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Objective: To describe the epidemiological, diagnostic, and progressive aspects of VHL disease. Patient and method: This was a case study of a patient who was seen in consultation and followed up, for whom we conducted a retrospective analysis of clinical and paraclinical data, as well as regular monitoring of her progression. Von Hippel-Lindau disease (VHL) is a rare, autosomal dominant, multisystemic disorder that predisposes individuals to the development of highly vascularized benign and malignant tumors. It is caused by germline mutations in the VHL tumor suppressor gene. We report the case of a patient who presented with de novo Von Hippel-Lindau disease with pancreatic involvement by several types of lesions. The interest of our observation lies in this mode of pancreatic involvement and clinical expression, which are rarely described, and in the need to remember that this condition also exists in sub-Saharan Africa and should be considered even in the absence of a family history of VHL disease.

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