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Unusual presentation of tuberous sclerosis complex

Favilla, Vincenzo and Cimino, Sebastiano and Castelli, Tommaso and Marchese, Francesco and Russo, Giorgio Ivan and Russo, Marco and Madonia, Massimo and Morgia, Giuseppe (2012) Unusual presentation of tuberous sclerosis complex. Urology, Vol. 80 (4), e39-e40. eISSN 1527-9995. Article.

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DOI: 10.1016/j.urology.2012.06.016


A 47-year-old woman presented with acute abdominal pain, hematuria, signs of hemorrhagic shock and presence of a palpable mass in the right hypochondria at physical examination. Total body computed tomography showed bilateral renal angiomyolipoma (AML), voluminous abdominal mass of annexial origin, pulmonary lymphangioleiomyomatosis (LAM), and multiple subependymal calcification in the brain. After failure of selective transarterial embolization of the right kidney, the patient underwent exeresis of the abdominal mass and right nephrectomy (2200 g). This is a rare systemic manifestation of tuberous sclerosis complex consisting of renal AML and pulmonary LAM.

Item Type:Article
ID Code:8714
Uncontrolled Keywords:Bilateral renal angiomyolipoma (AML), pulmonary lymphangioleiomyomatosis (LAM), tuberous sclerosis syndrome (TS)
Subjects:Area 06 - Scienze mediche > MED/24 Urologia
Divisions:001 Università di Sassari > 01-a Nuovi Dipartimenti dal 2012 > Scienze Chirurgiche, Microchirurgiche e Mediche
Publisher:Elsevier Science
Deposited On:28 Feb 2013 14:04

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