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神经内分泌肿瘤的诊断与治疗——13例临床病例系列(2014 - 2017年)

Diagnosis and treatment of neuroendocrine tumors - A series of 13 clinical cases (2014-2017).

作者信息

Sepúlveda Santos Tiago, Figueira Alberto, Rocha José, Coutinho João, Carvalho Leonor, Ducla-Soares J L

机构信息

Medicine I-C Department, CHLN, Centro Académico de Medicina de Lisboa, Portugal.

General Surgery Department, CHLN, Centro Académico de Medicina de Lisboa, Portugal.

出版信息

Int J Cardiol Hypertens. 2019 Jul 19;2:100019. doi: 10.1016/j.ijchy.2019.100019. eCollection 2019 Aug.

DOI:10.1016/j.ijchy.2019.100019
PMID:33447750
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7803053/
Abstract

Paragangliomas and pheocromocytomas are rare neuroendocrine tumors with different clinical presentation, being responsible for secondary arterial hypertension with target-organ lesions. Surgery is a curative therapy in these tumors and demands a multidisciplinary approach. These tumors are more frequent between the 4th and 5th decades of life and their clinical manifestations are related to catecholamines production: headache, palpitations, variable blood pressure. This article presents 13 clinical cases of patients with neuroendocrine tumors, with an median-age of 56,7 years, submitted to surgery between 2014 and 2017. The diagnosis was made based on clinical suspicion, serum and urinary catecholamines and metanephrins, imagiologic evaluation with CT or abdominal and pelvic MRI and MIBG scintigraphy. After surgery, the majority of patients remained with normal blood pressure, without anti-hypertensive therapy and follow-up was maintained in Outpatient Clinic, with periodic blood tests and imaging reevaluation.

摘要

副神经节瘤和嗜铬细胞瘤是罕见的神经内分泌肿瘤,临床表现各异,可导致继发性动脉高血压并伴有靶器官损害。手术是这些肿瘤的治愈性疗法,需要多学科方法。这些肿瘤在40至50岁之间更为常见,其临床表现与儿茶酚胺分泌有关:头痛、心悸、血压波动。本文介绍了13例神经内分泌肿瘤患者的临床病例,中位年龄为56.7岁,于2014年至2017年接受手术。诊断基于临床怀疑、血清和尿儿茶酚胺及甲氧基肾上腺素、CT或腹部及盆腔MRI影像学评估以及间碘苄胍闪烁显像。手术后,大多数患者血压恢复正常,无需抗高血压治疗,在门诊进行随访,定期进行血液检查和影像学复查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d642/7803053/baab2cb09af1/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d642/7803053/0000568d3bec/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d642/7803053/baab2cb09af1/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d642/7803053/0000568d3bec/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d642/7803053/baab2cb09af1/gr2.jpg

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Int J Surg. 2019 Jan;61:26-32. doi: 10.1016/j.ijsu.2018.11.018. Epub 2018 Nov 29.
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Risk factors for postoperative severe morbidity after pheochromocytoma surgery: A single center retrospective analysis of 262 patients.术后去甲肾上腺素细胞瘤手术后严重发病率的风险因素:对 262 名患者的单中心回顾性分析。
Int J Surg. 2018 Dec;60:188-193. doi: 10.1016/j.ijsu.2018.11.019. Epub 2018 Nov 20.
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The clinical genetics of phaeochromocytoma and paraganglioma.
嗜铬细胞瘤和副神经节瘤的临床遗传学
Arch Endocrinol Metab. 2017 Oct;61(5):490-500. doi: 10.1590/2359-3997000000299.
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Preoperative genetic testing in pheochromocytomas and paragangliomas influences the surgical approach and the extent of adrenal surgery.嗜铬细胞瘤和副神经节瘤的术前基因检测会影响手术方式及肾上腺手术范围。
Surgery. 2018 Jan;163(1):191-196. doi: 10.1016/j.surg.2017.05.025. Epub 2017 Nov 7.
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ACCURACY OF PLASMA FREE METANEPHRINES IN THE DIAGNOSIS OF PHEOCHROMOCYTOMA AND PARAGANGLIOMA: A SYSTEMATIC REVIEW AND META-ANALYSIS.血浆游离甲氧基肾上腺素在嗜铬细胞瘤和副神经节瘤诊断中的准确性:一项系统评价和荟萃分析
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