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Clinical decision making in small non-functioning VHL-related incidentalomas.
Roland Därr1,2, Jonas Kater2, Peggy Sekula3
1Department of Cardiology and Angiology I, Heart Center Freiburg University, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Endocrine Connections
|September 2, 2020
Summary
For small, non-functioning VHL-related tumors, monitoring size and metanephrines is key. Regular assessment helps predict growth and secretory activity for optimal management.
Area of Science:
- Endocrinology
- Oncology
- Genetics
Background:
- Optimal treatment for small, non-functioning VHL-related incidentalomas remains unclear.
- Pheochromocytoma/paraganglioma (PHEO/PGL) are common in VHL disease.
- Understanding tumor behavior is crucial for patient management.
Purpose of the Study:
- To investigate the characteristics and optimal monitoring strategies for small, non-functioning VHL-related incidentalomas.
- To identify predictors of tumor growth and secretory activity.
Main Methods:
- Retrospective analysis of the Freiburg VHL registry for patients with PHEO/PGL.
- Evaluation of tumor size, biochemical activity, and clinical symptoms.
- Logistic regression analysis to predict biochemical test results and tumor size.
Main Results:
- Inactive tumors were significantly smaller than active PHEO/PGL.
- Tumors <1.5 cm showed stable disease in 67% of patients.
- Tumor size, metanephrine levels, and a novel symptom score predict tumor behavior.
Conclusions:
- Standardized clinical assessment, including tumor size and metanephrine measurement, is recommended for VHL patients with non-functioning incidentalomas <1.5 cm.
- Annual follow-up is suggested, with individualized intervals based on growth, activity, and symptoms.
- This approach aids in predicting tumor progression and optimizing treatment strategies.
