symptoms at first. The tumor is often found by accident during surgery for appendicitis or during imaging done for another reason. When symptoms do appear, they can include belly pain, bloating, or a change in bowel habits. Because the appendix is small and symptoms are vague, diagnosis is often delayed. Treatment depends on the type and stage of the tumor. Surgery is the main treatment and may range from simple removal of the appendix to more extensive operations. For tumors that have spread into the belly, a specialized procedure called cytoreductive surgery combined with heated chemotherapy washed directly into the abdomen (HIPEC) may be recommended. Early-stage tumors often have a very good outlook, while more advanced cases require more aggressive treatment.
Also known as:
Key symptoms:
Dull or crampy pain in the lower right bellyBloating or a feeling of fullness in the abdomenIncreasing waist size without weight gainChange in bowel habits such as constipation or diarrheaNausea or vomitingNew hernia in the groin or belly button areaFeeling of pressure or heaviness in the pelvisUnexplained weight lossSymptoms that look like appendicitis (sudden sharp right-sided belly pain)Ovarian mass found on imaging in women
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
10 eventsAerospace Center Hospital — NA
University of Southampton — PHASE3
Fondazione IRCCS Istituto Nazionale dei Tumori, Milano — PHASE2
M.D. Anderson Cancer Center — PHASE1, PHASE2
M.D. Anderson Cancer Center — NA
Laval University — NA
Institut du Cancer de Montpellier - Val d'Aurelle — NA
Cybrexa Therapeutics — PHASE1, PHASE2
M.D. Anderson Cancer Center — PHASE2
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Epithelial tumor of the appendix.
11 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →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
Travel Grants
No travel grants are currently matched to Epithelial tumor of the appendix.
Community
No community posts yet. Be the first to share your experience with Epithelial tumor of the appendix.
Start the conversation →Latest news about Epithelial tumor of the appendix
Disease timeline:
New recruiting trial: A Phase I/II Study of Intraperitoneal Paclitaxel in Patients With Metastatic Appendiceal Adenocarcinoma
A new clinical trial is recruiting patients for Epithelial tumor of the appendix
New recruiting trial: A Prospective Feasibility Study Using ctDNA to Tailor Neoadjuvant Chemotherapy for Patients With Colorectal or Appendiceal Adenocarcinoma
A new clinical trial is recruiting patients for Epithelial tumor of the appendix
New recruiting trial: Study to Evaluate the Non-inferiority of Low-dose HIPEC Versus High-dose HIPEC in the Treatment of PMP (HIPEC-PMP)
A new clinical trial is recruiting patients for Epithelial tumor of the appendix
New recruiting trial: Locally ablatIVe thErapy for oLigo-progressive gastrOintestiNal maliGnancies (LIVELONG)
A new clinical trial is recruiting patients for Epithelial tumor of the appendix
New recruiting trial: Genetics of Appendix Cancer Study
A new clinical trial is recruiting patients for Epithelial tumor of the appendix
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.
Questions for your doctor
Bring these to your next appointment
- Q1.What exact type of epithelial tumor do I have, and what does the grade mean for my treatment and outlook?,Has the tumor spread beyond the appendix, and do I need additional surgery or chemotherapy?,Should I be referred to a center that specializes in cytoreductive surgery and HIPEC?,Should my tumor tissue be tested for specific genetic mutations that might affect my treatment options?,Are there any clinical trials I might be eligible for?,How often do I need follow-up scans and blood tests, and what are we watching for?,Should my family members be tested for hereditary cancer syndromes given my diagnosis?
Common questions about Epithelial tumor of the appendix
What is Epithelial tumor of the appendix?
Epithelial tumors of the appendix are a group of rare growths that start in the lining cells of the appendix — a small, finger-shaped pouch attached to the large intestine in the lower right side of the belly. These tumors are sometimes called appendiceal neoplasms or appendix cancers. They include several types, such as low-grade and high-grade mucinous neoplasms (also called LAMN and HAMN), mucinous adenocarcinoma, and non-mucinous adenocarcinoma. Some types produce a jelly-like substance called mucin, which can build up inside the belly if the tumor ruptures — a serious condition known as p
How is Epithelial tumor of the appendix inherited?
Epithelial tumor of the appendix follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Epithelial tumor of the appendix typically begin?
Typical onset of Epithelial tumor of the appendix is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Epithelial tumor of the appendix?
Yes — 11 recruiting clinical trials are currently listed for Epithelial tumor of the appendix on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Epithelial tumor of the appendix?
19 specialists and care centers treating Epithelial tumor of the appendix are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.