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). Here, PDE11A is linked to hereditary clear cell renal cell carcinoma.