Overview
Rare malignant epithelial tumors of the liver and intrahepatic biliary tract are a group of uncommon cancers that start in the cells lining the liver or the bile ducts located inside the liver. This broad category includes cancers such as intrahepatic cholangiocarcinoma (bile duct cancer inside the liver), combined hepatocellular-cholangiocarcinoma, and other rare epithelial cancers that do not fit neatly into the more common liver cancer types. These tumors are different from the most common liver cancer, hepatocellular carcinoma, though they share the same organ. These cancers affect the liver's ability to filter blood, produce bile, and carry out hundreds of chemical processes the body depends on. As the tumor grows, it can block bile flow, damage liver tissue, and spread to nearby lymph nodes or other organs. Common symptoms include yellowing of the skin and eyes (jaundice), abdominal pain, unexplained weight loss, fatigue, and changes in stool or urine color. Treatment depends on the specific tumor type, its size, and whether it has spread. Options may include surgery to remove the tumor, liver transplantation in select cases, chemotherapy, targeted therapy, immunotherapy, and radiation. Because these tumors are rare and often diagnosed at a late stage, treatment can be challenging. Research into new therapies, including targeted drugs based on genetic mutations in the tumor, is ongoing and offering new hope to patients.
Also known as:
Key symptoms:
Yellowing of the skin and whites of the eyes (jaundice)Pain or discomfort in the upper right side of the abdomenUnexplained weight lossPersistent fatigue and weaknessLoss of appetiteDark-colored urinePale or greasy stoolsItchy skinFever or night sweatsNausea or vomitingSwelling of the abdomen due to fluid buildup (ascites)A lump or mass felt in the abdomen
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
10 eventsUniversity Hospital, Angers — NA
Case Comprehensive Cancer Center — PHASE2
UNICANCER — PHASE2
Assistance Publique - Hôpitaux de Paris — PHASE2, PHASE3
CHA University
Yongjun Chen — PHASE2
Eisai Co., Ltd.
Hangzhou Hanx Biopharmaceuticals, Ltd. — PHASE1, PHASE2
Tongji Hospital
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
1 availableDyural 80-Lm
indicated in adults for the production of local or regional anesthesia or analgesia for diagnostic and therapeutic procedures
Specialists
View all specialists →No specialists are currently listed for Rare malignant epithelial tumor of liver and intrahepatic biliary tract.
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 malignant epithelial tumor of liver and intrahepatic biliary tract.
Community
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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 tumor I have, and has it spread beyond the liver?,Has my tumor been tested for genetic mutations like FGFR2, IDH1, or others that could guide treatment?,Am I a candidate for surgery, and if not, what are my treatment options?,Are there any clinical trials I might be eligible for?,What are the expected side effects of the recommended treatment, and how will they be managed?,Should I or my family members be tested for hereditary cancer syndromes?,What support services — such as nutrition counseling, palliative care, or mental health support — are available to me?
Common questions about Rare malignant epithelial tumor of liver and intrahepatic biliary tract
What is Rare malignant epithelial tumor of liver and intrahepatic biliary tract?
Rare malignant epithelial tumors of the liver and intrahepatic biliary tract are a group of uncommon cancers that start in the cells lining the liver or the bile ducts located inside the liver. This broad category includes cancers such as intrahepatic cholangiocarcinoma (bile duct cancer inside the liver), combined hepatocellular-cholangiocarcinoma, and other rare epithelial cancers that do not fit neatly into the more common liver cancer types. These tumors are different from the most common liver cancer, hepatocellular carcinoma, though they share the same organ. These cancers affect the li
How is Rare malignant epithelial tumor of liver and intrahepatic biliary tract inherited?
Rare malignant epithelial tumor of liver and intrahepatic biliary tract follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Rare malignant epithelial tumor of liver and intrahepatic biliary tract typically begin?
Typical onset of Rare malignant epithelial tumor of liver and intrahepatic biliary tract is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Rare malignant epithelial tumor of liver and intrahepatic biliary tract?
Yes — 19 recruiting clinical trials are currently listed for Rare malignant epithelial tumor of liver and intrahepatic biliary tract on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.