ENFERMEDAD DE PRINGLE-BOURNEVILLE. LESIONES VISCERALES. PRESENTACIÓN DE UN PACIENTE
Elio Llerena RodrÃguez, Addys Hernández GarcÃa, Rafael Ibáñez Azán
Resumen
Inicialmente descrita por Von Recklinghausen en 1862 y estudiada a profundidad por Bourneville en 1880, esta rara afección pertenece al grupo de las facomatosis1.
Esta compleja enfermedad se caracteriza por manifestaciones neurocutáneas (adenomas sebáceos, epilepsia, retraso mental) y tumores hamartomatosos en diversos órganos; el más afectado es el riñón, muy por encima de la participación cardÃaca y pulmonar2.
El hamartoma es un tumor formado por elementos hÃsticos normales, los cuales son únicamente anormales en sus valores cuantitativos. La mayorÃa de estos hamartomas renales son angiomiolipomas, y su integración histológica está constituida por tejido muscular liso, adiposo y vasos, en proporciones variables de unos pacientes a otros3.
En la actualidad, a pesar de un incremento en el diagnóstico, continúan siendo infrecuentes, lo que ha motivado la presentación de este paciente.
Este artÃculo está licenciado bajo la licencia
Creative Commons Reconocimiento-NoComercial 4.0 Internacional .
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