Overview
Rare epithelial tumors of the pancreas are an uncommon group of growths that start in the lining cells (epithelial cells) of the pancreas — the organ behind your stomach that helps with digestion and controls blood sugar. This broad category includes several distinct tumor types that do not fit neatly into the more common pancreatic cancers like pancreatic ductal adenocarcinoma. Examples in this group may include acinar cell carcinoma, pancreatoblastoma, solid pseudopapillary neoplasm (also called Frantz tumor or solid pseudopapillary tumor), and other rare epithelial variants. Because the pancreas has both digestive (exocrine) and hormone-producing (endocrine) functions, tumors in this category can disrupt either or both systems depending on where they arise. Symptoms often develop slowly and can be vague at first, which makes early diagnosis challenging. People may notice abdominal pain or discomfort, unexplained weight loss, nausea, or changes in digestion. Some tumors are found accidentally during imaging done for another reason. The behavior of these tumors varies widely — some grow slowly and can be cured with surgery, while others are more aggressive and require additional treatments like chemotherapy. Treatment depends heavily on the specific tumor type, its size, and whether it has spread. Surgery to remove the tumor is the main treatment when possible. Some types respond to chemotherapy or targeted therapies. Because these tumors are so rare, care is best managed at specialized cancer centers with experience in pancreatic diseases. Research is ongoing, and clinical trials may offer additional options for some patients.
Also known as:
Key symptoms:
Dull or aching pain in the upper abdomen or backUnexplained weight lossNausea or vomitingFeeling full quickly when eatingYellowing of the skin or eyes (jaundice)Pale or greasy stoolsDark urineNew or worsening diabetes or blood sugar problemsA lump or mass felt in the abdomenFatigue and low energyLoss of appetiteDigestive problems or diarrhea
Variable
Can be inherited in different ways depending on the underlying gene
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Rare epithelial tumor of pancreas.
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Rare Disease Specialist
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 Rare epithelial tumor of pancreas.
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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 type of rare epithelial pancreatic tumor I have, and how does that affect my treatment options?,Has my tumor been tested for genetic or molecular changes that could guide treatment?,Is surgery an option for me, and what type of surgery would be recommended?,Should I be tested for a hereditary cancer syndrome that might affect my family members?,Are there any clinical trials I might be eligible for?,What supportive treatments are available to help with digestion and blood sugar if my pancreas is affected?,How often will I need follow-up imaging, and what signs of recurrence should I watch for?
Common questions about Rare epithelial tumor of pancreas
What is Rare epithelial tumor of pancreas?
Rare epithelial tumors of the pancreas are an uncommon group of growths that start in the lining cells (epithelial cells) of the pancreas — the organ behind your stomach that helps with digestion and controls blood sugar. This broad category includes several distinct tumor types that do not fit neatly into the more common pancreatic cancers like pancreatic ductal adenocarcinoma. Examples in this group may include acinar cell carcinoma, pancreatoblastoma, solid pseudopapillary neoplasm (also called Frantz tumor or solid pseudopapillary tumor), and other rare epithelial variants. Because the pan
Which specialists treat Rare epithelial tumor of pancreas?
3 specialists and care centers treating Rare epithelial tumor of pancreas are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.