Patient with Von Hipple-Lindau Disease

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Patient with Von Hipple-Lindau Disease

With a prevalence of 1 per 36,000 people [1], von Hippel-Lindau (VHL) disease is an autosomal dominant syndrome that, due to loss of tumor suppressor gene function, predisposes affected individuals to various benign and malignant tumors. Such tumors have historically included hemangioblastomas of the retina and central nervous system, renal cell carcinoma (RCC), pheochromocytomas, and tumors of the pancreas, middle ear, epididymis, and broad ligament [1-3]. Lymphomas, on the other hand, are a varied group of clonal diseases that arise from a lymphocyte progenitor and can affect any site of the lymphatic system [4]. Specifically, Non Hodgkin’s lymphoma (NHL) is the fifth most common malignancy in American adults with 30- 40% of new cases classified as diffuse large B-cell Lymphoma (DLBCL), an aggressive subtype with the highest overall incidence [5,6]. Though the association between NHL and RCC is significant, no clear explanation for such association has been provided [7-10]. To the best of our knowledge, this is the first reported case of a patient presenting with VHL and NHL. Additionally, we provide a potential explanation for the association between NHL and RCC through the role of defective VHL product causing increased Vascular Endothelial Growth Factor Receptor (VEGF) levels ultimately leading to increased tumor angiogenesis.(

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