| Liver transplantation for multiple angiomyolipomas complicating tuberous sclerosis complex. | |
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MedLine Citation:
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PMID: 20674202 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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J Dumortier; O Guillaud; T Walter; C E Ber; C Partensky; O Boillot; J-Y Scoazec |
Publication Detail:
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Type: Case Reports; Journal Article Date: 2010-07-31 |
Journal Detail:
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Title: Gastroentérologie clinique et biologique Volume: 34 ISSN: 2210-7401 ISO Abbreviation: Gastroenterol. Clin. Biol. Publication Date: 2010 Sep |
Date Detail:
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Created Date: 2010-09-29 Completed Date: 2011-01-10 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7704825 Medline TA: Gastroenterol Clin Biol Country: France |
Other Details:
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Languages: eng Pagination: 494-8 Citation Subset: IM |
Copyright Information:
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Copyright © 2010. Published by Elsevier Masson SAS. |
Affiliation:
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Fédération des spécialités digestives, hospices Civils de Lyon, hôpital Edouard-Herriot, Lyon cedex 03, France. jerome.dumortier@chu-lyon.fr |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Angiomyolipoma / complications, pathology, surgery* Fatal Outcome Humans Liver Neoplasms / complications, pathology, surgery* Liver Transplantation* Male Neoplasms, Multiple Primary / pathology, surgery* Pneumonia, Bacterial Postoperative Complications Pseudomonas Infections Pseudomonas aeruginosa Tuberous Sclerosis / complications* |
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