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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).
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