ELKH Hereditary Cancer Research Group, 1052 Budapest, Hungary.
Department of Molecular Genetics, National Institute of Oncology, 1122 Budapest, Hungary.
Int J Mol Sci. 2022 Jan 27;23(3):1450. doi: 10.3390/ijms23031450.
Pheochromocytomas and paragangliomas are the most heritable endocrine tumors. In addition to the inherited mutation other driver mutations have also been identified in tumor tissues. All these genetic alterations are clustered in distinct groups which determine the pathomechanisms. Most of these tumors are benign and their surgical removal will resolve patient management. However, 5-15% of them are malignant and therapeutical possibilities for them are limited. This review provides a brief insight about the tumorigenesis associated with pheochromocytomas/paragangliomas in order to present them as potential therapeutical targets.
嗜铬细胞瘤和副神经节瘤是最具遗传性的内分泌肿瘤。除了遗传突变,在肿瘤组织中还发现了其他驱动突变。所有这些遗传改变都集中在不同的群组中,决定了发病机制。这些肿瘤大多数是良性的,通过手术切除可以解决患者的管理问题。然而,其中有 5-15%是恶性的,它们的治疗方法有限。本文简要概述了与嗜铬细胞瘤/副神经节瘤相关的肿瘤发生机制,以将其作为潜在的治疗靶点。