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):
• A clinical feature score (−1 to +7 points) for signs and symptoms to triage patients according to their likelihood of PPGLs has very recently been published (86): - Pallor +1 point - Hyperhidrosis +1 point - Palpitations +1 point - Tremor +1 point - Nausea +1 point - Body mass index (BMI) < 25 kg/m² +1 point - Heart rate of 85 bpm or higher + 1 point - Obesity (BMI > 30 kg/m²) −1 point • 5.8-fold higher probability of a PPGL with a high score of 3 or more. • Cluster 1–related PPGLs are more likely to be associated with lower basic symptom scores and sustained hypertension, compared with cluster 2-related PPGLs. • Cluster 2–related PPGLs are more likely to be associated with higher basic symptom scores, an episodic nature of symptoms, with tremor, anxiety/panic and pallor, and older age at first diagnosis, compared with cluster 1–related PPGLs.

Practical tip/synthesis (applies to all clusters):

• A clinical feature score (−1 to +7 points) for signs and symptoms to triage patients according to their likelihood of PPGLs has very recently been published (86):
- Pallor +1 point
- Hyperhidrosis +1 point
- Palpitations +1 point
- Tremor +1 point
- Nausea +1 point
- Body mass index (BMI) < 25 kg/m² +1 point
- Heart rate of 85 bpm or higher + 1 point
- Obesity (BMI > 30 kg/m²) −1 point
• 5.8-fold higher probability of a PPGL with a high score of 3 or more.
• Cluster 1–related PPGLs are more likely to be associated with lower basic symptom scores and sustained hypertension, compared with cluster 2-related PPGLs.
• Cluster 2–related PPGLs are more likely to be associated with higher basic symptom scores, an episodic nature of symptoms, with tremor, anxiety/panic and pallor, and older age at first diagnosis, compared with cluster 1–related PPGLs.