Overview
Acinar cell carcinoma of the pancreas (ACC) is a rare type of pancreatic cancer that starts in the acinar cells — the cells that normally make digestive enzymes in the pancreas. It is sometimes called pancreatic acinar cell carcinoma or ACC of the pancreas. Unlike the more common type of pancreatic cancer (called pancreatic ductal adenocarcinoma), ACC has some different features and may respond differently to treatment. The pancreas sits behind the stomach and has two main jobs: making digestive juices (enzymes) and controlling blood sugar with hormones like insulin. When acinar cells become cancerous, they can form a tumor that grows and may spread to nearby organs, lymph nodes, or distant parts of the body like the liver. In some cases, the tumor releases too many digestive enzymes into the bloodstream, causing a unique set of symptoms including joint pain, skin rashes, and fatty tissue breakdown under the skin — a condition sometimes called lipase hypersecretion syndrome. Common symptoms include belly pain, unexplained weight loss, nausea, and sometimes a lump in the abdomen. Treatment usually involves surgery when the tumor can be removed, along with chemotherapy and sometimes radiation. Because ACC is rare, treatment is often guided by cancer centers with experience in rare pancreatic tumors. Compared to the most common pancreatic cancer, ACC may have a somewhat better outlook, especially when caught early.
Also known as:
Key symptoms:
Abdominal or belly pain, often in the upper or middle abdomenUnexplained weight lossNausea and vomitingLoss of appetiteA lump or mass felt in the abdomenFatigue and general weaknessJaundice (yellowing of the skin or eyes) if the tumor blocks the bile ductJoint pain or aching, especially in multiple jointsSkin nodules or tender red bumps under the skin (from fat breakdown)Diarrhea or changes in bowel habitsBack painFever in some cases
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
8 eventsAlliance for Clinical Trials in Oncology — PHASE2, PHASE3
National Cancer Institute (NCI) — PHASE2
Artios Pharma Ltd — PHASE1, PHASE2
General Oncology, Inc. — PHASE1
National Cancer Institute (NCI) — PHASE2
Sidney Kimmel Comprehensive Cancer Center at Thomas Jefferson University — NA
National Cancer Institute (NCI) — PHASE1
National Cancer Institute (NCI) — PHASE1
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Acinar cell carcinoma of pancreas.
8 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →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
Financial Resources
1 resourcesOPDIVO
E.R. Squibb & Sons, L.L.C.
OPDIVO — Contact E.R. Squibb & Sons, L.L.C.
Travel Grants
No travel grants are currently matched to Acinar cell carcinoma of pancreas.
Community
No community posts yet. Be the first to share your experience with Acinar cell carcinoma of pancreas.
Start the conversation →Latest news about Acinar cell carcinoma of pancreas
1 articlesCaregiver 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.Has my tumor been tested for specific genetic changes, and could those changes make me eligible for targeted therapy or a clinical trial?,Is my tumor able to be removed with surgery, and what are the risks and benefits of that operation?,What chemotherapy regimen do you recommend for my situation, and what side effects should I expect?,Should I get germline (inherited) genetic testing to see if a hereditary cancer syndrome runs in my family?,Are there any clinical trials I should consider given how rare this cancer is?,Will I need pancreatic enzyme supplements or diabetes management after treatment?,How often will I need follow-up scans, and what are the signs that the cancer may be coming back?
Common questions about Acinar cell carcinoma of pancreas
What is Acinar cell carcinoma of pancreas?
Acinar cell carcinoma of the pancreas (ACC) is a rare type of pancreatic cancer that starts in the acinar cells — the cells that normally make digestive enzymes in the pancreas. It is sometimes called pancreatic acinar cell carcinoma or ACC of the pancreas. Unlike the more common type of pancreatic cancer (called pancreatic ductal adenocarcinoma), ACC has some different features and may respond differently to treatment. The pancreas sits behind the stomach and has two main jobs: making digestive juices (enzymes) and controlling blood sugar with hormones like insulin. When acinar cells become
How is Acinar cell carcinoma of pancreas inherited?
Acinar cell carcinoma of pancreas follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Acinar cell carcinoma of pancreas typically begin?
Typical onset of Acinar cell carcinoma of pancreas is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Acinar cell carcinoma of pancreas?
Yes — 8 recruiting clinical trials are currently listed for Acinar cell carcinoma of pancreas on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Acinar cell carcinoma of pancreas?
25 specialists and care centers treating Acinar cell carcinoma of pancreas are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.
What treatment and support options exist for Acinar cell carcinoma of pancreas?
1 patient support program are currently tracked on UniteRare for Acinar cell carcinoma of pancreas. See the treatments and support programs sections for copay assistance, eligibility, and contact details.