Tena SML, Rembao BD, Salinas LC

Language: Spanish
References: 23
Page: s5-s10
PDF size: 361.85 Kb.

ABSTRACT

Von Hippel-Lindau (VHL) disease is a dominant autosomal disorder inducing the development of many tumors, such as hemangioblastoma in the central nervous system and retina, cysts or tumors (benign or malignant) in the kidneys and/or the pancreas. We reported a male 39 years old with long time of psychotic and depressive disturbances. The autopsy reveled a Von Hippel Lindau disease, with cerebellar hemangioblastoma, multiple renal bilateral carcinoma and pancreatic cyst papillary and solid neuroendocrine carcinoma. Also he showed epydidimal cyst associated to gene of VHL axon 1. Without feocromocytoma. In the present case we report a rare case of Von Hippel Lindau disease with an exceptional pancreatic papillary cyst and solid carcinoma with neuroendocrine differentiation.

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