Long-Term Persistence of Glycemic Dysregulation in Patients With a History of Pheochromocytoma/Paraganglioma
By
Alessa Fischer
Hanna Remde
Christina Pamporaki
Ulrich Dischinger
Nicole Bechmann
Mercedes Robledo
Katharina Wang
Diana Vetter
José Oberholzer
Grégoire B Morand
Simon Andreas Mueller
Alexander Huber
Ralph Fritsch
Sven Gruber
Constanze Hantel
Kathrin Zitzmann
Martin Reincke
Christoph J Auernhammer
Karel Pacak
Ashley B Grossman
Felix Beuschlein
Svenja Nölting
January 2, 2025
Clinical Scorecard: Chronic Glycemic Dysregulation in Individuals With a History of Pheochromocytoma or Paraganglioma
At a Glance
Category Detail
Condition Pheochromocytomas and paragangliomas (PPGLs), rare neuroendocrine tumors producing catecholamines Key Mechanisms Catecholamine secretion causing cardiometabolic complications including hyperglycemia and cardiovascular events Target Population Patients with current or history of PPGL, including those with metastatic disease or pathogenic variants predisposing to PPGL Care Setting Multicenter endocrine and oncology clinics with surgical and long-term follow-up capabilities
Key Highlights
Patients with a history of PPGL exhibit persistent hyperglycemic disorders and elevated HbA1c levels long after tumor resection. Catecholamine-induced cardiometabolic complications contribute significantly to morbidity and mortality in PPGL patients. Despite surgical removal, long-term cardiometabolic risk factors such as hyperglycemia and hypertension often persist. Guideline-Based Recommendations
Diagnosis
Evaluate biochemical activity of PPGL via catecholamine measurements. Assess genetic testing for pathogenic variants in patients with PPGL or family history. Screen for cardiometabolic risk factors including glycemic status, lipid profile, and BMI at diagnosis and follow-up. Management
Surgical resection of PPGL is primary treatment to reduce catecholamine excess. Postoperative monitoring and management of persistent hyperglycemia and hypertension are essential. Consider multidisciplinary care involving endocrinology, cardiology, and genetics. Monitoring & Follow-up
Long-term follow-up of glycemic control using HbA1c measurements. Regular assessment of blood pressure, lipid profile, and body weight. Surveillance for tumor recurrence or metastasis, especially in patients with pathogenic variants. Risks
Persistent hyperglycemia and increased cardiometabolic risk despite tumor removal. Potential for metastatic or recurrent disease leading to sustained catecholamine elevation. Cardiovascular complications including cardiomyopathy, arrhythmias, and stroke. Patient & Prescribing Data
Patients with current or history of PPGL, including those post-surgical resection and genetic variant carriers
Surgical resection improves catecholamine levels and short-term glycemic control, but persistent hyperglycemia and cardiometabolic risks require ongoing management.
Clinical Best Practices
Perform comprehensive cardiometabolic risk assessment at diagnosis and during long-term follow-up. Implement multidisciplinary care approaches to address endocrine, cardiovascular, and metabolic complications. Educate patients on the potential for persistent glycemic disturbances post-PPGL resection and the importance of regular monitoring. Use HbA1c as a key biomarker for monitoring glycemic control in PPGL patients post-surgery. Screen asymptomatic pathogenic variant carriers for early detection and prevention of PPGL-related complications. References
