Management of pheochromocytomas and paragangliomas: Review of current diagnosis and treatment options

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Authors

EID Michal FOUKAL Jakub SOCHOROVÁ Dana TUČEK Štěpán STARÝ Karel KALA Zdeněk MAYER Jiří NĚMEČEK Radim TRNA Jan KUNOVSKÝ Lumír

Year of publication 2023
Type Article in Periodical
Magazine / Source Cancer Medicine
MU Faculty or unit

Faculty of Medicine

Citation
Web https://onlinelibrary.wiley.com/doi/10.1002/cam4.6010
Doi http://dx.doi.org/10.1002/cam4.6010
Keywords biomarkers; chemotherapy; molecular biology; prognostic factor; surgery; target therapy
Description Pheochromocytomas (PCCs) are rare neuroendocrine tumors derived from the chromaffin cells of the adrenal medulla. When these tumors have an extra-adrenal location, they are called paragangliomas (PGLs) and arise from sympathetic and parasympathetic ganglia, particularly of the para-aortic location. Up to 25% of PCCs/PGLs are associated with inherited genetic disorders. The majority of PCCs/PGLs exhibit indolent behavior. However, according to their affiliation to molecular clusters based on underlying genetic aberrations, their tumorigenesis, location, clinical symptomatology, and potential to metastasize are heterogenous. Thus, PCCs/PGLs are often associated with diagnostic difficulties. In recent years, extensive research revealed a broad genetic background and multiple signaling pathways leading to tumor development. Along with this, the diagnostic and therapeutic options were also expanded. In this review, we focus on the current knowledge and recent advancements in the diagnosis and treatment of PCCs/PGLs with respect to the underlying gene alterations while also discussing future perspectives in this field.
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