Overview
Mucinous adenocarcinoma of the appendix is a rare type of cancer that starts in the appendix, a small pouch attached to the large intestine. This cancer is called "mucinous" because the tumor cells produce a thick, jelly-like substance called mucin. It is sometimes referred to as appendiceal mucinous adenocarcinoma or mucinous appendiceal cancer. Unlike more common cancers, this type can behave in unusual ways — it may spread mucin-producing cells throughout the abdominal cavity, a condition known as pseudomyxoma peritonei (PMP), where jelly-like material fills the belly. Many people have no symptoms early on. The disease is often discovered unexpectedly during surgery for what appears to be appendicitis or during imaging done for other reasons. When symptoms do occur, they may include abdominal pain (often in the lower right side), bloating, a growing waistline without weight gain elsewhere, changes in bowel habits, or a mass felt in the abdomen. Some patients experience nausea, loss of appetite, or pelvic pressure. Treatment typically involves surgery to remove the appendix and surrounding tissue. In more advanced cases, especially when the cancer has spread within the abdomen, a specialized procedure called cytoreductive surgery (CRS) combined with heated intraperitoneal chemotherapy (HIPEC) may be recommended. This involves removing all visible tumor from the abdominal cavity and then bathing the area in heated chemotherapy. Systemic chemotherapy may also be used. Because this cancer is rare, treatment is best managed at specialized centers with experience in peritoneal surface cancers.
Also known as:
Key symptoms:
Abdominal pain, often in the lower right sideAbdominal bloating or swellingIncreasing waist size without overall weight gainA lump or mass felt in the abdomenChanges in bowel habits such as constipation or diarrheaNausea or vomitingLoss of appetiteUnexplained weight lossPelvic pressure or discomfortSymptoms mimicking appendicitisFeeling full quickly when eatingFatigueNew onset hernia
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
3 eventsPeking University — PHASE1
University Hospital, Akershus — PHASE2
M.D. Anderson Cancer Center — NA
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
1 availableIMFINZI
in combination with fluorouracil, leucovorin, oxaliplatin, and docetaxel (FLOT) chemotherapy as neoadjuvant and adjuvant treatment, followed by single agent IMFINZI, for the treatment of adult patient…
in combination with fluorouracil, leucovorin, oxaliplatin, and docetaxel (FLOT) chemotherapy as neoadjuvant and adjuvant treatment, followed by single agent IMFINZI, for the treatment of adult patients with resectable gastric or gastroesophageal junction adenocarcinoma (GC/GEJC)
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 resourcesTravel Grants
No travel grants are currently matched to Mucinous adenocarcinoma of the appendix.
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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 is the exact grade and stage of my appendiceal cancer, and what does that mean for my outlook?,Am I a candidate for cytoreductive surgery with HIPEC, and should I be referred to a specialized center?,What chemotherapy options are available for my specific situation, and what are the expected side effects?,Should molecular or genetic testing be done on my tumor to guide treatment decisions?,How often will I need follow-up imaging and blood tests, and what will you be looking for?,Are there any clinical trials available for appendiceal mucinous adenocarcinoma that I might be eligible for?,What symptoms should prompt me to seek emergency care between appointments?
Common questions about Mucinous adenocarcinoma of the appendix
What is Mucinous adenocarcinoma of the appendix?
Mucinous adenocarcinoma of the appendix is a rare type of cancer that starts in the appendix, a small pouch attached to the large intestine. This cancer is called "mucinous" because the tumor cells produce a thick, jelly-like substance called mucin. It is sometimes referred to as appendiceal mucinous adenocarcinoma or mucinous appendiceal cancer. Unlike more common cancers, this type can behave in unusual ways — it may spread mucin-producing cells throughout the abdominal cavity, a condition known as pseudomyxoma peritonei (PMP), where jelly-like material fills the belly. Many people have no
How is Mucinous adenocarcinoma of the appendix inherited?
Mucinous adenocarcinoma of the appendix follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Mucinous adenocarcinoma of the appendix typically begin?
Typical onset of Mucinous adenocarcinoma of the appendix is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Mucinous adenocarcinoma of the appendix?
Yes — 2 recruiting clinical trials are currently listed for Mucinous adenocarcinoma 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 Mucinous adenocarcinoma of the appendix?
25 specialists and care centers treating Mucinous adenocarcinoma of the appendix are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.