For a limited number of patients with MITF p.E318K alterations and renal tumors, in which the histologic subtype was specified, both papillary and clear cell RCC have been reported.7,11,12,13 The clear cell RCC with a MITF p.E318K alteration in this study had a loss of function alteration of the VHL gene, while the papillary RCC reported by Lang et al11 exhibited gains of chromosomes 7 and 17, both molecular hallmarks of the underlying tumor types. Here, VHL is linked to kidney neoplasm.