Personalized Management of Pheochromocytoma and Paraganglioma

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

Practical tip/synthesis:
• Table 8 summarizes the penetrance, metastatic risk, and location of cluster 2–related PPGLs. • RET-related penetrance of PCCs depends on the specific RET mutation and is high (around 50%, multiple/bilateral 50%-80%), while the penetrance of NF1, TMEM127, and MAX is much lower. • Of all metastatic PPGLs, only around 2%-4% belong to cluster 2 (see under “Personalized Management: Molecular Cluster 1” subsection “Penetrance, Epidemiology, and Metastatic Risk”). • The metastatic risk of cluster 2–related PPGLs is low (around 4%-5%) (see under “Personalized Management: Molecular Cluster 1” subsection “Penetrance, Epidemiology, and Metastatic Risk”). • Cluster 2–related tumors are almost exclusively located adrenally (PCCs).

Practical tip/synthesis:

• Table 8 summarizes the penetrance, metastatic risk, and location of cluster 2–related PPGLs.
• RET-related penetrance of PCCs depends on the specific RET mutation and is high (around 50%, multiple/bilateral 50%-80%), while the penetrance of NF1, TMEM127, and MAX is much lower.
• Of all metastatic PPGLs, only around 2%-4% belong to cluster 2 (see under “Personalized Management: Molecular Cluster 1” subsection “Penetrance, Epidemiology, and Metastatic Risk”).
• The metastatic risk of cluster 2–related PPGLs is low (around 4%-5%) (see under “Personalized Management: Molecular Cluster 1” subsection “Penetrance, Epidemiology, and Metastatic Risk”).
• Cluster 2–related tumors are almost exclusively located adrenally (PCCs).