[Angiomyolipoma and Lenk syndrome: case report]. / Triada de Lenk como presentación clínica del angiomiolipoma.

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.

Tríada de Lenk como presentación clínica del angiomiolipoma / Angiomyolipoma and Lenk syndrome: case report

El angiomiolipoma (AML) es una neoplasia clonal benigna compuesta por cantidades variables de tejido adiposo maduro, músculo liso y vasos sanguíneos de paredes gruesas. Aproximadamente un 20% de todos los AML, se detectan en pacientes con el Síndrome de la Esclerosis Tuberosa, trastorno autonómico dominante que se asocia a retraso mental, epilepsia, adenoma sebáceo. La presencia de hemorragia retroperitoneal masiva, conocida como Síndrome de Wunderlich se ha documentado en hasta un 10% de los pacientes y representa la complicación más importante y temida de estas lesiones. La TAC identifica las zonas del tejido adiposo, correspondiente a una baja atenuación entre 20-100Hounsfield, dicho coeficiente dependerá del contenido y madurez de la grasa intratumoral y de la presencia o no de hemorragias. En el caso de pacientes con AML pequeños que requieren intervención debido a la presencia de síntomas, en pacientes con ET o AML multicéntricos y en pacientes en quienes la preservación de la función renal reviste particular importancia el enfoque preferido es la cirugía ahorradora de nefronas con nefrectomía parcial o embolizaciòn selectiva. Presentamos un caso de una paciente de 59 años con un angiomiolipoma gigante quién debuta con el Síndrome clínico conocido como Tríada de Lenk (AU)

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 autosomaldominant 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 Hounsfieldun it’s <=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 (AU)