Abstract：
Pheochromocytomas and paragangliomas are rare neuroendocrine tumors, developed respectively in the adrenal medulla and in extra-adrenal locations. Their malignancy is defined by the presence of distant metastases. Forty percent of them are inherited and can be part of different hereditary syndromes. Their management is ensured in France by the multidisciplinary expert centers of the ENDOCAN-COMETE national network \"Cancers of the Adrenal gland\", certified by the National Cancer Institute and discussed within multidisciplinary team meetings. The diagnostic and therapeutic work-up must be standardized, based on an expert analysis of clinical symptoms, hormonal biological secretions, genetics, morphological and specific metabolic imaging. In the context of a heterogeneous survival sometimes beyond seven to ten years, therapeutic intervention must be justified. This is multidisciplinary and relies on surgery, interventional radiology, external or internal radiotherapy and medical treatments such as sunitinib or dacarbazine and temodal chemotherapy. The personalized approach based on functional imaging fixation status and genetics is progressing despite the extreme rarity of this disease.
摘要：
嗜铬细胞瘤和副神经节瘤是罕见的神经内分泌肿瘤，分别在肾上腺髓质和肾上腺外位置发展。它们的恶性由远处转移的存在定义。其中40％是遗传的，可能是不同遗传综合征的一部分。他们的管理在法国由ENDOCAN-COMETE国家网络“肾上腺癌”的多学科专家中心确保，由国家癌症研究所认证，并在多学科小组会议上讨论。诊断和治疗工作必须标准化，根据专家对临床症状的分析，荷尔蒙生物分泌物,遗传学,形态学和特异性代谢成像。在异质生存的背景下，有时超过七到十年，治疗干预必须是合理的。这是多学科的，依赖于手术，介入放射学,外部或内部放疗和药物治疗，如舒尼替尼或达卡巴嗪和temodal化疗。尽管这种疾病极为罕见，但基于功能成像固定状态和遗传学的个性化方法仍在进步。
