Science and Technology Production
Article
Authorship
Pennisi Patricia
;
Fernández María Celia
;
MARTIN, AYELEN
Date
2020
Publishing House and Editing Place
YS Medical Media Ltd.
Magazine
Pediatric Endocrinology Reviews
- ISSN 1565-4753
YS Medical Media Ltd.
YS Medical Media Ltd.
ISSN
1565-4753
Summary
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Pheochromocytomas/paragangliomas (PCCs/PGLs) are rare neuroendocrine tumors,developed from chromaffin cells derived from the neural crest. From a genetic point of view PCCs/PGLs are divided as sporadic cases, and inherited cases as part of hereditary (familial) syndromes. While the majority is benign, up to 26% of PCCs/PGLs will undergo malignant transformation. Validated prognostic pathological parameters for malignant PCCs/PGLs are still lacking. Signaling that follows the interactions betwee...
Pheochromocytomas/paragangliomas (PCCs/PGLs) are rare neuroendocrine tumors,developed from chromaffin cells derived from the neural crest. From a genetic point of view PCCs/PGLs are divided as sporadic cases, and inherited cases as part of hereditary (familial) syndromes. While the majority is benign, up to 26% of PCCs/PGLs will undergo malignant transformation. Validated prognostic pathological parameters for malignant PCCs/PGLs are still lacking. Signaling that follows the interactions between IGFs and their receptor/s in tumor cells received extensive attention when investigating the role of IGF1R in cancer. Increased IGF1R expression has been shown during progression to metastatic phenotypes and associated with worse prognosis in several types of cancer. In this review we provide evidence supporting a role for the IGFs system on PCC/PGL tumor biology and malignant behavior, endocrine actions to sustain tumor phenotype as well as heterotypic interaction´s regulation by IGF1,encouraging further research for targeted therapeutic options for these tumors.
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Key Words
PHEOCHROMOCYTOMAIGF1RIGF1PARAGANGLIOMA