Personalized Management of Pheochromocytoma and Paraganglioma

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

Practical tip/synthesis (applies to all clusters):
• The recently established signs and symptoms score (−1 to +7 points) to triage patients according to their likelihood of PPGLs is provided under “Personalized Management: Molecular Cluster 1” subsection “Clinical Presentations” (“Synthesis”). • Signs and symptoms of cluster 2–related PPGLs are mainly of an episodic nature associated with paroxysmal excessive catecholamine secretion (triggered by stimuli) due to a high catecholamine content, but low rates of constant secretion and a well-developed regulatory control, in contrast to cluster 1 tumors. • Patients with cluster 2–related PPGLs have higher signs and symptoms scores and more often suffer from pallor, tremor, and anxiety/panic, compared with those with cluster 1–related PPGLs.

Practical tip/synthesis (applies to all clusters):

• The recently established signs and symptoms score (−1 to +7 points) to triage patients according to their likelihood of PPGLs is provided under “Personalized Management: Molecular Cluster 1” subsection “Clinical Presentations” (“Synthesis”).
• Signs and symptoms of cluster 2–related PPGLs are mainly of an episodic nature associated with paroxysmal excessive catecholamine secretion (triggered by stimuli) due to a high catecholamine content, but low rates of constant secretion and a well-developed regulatory control, in contrast to cluster 1 tumors.
• Patients with cluster 2–related PPGLs have higher signs and symptoms scores and more often suffer from pallor, tremor, and anxiety/panic, compared with those with cluster 1–related PPGLs.