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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 including renal cell cancer. In contrast, lymphomas are a varied group of clonal diseases arising from a lymphocyte progenitor and can affect any site of the lymphatic system. We present the case of a 56 year old female with Von Hippel-Lindau disease and clear cell renal carcinoma (CCRC), who developed a nasopharyngeal mass with cervical and submandibular lymphadenopathy subsequently proven to be a Non Hodgkin’s lymphoma (NHL). Though the association between VHL and renal cell cancer is significant, a mechanism linking these two diseases has previously been unknown. To the best of our knowledge, this is the first reported case of a patient presenting with coexistent VHL and NHL. In an effort to explain this rarity, we propose that a defective VHL allele may serve as a possible link between VHL and RCC, thus leading to an environment that would favour the development of a malignant clone, our patient’s NHL.(
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