[Angiomyolipoma and Lenk syndrome: case report].
Angiomyolipoma (AML) is a benign clonal neoplasm that consists of varying amounts of mature adipose tissue, smooth muscle, and thick-walled vessels. Approximately 20% of AMLs are found in patients with tuberous sclerosis syndrome (TS), an autosomal-dominant disorder characterized by mental retardation, epilepsy, and adenoma sebaceous, a distinctive skin lesion. Massive retroperitoneal hemorrhage from AML, also known as Wunderlich's syndrome, has been found in up to 10% of patients and represents the most significant and feared complication. The presence of even a small amount of fat within a renal lesion on CT scan (confirmed by Hounsfield unit's < or = 10) is considered diagnostic of AML. Intervention should be considered for larger tumors, particularly if the patient is symptomatic, taking into account patient age, comorbidities, and other related factors. A nephron-sparing approach, via either partial nephrectomy or selective embolization, is clearly preferred. We present the case of a fifty-nine-year old patient with an angiomyolipoma and massive retroperitoneal hemorrhage with Lenk syndrome.
Pedemonte
J G
JG
Departamento de Cirugía, Servicio de Urología, Hospital Italiano Garibaldi, Instituto Universitario Italiano, Rosario, Santa Fe, Argentina. jgpedemonte@yahoo.com.ar
Degiovanni
D
D
Pusterla
D
D
Reibel
C
C
Di Nucci
J
J
Boccio
C
C
Degiovanni
R
R
Dotta
A
A
D018207:Angiomyolipoma / Q000150:complications*; D005260:Female; D021501:Flank Pain / Q000150:complications*; D006417:Hematuria / Q000150:complications*; D006801:Humans; D007674:Kidney Diseases / Q000150:complications*; D007680:Kidney Neoplasms / Q000150:complications*; D008875:Middle Aged; D013577:Syndrome