Reduction in size of renal angiomyolipoma after treatment with everolimus in lung transplantation due to lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is a rare disease characterized by abnormal proliferation of immature smooth muscle cells and cystic lung destruction, which determines the prognosis of the disease. The kidney angiomyolipomas are usually very common in this disease and are usually asymptomatic unless complications arise. In the absence of a curative treatment, recent publications show promising results in molecular therapy to prevent functional decline and to control the size of the angiomyolipomas. These therapies include mTOR complex inhibitors, especially sirolimus. We report a case of a patient diagnosed with LAM who underwent lung transplantation with reduction of renal angiomyolipoma size after treatment with the mTOR inhibitor everolimus.

Reducción de tamaño del angiomiolipoma renal tras el tratamiento con everolimus en trasplante pulmonar por linfangioleiomiomatosis / Reduction in Size of Renal Angiomyolipoma after Treatment with Everolimus in Lung Transplantation due to Lymphangioleiomyomatosis

La linfangioleiomiomatosis (LAM) es una enfermedad rara caracterizada por la proliferación anormal de células musculares lisas inmaduras y una destrucción quística del pulmón, que condiciona el pronóstico de la enfermedad. Los angiomiolipomas renales suelen ser muy frecuentes en esta enfermedad, generalmente de curso asintomático, salvo complicaciones. Ante la ausencia de un tratamiento curativo, las últimas publicaciones reflejan resultados esperanzadores en la terapia molecular para evitar el deterioro funcional y el control del tamaño de los angiomiolipomas. Entre estas terapias destacan los inhibidores del complejo mTOR, sobre todo sirolimus. Presentamos un caso clínico de una paciente diagnosticada de LAM sometida a trasplante pulmonar con reducción del tamaño del angiomiolipoma renal tras el tratamiento con el inhibidor mTOR everolimus(AU)

Lymphangioleiomyomatosis (LAM) is a rare disease characterized by abnormal proliferation of immature smooth muscle cells and cystic lung destruction, which determines the prognosis of the disease. The kidney angiomyolipomas are usually very common in this disease and are usually asymptomatic unless complications arise. In the absence of a curative treatment, recent publications show promising results in molecular therapy to prevent functional decline and to control the size of the angiomyolipomas. These therapies include mTOR complex inhibitors, especially sirolimus. We report a case of a patient diagnosed with LAM who underwent lung transplantation with reduction of renal angiomyolipoma size after treatment with the mTOR inhibitor everolimus(AU)