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Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline
Jacques W M Lenders1, Quan-Yang Duh, Graeme Eisenhofer
1Radboud University Medical Center (J.W.M.L.), 6500 HB Nijmegen, The Netherlands; VA Medical Center and University of California, San Francisco (Q.-Y.D.), San Francisco, California 94121; University Hospital Dresden (G.E.), 01307 Dresden, Germany; Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Génétique, (A.-P.G.-R.), F-75015 Paris, France; Université Paris Descartes (A.-P.G.-R.), F-75006 Paris, France; Mayo Clinic (S.K.G.G., M.H.M.), Rochester, Minnesota 55905; National Hospital Organisation Kyoto Medical Center (M.N.), Kyoto 612-8555; Japan; Eunice Kennedy Shriver National Institute of Child Health & Human Development (K.P.), Bethesda, Maryland 20892; and Mayo Clinic (W.F.Y.), Rochester, Minnesota 55905.
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. (123)I-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.