Pennisi Patricia A, Fernández María Celia, Martin Ayelen
Centro de Investigaciones Endocrinológicas "Dr. César Bergadá" CEDIE, CONICET - FEI - División de Endocrinología, Hospital de Niños Ricardo Gutiérrez, Gallo 1330 - C1425EFD Buenos Aires, Argentina, E-mail:
Centro de Investigaciones Endocrinológicas "Dr. César Bergadá" CEDIE CONICET - FEI - División de Endocrinología, Hospital de Niños Ricardo Gutiérrez, Gallo 1330 - C1425EFD Buenos Aires, Argentina.
Pediatr Endocrinol Rev. 2020 Aug;17(4):331-342. doi: 10.17458/per.vol17.2020.kc.insightspheochromocytomabiology.
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.
嗜铬细胞瘤/副神经节瘤（PCCs/PGLs）是一种罕见的神经内分泌肿瘤，由神经嵴来源的嗜铬细胞发展而来。从遗传学角度来看，PCCs/PGLs分为散发性病例和作为遗传性（家族性）综合征一部分的遗传性病例。虽然大多数是良性的，但高达26%的PCCs/PGLs会发生恶性转化。目前仍缺乏针对恶性PCCs/PGLs的经过验证的预后病理参数。在研究IGF1R在癌症中的作用时，肿瘤细胞中IGFs与其受体之间相互作用所引发的信号传导受到了广泛关注。在进展为转移表型的过程中，IGF1R表达增加，并且在几种类型的癌症中与较差的预后相关。在本综述中，我们提供了证据，支持IGFs系统在PCC/PGL肿瘤生物学和恶性行为中的作用、维持肿瘤表型的内分泌作用以及IGF1对异型相互作用的调节，鼓励对这些肿瘤的靶向治疗选择进行进一步研究。
