We also found a unique association of PV in the TMEM127 and TP53 genes (R337H) in a 47-year-old patient who developed bilateral metastatic breast cancer, meningioma of the CNS, and pancreatic cancer; her 20-year-old daughter is a carrier of the same variants who did not develop PPGL thus far but faced bilateral breast cancer and meningioma of the CNS and her 5-year-old granddaughter carries only the TMEM127 gene variant and currently has no clinical or laboratory evidence of PPGL. Here, TMEM127 is linked to familial pancreatic carcinoma.