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. 2021 Jun 19;43(2):199–239. doi: 10.1210/endrev/bnab019

Table 8.

Penetrance/prevalence, metastatic risk, location of cluster 2–related PCCs

RET NF1 TMEM127 MAX
Penetrance Around 50% (50%-80% multiple) Around 7%-8% (12% multiple) Penetrance unknown, prevalence around 2% (single tumors) Penetrance unknown, prevalence around 1% (67% multiple)
Metastatic risk <5% (3.5%) Around 2%-12% Mostly benign Around 10%
Location adrenal