The formerly called PRCC type 1 is not a unique entity and includes several new variants, i.e., renal neoplasm with inverted polarity (mutated in KRAS), psammomatous hyalinizing RCC (mutated in NF2), biphasic alveolo-squamoid renal RCC, and thyroid-like follicular RCC (EWSR1-PATZ1 fusion). Here, NF2 is linked to renal cell carcinoma.