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Liver transplantation for multiple angiomyolipomas complicating tuberous sclerosis complex
J Dumortier1, O Guillaud, T Walter
1Fédération des spécialités digestives, hospices Civils de Lyon, hôpital Edouard-Herriot, Lyon cedex 03, France. jerome.dumortier@chu-lyon.fr
Gastroenterologie Clinique Et Biologique
|August 3, 2010
Summary
Tuberous sclerosis complex (TSC) can cause rare liver angiomyolipomas. Liver transplantation was necessary for a TSC patient with severe complications from massive, multiple benign liver tumors.
Area of Science:
- Hepatology
- Genetics
- Oncology
Background:
- Tuberous sclerosis complex (TSC) is a genetic disorder causing hamartomas in multiple organs.
- Hepatic angiomyolipomas are rare, usually asymptomatic TSC complications.
Observation:
- A TSC patient presented with severe malnutrition, anorexia, and pain due to massive liver angiomyolipomas.
- Seventeen tumors (2-16 cm) were found in the explanted liver.
Findings:
- The liver tumors were benign, showing no signs of malignancy.
- The sheer size and number of tumors precluded surgical resection.
Implications:
- This case highlights liver transplantation as a viable option for benign liver tumors with extensive, unresectable lesions.
- It expands the indications for liver transplantation in complex genetic disorders like TSC.
