The DOI matches its Crossref record; 196 of the 196 references it cites were found in a registry or an index.
Personalized Management of Pheochromocytoma and Paraganglioma
Open accessAbstract
Pheochromocytomas/paragangliomas are characterized by a unique molecular landscape that allows their assignment to clusters based on underlying genetic alterations. With around 30% to 35% of Caucasian patients (a lower percentage in the Chinese population) showing germline mutations in susceptibility genes, pheochromocytomas/paragangliomas have the highest rate of heritability among all tumors. A further 35% to 40% of Caucasian patients (a higher percentage in the Chinese population) are affected by somatic driver mutations. Thus, around 70% of all patients with pheochromocytoma/paraganglioma can be assigned to 1 of 3 main molecular clusters with different phenotypes and clinical behavior. Krebs cycle/VHL/EPAS1-related cluster 1 tumors tend to a noradrenergic biochemical phenotype and require very close follow-up due to the risk of metastasis and recurrence. In contrast, kinase signaling–related cluster 2 tumors are characterized by an adrenergic phenotype and episodic symptoms, with generally a less aggressive course. The clinical correlates of patients with Wnt signaling–related cluster 3 tumors are currently poorly described, but aggressive behavior seems likely. In this review, we explore and explain why cluster-specific (personalized) management of pheochromocytoma/paraganglioma is essential to ascertain clinical behavior and prognosis, guide individual diagnostic procedures (biochemical interpretation, choice of the most sensitive imaging modalities), and provide personalized management and follow-up. Although cluster-specific therapy of inoperable/metastatic disease has not yet entered routine clinical practice, we suggest that informed personalized genetic-driven treatment should be implemented as a logical next step. This review amalgamates published guidelines and expert views within each cluster for a coherent individualized patient management plan.
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Cites
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Background
Cited by
Wang, Y., Liu, B., Li, F., Zhang, Y., Gao, X., Wang, Y., & Zhou, H. (2023) · Frontiers in Endocrinology · doi:10.3389/fendo.2023.1274239 · Cited by 18
Metadata
- Authors
- Nölting, S., Bechmann, N., Taieb, D., Beuschlein, F., Fassnacht, M., Kroiss, M., Eisenhofer, G., Grossman, A., & Pacak, K.
- Venue
- Endocrine Reviews
- Publisher
- The Endocrine Society
- Published
- 2021-06-19
- Volume
- 43
- Issue
- 2
- Pages
- 199-239
- ISSN
- 0163-769X (ISSN-L)
- 1945-7189
- OpenAlex
- W3166036810
- Cited by
- 417
- References
- 196
- FWCI
- 52.75
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Nölting, S., Bechmann, N., Taieb, D., Beuschlein, F., Fassnacht, M., Kroiss, M., Eisenhofer, G., Grossman, A., & Pacak, K. (2022). Personalized Management of Pheochromocytoma and Paraganglioma. Endocrine Reviews. https://doi.org/10.1210/endrev/bnab019
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