Pheochromocytoma and Paraganglioma: An Endocrine Society Clinical Practice Guideline
Pheochromocytoma and Paraganglioma: An Endocrine Society Clinical Practice Guideline
复制标题
DOI：
10.1210/jc.2014-1498
复制
发表时间：
2014-06-01
影响因子：
5.8
通讯作者：
Young, William F., Jr.
中科院分区：
文献类型：
--
作者：
Lenders, Jacques W. M.;Duh, Quan-Yang;Young, William F., Jr.
目的：目的是制定嗜铬细胞瘤和paraganglioma（PPGL）的临床实践指南（PPGL）。参与者：工作组包括由内分泌学会临床准则小组委员会（CGS）选择的主席，该领域的七个专家和一名方法学者。作者没有获得公司资金或报酬。实行：该基于证据的指南是使用建议，评估，开发和评估（等级）系统的评分制定的，以描述建议的强度和证据质量。该工作队审查了主要证据，并委托了两个系统的系统评论。CONSENSUS流程：一次小组会议，几次电话会议和电子邮件通信启用了共识。内分泌学会，欧洲内分泌学学会和临床化学协会的委员会和成员审查了指南的草稿。结论：工作队建议对PPGL的初始生化测试应包括血浆免费或尿液分解甲词的测量。应考虑产生假阳性或假阴性结果的放分析因子。所有积极的结果都需要随访。建议用于初始成像的计算机断层扫描，但是磁共振是转移性疾病患者或辐射暴露必须受到限制的更好选择。 I-123-iododobenzylguanidine闪烁显像是转移性PPGL的有用成像方式。我们建议考虑所有患者的基因检测，并由认可的实验室进行测试。副神经瘤患者应测试SDHX突变，患有转移性疾病的患者进行SDHB突变。所有功能性PPGL的患者均应受到术前阻滞，以防止围手术期并发症。准备工作应包括高钠饮食和液体摄入量，以防止术后低血压。我们建议对大多数大多数paragangliomas开放切除术的大多数嗜铬细胞瘤的最小侵入性肾上腺切除术。部分肾上腺切除术是选定患者的一种选择。建议终身随访以检测复发或转移性疾病。我们建议通过具有适当专业知识的多学科团队进行评估和治疗的个性化管理，以确保有利的成果。
Objective: The aim was to formulate clinical practice guidelines for pheochromocytoma and paraganglioma (PPGL).Participants: The Task Force included a chair selected by the Endocrine Society Clinical Guidelines Subcommittee (CGS), seven experts in the field, and a methodologist. The authors received no corporate funding or remuneration.Evidence: This evidence-based guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to describe both the strength of recommendations and the quality of evidence. The Task Force reviewed primary evidence and commissioned two additional systematic reviews.Consensus Process: One group meeting, several conference calls, and e-mail communications enabled consensus. Committees and members of the Endocrine Society, European Society of Endocrinology, and Americal Association for Clinical Chemistry reviewed drafts of the guidelines.Conclusions: The Task Force recommends that initial biochemical testing for PPGLs should include measurements of plasma free or urinary fractionated metanephrines. Consideration should be given to preanalytical factors leading to false-positive or false-negative results. All positive results require follow-up. Computed tomography is suggested for initial imaging, but magnetic resonance is a better option in patients with metastatic disease or when radiation exposure must be limited. I-123-metaiodobenzylguanidine scintigraphy is a useful imaging modality for metastatic PPGLs. We recommend consideration of genetic testing in all patients, with testing by accredited laboratories. Patients with paraganglioma should be tested for SDHx mutations, and those with metastatic disease for SDHB mutations. All patients with functional PPGLs should undergo preoperative blockade to prevent perioperative complications. Preparation should include a high-sodium diet and fluid intake to prevent postoperative hypotension. We recommend minimally invasive adrenalectomy for most pheochromocytomas with open resection for most paragangliomas. Partial adrenalectomy is an option for selected patients. Lifelong follow-up is suggested to detect recurrent or metastatic disease. We suggest personalized management with evaluation and treatment by multidisciplinary teams with appropriate expertise to ensure favorable outcomes.