Overview
Rare tumor of the liver and intrahepatic biliary tract refers to a group of uncommon cancers that develop in the liver or in the bile ducts located inside the liver. These tumors are distinct from the more common types of liver cancer, such as hepatocellular carcinoma and typical cholangiocarcinoma. This category includes several unusual tumor types such as hepatoblastoma in adults, combined hepatocellular-cholangiocarcinoma, undifferentiated embryonal sarcoma, primary hepatic lymphoma, angiosarcoma of the liver, epithelioid hemangioendothelioma, and other very rare growths. Symptoms often develop slowly and may include pain or a feeling of fullness in the upper right part of the abdomen, unexplained weight loss, loss of appetite, fatigue, yellowing of the skin and eyes (jaundice), nausea, and sometimes a noticeable lump in the abdomen. Because these tumors are rare, they can be difficult to diagnose and may be found incidentally during imaging for other reasons. Treatment depends on the specific tumor type, its size, location, and whether it has spread. Options may include surgery to remove the tumor, liver transplantation in select cases, chemotherapy, radiation therapy, targeted therapy, or a combination of these approaches. Because of their rarity, management often requires a multidisciplinary team at a specialized center. Clinical trials may also be available and can offer access to newer treatments. The prognosis varies widely depending on the exact type of tumor and how early it is detected.
Also known as:
Key symptoms:
Pain or discomfort in the upper right abdomenUnexplained weight lossLoss of appetiteFatigue and general weaknessYellowing of the skin and eyes (jaundice)Nausea or vomitingA noticeable lump or swelling in the abdomenFever of unknown originItchy skinDark-colored urinePale or clay-colored stoolsFeeling full after eating only a small amountAbdominal bloating or fluid buildup (ascites)
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
7 eventsEastern Hepatobiliary Surgery Hospital — PHASE2
Shanghai Zhongshan Hospital — NA
JIANGSU SHENMING Medical Technology CO., Ltd — NA
National Cancer Institute (NCI)
University of Sydney — NA
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Rare tumor of liver and intrahepatic biliary tract.
3 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 Rare tumor of liver and intrahepatic biliary tract.
Community
No community posts yet. Be the first to share your experience with Rare tumor of liver and intrahepatic biliary tract.
Start the conversation →Latest news about Rare tumor of liver and intrahepatic biliary tract
Disease timeline:
New recruiting trial: Clinical Trial Evaluating the Safety and Efficacy of Tumor Thermosensitive Embolic Agent in Transcatheter Arterial Chemoembolization for Primary Liver Cancer
A new clinical trial is recruiting patients for Rare tumor of liver and intrahepatic biliary tract
New recruiting trial: Outpatient Versus Inpatient Care Pathway for Intra-arterial Treatment of Primary Liver Cancer (CHOC)
A new clinical trial is recruiting patients for Rare tumor of liver and intrahepatic biliary tract
New recruiting trial: An Exosome-Based Liquid Biopsy for the Differential Diagnosis of Primary Liver Cancer
A new clinical trial is recruiting patients for Rare tumor of liver and intrahepatic biliary tract
New recruiting trial: Stroke Volume Variation Versus Central Venous Pressure Guidance for Reducing Perioperative Blood Loss During Open Liver Resection
A new clinical trial is recruiting patients for Rare tumor of liver and intrahepatic biliary tract
New recruiting trial: Real-world Evaluation of the HistoSonics Edison System for Treatment of Liver Tumors Across Multidisciplinary Users (BOOMBOX: Master Study)
A new clinical trial is recruiting patients for Rare tumor of liver and intrahepatic biliary tract
New recruiting trial: cfDNA Methylation for Liver Cancer Recurrence Detection
A new clinical trial is recruiting patients for Rare tumor of liver and intrahepatic biliary tract
New recruiting trial: Radiotherapy Delays Second-line Drug Therapy for Oligo Progressive Primary Liver Cancer
A new clinical trial is recruiting patients for Rare tumor of liver and intrahepatic biliary tract
New recruiting trial: Stereotactic Radiosurgery Versus Radiofrequency Ablation for Primary Liver Cancer
A new clinical trial is recruiting patients for Rare tumor of liver and intrahepatic biliary tract
New recruiting trial: Clinical Study of VG161 in Subjects with Advanced Primary Liver Cancer
A new clinical trial is recruiting patients for Rare tumor of liver and intrahepatic biliary tract
New recruiting trial: Proton Beam Radiotherapy Followed by Tecentriq and Avastin for Primary Liver Cancer With Vp2-4 Portal Vein Invasion
A new clinical trial is recruiting patients for Rare tumor of liver and intrahepatic biliary tract
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 specific type of rare liver tumor do I have, and what does that mean for my treatment and outlook?,Is my tumor eligible for surgical removal or liver transplantation?,Are there any clinical trials available for my specific tumor type?,Should molecular or genetic testing be done on my tumor to guide treatment decisions?,What are the potential side effects of the recommended treatment, and how can they be managed?,How often will I need follow-up imaging and blood tests?,Are there any dietary or lifestyle changes I should make to support my liver health during treatment?
Common questions about Rare tumor of liver and intrahepatic biliary tract
What is Rare tumor of liver and intrahepatic biliary tract?
Rare tumor of the liver and intrahepatic biliary tract refers to a group of uncommon cancers that develop in the liver or in the bile ducts located inside the liver. These tumors are distinct from the more common types of liver cancer, such as hepatocellular carcinoma and typical cholangiocarcinoma. This category includes several unusual tumor types such as hepatoblastoma in adults, combined hepatocellular-cholangiocarcinoma, undifferentiated embryonal sarcoma, primary hepatic lymphoma, angiosarcoma of the liver, epithelioid hemangioendothelioma, and other very rare growths. Symptoms often de
How is Rare tumor of liver and intrahepatic biliary tract inherited?
Rare tumor of liver and intrahepatic biliary tract follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Are there clinical trials for Rare tumor of liver and intrahepatic biliary tract?
Yes — 3 recruiting clinical trials are currently listed for Rare 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.
Which specialists treat Rare tumor of liver and intrahepatic biliary tract?
25 specialists and care centers treating Rare tumor of liver and intrahepatic biliary tract are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.