[Angiomyolipoma and Lenk syndrome: case report]. / Triada de Lenk como presentación clínica del angiomiolipoma.
Actas Urol Esp
; 32(8): 850-4, 2008 Sep.
Article
en Es
| MEDLINE
| ID: mdl-19013986
ABSTRACT
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.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Angiomiolipoma
/
Dolor en el Flanco
/
Hematuria
/
Enfermedades Renales
/
Neoplasias Renales
Límite:
Female
/
Humans
/
Middle aged
Idioma:
Es
Revista:
Actas Urol Esp
Año:
2008
Tipo del documento:
Article
País de afiliación:
Argentina