The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer.
The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer.
复制标题
DOI：
10.1097/mpa.0b013e3181ebb4f0
复制
发表时间：
2010-08
期刊：
影响因子：
2.9
通讯作者：
North American Neuroendocrine Tumor Society (NANETS)
中科院分区：
文献类型：
--
作者：
Chen H;Sippel RS;O'Dorisio MS;Vinik AI;Lloyd RV;Pacak K;North American Neuroendocrine Tumor Society (NANETS)
嗜铬细胞瘤、肾上腺内副神经节瘤和肾上腺外交感神经和副交感神经副神经节瘤分别是源自肾上腺嗜铬细胞或肾上腺外交感神经和副交感神经副神经节中的类似细胞的神经内分泌肿瘤。与这些肿瘤相关的严重发病率和死亡率与儿茶酚胺对各种器官，特别是心血管系统的有效作用有关。在进行任何外科手术之前，术前阻断是必要的，以保护患者免受由于麻醉和肿瘤手术操作而导致的儿茶酚胺的显著释放。治疗方案因疾病的程度而异，腹腔镜手术是切除原发性肿瘤的首选治疗方法。甲状腺髓样癌（MTC）是甲状腺C细胞或滤泡旁细胞的恶性肿瘤。甲状腺c细胞合成许多肽和激素，例如降钙素、癌胚抗原和嗜铬粒蛋白A。这些标记物中的一些或全部在MTC患者中升高，并且可用于确认诊断以及纵向跟踪患者的复发。MTC由一系列疾病组成，范围从多年稳定的极惰性肿瘤到与高死亡率相关的侵袭性类型。RET突变的基因检测允许识别家族性病例和预防性甲状腺切除术以治愈。唯一有效的治疗方法是完全手术切除。
Pheochromocytomas, intra-adrenal paraganglioma, and extra-adrenal sympathetic and parasympathetic paragangliomas are neuroendocrine tumors derived from adrenal chromaffin cells or similar cells in extra-adrenal sympathetic and parasympathetic paraganglia, respectively. Serious morbidity and mortality rates associated with these tumors are related to the potent effects of catecholamines on various organs, especially those of the cardiovascular system. Before any surgical procedure is done, pre-operative blockade is necessary to protect the patient against significant release of catecholamines due to anesthesia and surgical manipulation of the tumor. Treatment options vary with the extent of the disease with laparoscopic surgery being the preferred treatment for removal of primary tumors. Medullary thyroid cancer (MTC) is a malignancy of the thyroid C-cells or parafollicular cells. Thyroid c-cells elaborate a number of peptides and hormones, such as calcitonin, CEA, and chromogranin A. Some or all of these markers are elevated in patients with MTC and can be used to confirm the diagnosis as well as to follow patients longitudinally for recurrence. MTC consists of a spectrum of disease that ranges from extremely indolent tumors that are stable for many years to aggressive types associated with a high mortality rate. Genetic testing for RET mutations has allowed identification of familial cases and prophylactic thyroidectomy for cure. The only curative treatment is complete surgical resection.