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Recent progress in the pathologic classification of pheochromocytomas and paragangliomas
Ozgur Mete1, C Christofer Juhlin2
1Department of Pathology, University Health Network, Toronto, ON, Canada; Department of Laboratory Medicine and Pathobiology, University of Toronto, Temerty Faculty of Medicine, Toronto, ON, Canada; Endocrine Oncology Site, Princess Margaret Cancer Centre, Toronto, ON, Canada.
Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors with diverse genetics. Modern pathology integrates morphology, biomarkers, and genetics for accurate diagnosis and risk assessment of metastatic potential.
Area of Science:
- Endocrinology
- Oncology
- Pathology
Background:
- Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine neoplasms with significant genetic diversity.
- These tumors can secrete catecholamines and carry a risk of metastatic spread, similar to epithelial neuroendocrine neoplasms.
Purpose of the Study:
- To provide pathologists with an update on diagnostic and risk assessment strategies for PPGLs.
- To emphasize the integration of morphology, biomarkers, and genetic profiling in PPGL assessment.
Main Methods:
- Review of advances in understanding PPGL biology and genetics.
- Focus on genotype-phenotype correlations guiding diagnosis and prognostication.
- Integration of morphology with functional, structural, and pathogenesis-related biomarker studies.
Main Results:
- Robust genotype-phenotype correlations aid in diagnosis and prognostication.
- The 2022 WHO classification promotes integrated diagnostic approaches.
- Germline mutations are observed in at least 40% of PPGL cases, impacting risk assessment.
Conclusions:
- Modern PPGL diagnosis requires integrating morphology with biomarker and genetic studies.
- Distinguishing metastatic disease from multifocal tumors is a key challenge.
- Improved multidisciplinary care is essential for patients with PPGLs.
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