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The etiology and pathogenesis of the neoplasm are unknown; however, the predilection for women suggest sex hormones or genetic factors may play a role. An association with von Hippel-Lindau disease (loss of heterozygosity at the von Hippel-Lindau (VHL) gene locus 3p25) has been reported . The neoplasm has wild-type KRAS and lacks immunoreactivity for TP53.