Linfangioleiomiomatosis esporádica. Presentación de un caso con enfermedad pulmonar y linfangioleiomiomas retroperitoneales
Sporadic lymphangioleiomyomatosis. Presentation of a case of pulmonary disease with retroperitoneal lymphangioleiomyomas
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La linfangioleiomiomatosis (LAM) es una enfermedad multisistémica asociada a mutaciones del gen TSC 2, que se presenta en forma esporádica (LMA-E) o asociada al complejo de esclerosis tuberosa (LAM-CET). La LAM cursa con enfermedad pulmonar relacionada con quistes en el parénquima. Sin embargo, los pacientes con LAM-E pueden evidenciar alteraciones mediastinales, retroperitoneales y renales, con adenomegalias, linfangioleiomiomas y angiomiolipomas.
Presentamos el caso de una paciente con LAM-E y enfermedad pulmonar quística asociada a linfangioleiomiomas retroperitoneales y pélvicos.
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