title: Liver transplantation for multiple angiomyolipomas complicating tuberous sclerosis complex.
abstract: Tuberous sclerosis complex is a genetic multisystem disorder characterised by widespread hamartomas in several organs, including the brain, heart, skin, eyes, kidney, lung, and liver. Hepatic multiple, bilateral angiomyolipomas are a rare and usually asymptomatic complication in patients with tuberous sclerosis. We report here the case of a patient who needed liver transplantation because of debilitating manifestations and mechanical complications of massive liver involvement by multiple angiomyolipomas (severe malnutrition, anorexia and abdominal pain). Seventeen tumors, from 2 to 16 cm in diameter, were identified at examination of the liver explant. No feature suggestive of malignant behaviour was identified at histological examination. In conclusion, this unusual indication of liver transplantation underlines the interest of this therapeutic approach for benign tumors for which the multiplicity of the lesions and their huge volume prevent any attempt at surgical resection.
authors.affiliation: Fédération des spécialités digestives, hospices Civils de Lyon, hôpital Edouard-Herriot, Lyon cedex 03, France. jerome.dumortier@chu-lyon.fr
authors.forename: J
authors.initials: J
authors.lastname: Dumortier
authors.forename: O
authors.initials: O
authors.lastname: Guillaud
authors.forename: T
authors.initials: T
authors.lastname: Walter
authors.forename: C E
authors.initials: CE
authors.lastname: Ber
authors.forename: C
authors.initials: C
authors.lastname: Partensky
authors.forename: O
authors.initials: O
authors.lastname: Boillot
authors.forename: J-Y
authors.initials: JY
authors.lastname: Scoazec
mesh_terms: D000328:Adult; D018207:Angiomyolipoma / Q000150:complications / Q000473:pathology / Q000601:surgery*; D017809:Fatal Outcome; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications / Q000473:pathology / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000473:pathology / Q000601:surgery*; D018410:Pneumonia, Bacterial; D011183:Postoperative Complications; D011552:Pseudomonas Infections; D011550:Pseudomonas aeruginosa; D014402:Tuberous Sclerosis / Q000150:complications*