Tena SML, Rembao BD, Salinas LC

Idioma: Español
Referencias bibliográficas: 23
Paginas: s5-s10
Archivo PDF: 361.85 Kb.

RESUMEN

La enfermedad de Von Hippel-Lindau (VHL) es una enfermedad autosómica dominante rara. Se caracteriza por tumores múltiples, hemangioblastoma del cerebelo y de la retina, carcinoma renal, feocromocitoma, paraganglioma, quistes, adenomas y carcinoma de páncreas. El carcinoma pancreático es un evento raro en este síndrome y ocurre en 1 a 2%. Presentamos el caso raro de un hombre de 39 años de edad con cuadros psicóticos y depresivos de larga evolución que presentaba enfermedad de Von Hippel Lindau florida con hemangioma cerebeloso, carcinoma renal de células claras múltiple y bilateral, carcinoma papilar y sólido con diferenciación neuroendocrina pancreático y, además, quistes del epidídimo asociado con el gen supresor de VHL axón 1. Sin evidencia de feocromocitoma. Presentamos un caso raro de enfermedad de Von Hippel Lindau con un tumor pancreático papilar y sólido con diferenciación neuroendocrina.

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