PBMAH may also result from alterations in MC2R, PRKACA, and phosphodiesterase (PDE)11A genes [1, 2], and it can occur as part of familial tumor syndromes such as McCune-Albright syndrome (GNAS), MEN1, familial APC, or hereditary leiomyomatosis and renal cell carcinoma (FH gene variant). This evidence concerns the gene MC2R and renal cell carcinoma.