Table 2.

Clinical characteristics associated with PCC/PGL susceptibility genes.

PCC/PGL susceptibility gene	Transmission	Penetrance for PCC/PGL	Adrenal PCC	HNPGL	Extra-adrenal PGL	Risk of multifocal primary PCC/PGL	Risk of metastatic disease
VHL	Autosomal dominant	19% 60% for type 2	+++	+	+	+++	<5%
RET	Autosomal dominant	50%	+++	+	+	+++	<5%
NF1	Autosomal dominant	7.7–14%	+++	+	+	++	~12%
SDHB	Autosomal dominant	22–26% by age 60	++	++	+++	++	25–50%
SDHD	Autosomal dominant – paternal inheritance	43% by age 60	++	+++	++	+++	3–8%
SDHC	Autosomal dominant	25% by age 60a	+	+++	+	++	<5%
SDHA	Autosomal dominant	10% by age 70	++	++	++	+	12%a
SDHAF2	Autosomal dominant – paternal inheritance	Uknb	Uknb	+++	Uknb	+++	<5%
TMEM127	Autosomal dominant	Uknb	+++	+	+	++	<5%
MAX	Autosomal dominant	Uknb	+++	Uknb	+	+++	<10%a

+++, very likely; ++, likely; +, less likely but can occur. ^aSmall sample size may skew estimates. ^bUkn, unknown.