Pheochromocytoma and Paraganglioma: An Endocrine Society Clinical Practice Guideline

Pheochromocytoma and Paraganglioma: An Endocrine Society Clinical Practice Guideline

June 2014 | Jacques W. M. Lenders, Quan-Yang Duh, Graeme Eisenhofer, Anne-Paule Gimenez-Roqueplo, Stefan K. G. Grebe, Mohammad Hassan Murad, Mitsuhide Naruse, Karel Pacak, and William F. Young, Jr
The Endocrine Society has issued a clinical practice guideline for pheochromocytoma and paraganglioma (PPGL). The guideline recommends initial biochemical testing for PPGLs should include measurements of plasma free or urinary fractionated metanephrines. It suggests using liquid chromatography with mass spectrometric or electrochemical detection methods for diagnosis. Blood should be drawn with the patient in the supine position, and reference intervals should be established in the same position. 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. $ ^{123} $ I-metaio-dobenzylguanidine scintigraphy is a useful imaging modality for metastatic PPGLs. Genetic testing is recommended for 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. Minimally invasive adrenalectomy is recommended for most pheochromocytomas, while open resection is recommended for most paragangliomas. Lifelong follow-up is suggested to detect recurrent or metastatic disease. Personalized management with evaluation and treatment by multidisciplinary teams is recommended to ensure favorable outcomes. The guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system. The Task Force reviewed primary evidence and commissioned two additional systematic reviews. Consensus was reached through one group meeting, several conference calls, and e-mail communications. Committees and members of the Endocrine Society, European Society of Endocrinology, and American Association for Clinical Chemistry reviewed drafts of the guidelines. The guideline emphasizes the importance of biochemical testing, imaging studies, genetic testing, perioperative medical management, surgery, and personalized management for PPGLs. It recommends that all patients with PPGLs should be engaged in shared decision making for genetic testing. A clinical feature-driven diagnostic algorithm is recommended to establish priorities for specific genetic testing in PPGL patients with suspected germline mutations. 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. Minimally invasive adrenalectomy is recommended for most pheochromocytomas, while open resection is recommended for most paragangliomas. Lifelong follow-up is suggested to detect recurrent or metastatic disease. Personalized management with evaluation and treatment by multidisciplinary teams isThe Endocrine Society has issued a clinical practice guideline for pheochromocytoma and paraganglioma (PPGL). The guideline recommends initial biochemical testing for PPGLs should include measurements of plasma free or urinary fractionated metanephrines. It suggests using liquid chromatography with mass spectrometric or electrochemical detection methods for diagnosis. Blood should be drawn with the patient in the supine position, and reference intervals should be established in the same position. 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. $ ^{123} $ I-metaio-dobenzylguanidine scintigraphy is a useful imaging modality for metastatic PPGLs. Genetic testing is recommended for 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. Minimally invasive adrenalectomy is recommended for most pheochromocytomas, while open resection is recommended for most paragangliomas. Lifelong follow-up is suggested to detect recurrent or metastatic disease. Personalized management with evaluation and treatment by multidisciplinary teams is recommended to ensure favorable outcomes. The guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system. The Task Force reviewed primary evidence and commissioned two additional systematic reviews. Consensus was reached through one group meeting, several conference calls, and e-mail communications. Committees and members of the Endocrine Society, European Society of Endocrinology, and American Association for Clinical Chemistry reviewed drafts of the guidelines. The guideline emphasizes the importance of biochemical testing, imaging studies, genetic testing, perioperative medical management, surgery, and personalized management for PPGLs. It recommends that all patients with PPGLs should be engaged in shared decision making for genetic testing. A clinical feature-driven diagnostic algorithm is recommended to establish priorities for specific genetic testing in PPGL patients with suspected germline mutations. 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. Minimally invasive adrenalectomy is recommended for most pheochromocytomas, while open resection is recommended for most paragangliomas. Lifelong follow-up is suggested to detect recurrent or metastatic disease. Personalized management with evaluation and treatment by multidisciplinary teams is
Reach us at info@study.space
[slides and audio] Pheochromocytoma and paraganglioma%3A an endocrine society clinical practice guideline.