Cited by 2,827Adrenal and Paraganglionic TumorsNeuroendocrine Tumor Research AdvancesRenal cell carcinoma treatment

Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline

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Lenders, Jacques W. M., Duh, Quan-Yang, Eisenhofer, Graeme, Gimenez-Roqueplo, Anne-Paule, Grebe, Stefan K. G., Murad, Mohammad Hassan, Naruse, Mitsuhide, Pacak, Karel +1 more·2014·The Journal of Clinical Endocrinology & Metabolism

Abstract

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.

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  • SDHB (10.3%) and SDHD (8.9%) mutations are the most frequent germline mutations in pheochromocytoma and paragangliomas.

    “SDHB (10.3%) and SDHD (8.9%) mutations are the most frequent germline mutations in pheochromocytoma and paragangliomas (180).”

    From Adrenal incidentaloma (2020) · cites this paper as [180]

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Research works printed but not linked: 5

  1. Lenders JW, Eisenhofer G, Mannelli M, Pacak K. Phaeochromo- cytoma. Lancet. 2005;366:665-675.
  2. Prejbisz A, Lenders JW, Eisenhofer G, Januszewicz A. Cardiovas- cular manifestations of phaeochromocytoma. J Hypertens. 2011; 29:2049 -2060.
  3. Manu P, Runge LA. Biochemical screening for pheochromocy- toma. Superiority of urinary metanephrines measurements. Am J Epidemiol. 1984;120:788 -790.
  4. Därr R, Lenders JW, Stange K, et al. Diagnosis of pheochromocy- toma and paraganglioma: the clonidine suppression test in patients with borderline elevations of plasma free normetanephrine [in Ger- man]. Dtsch Med Wochenschr. 2013;138:76 -81.
  5. Shao Y, Chen R, Shen ZJ, et al. Preoperative ␣ blockade for nor- motensive pheochromocytoma: is it necessary? J Hypertens. 2011; 29:2429 -2432.

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Authors
Lenders, J. W. M., Duh, Q.-Y., Eisenhofer, G., Gimenez-Roqueplo, A.-P., Grebe, S. K. G., Murad, M. H., Naruse, M., Pacak, K., & Young, W. F.
Affiliations
  • Radboud University Medical Center (NL)
  • San Francisco VA Medical Center (US)
  • 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;
  • Mayo Clinic (US)
  • Kyoto Medical Center (JP)
  • Eunice Kennedy Shriver National Institute of Child Health & Human Development (K.P.), Bethesda, Maryland 20892;
Venue
The Journal of Clinical Endocrinology & Metabolism
Published
2014-06-01
Volume
99
Issue
6
Pages
1915-1942
ISSN
  • 0021-972X (ISSN-L)
  • 1945-7197
OpenAlex
W2135003179
Cited by
2,827
References
267
Citations by year
2026: 208 · 2025: 251 · 2024: 253 · 2023: 265 · 2022: 299 · 2021: 321 · 2020: 292 · 2019: 250 · 2018: 217 · 2017: 181 · 2016: 140 · 2015: 121 · 2014: 23
FWCI
153.60
Type
journal article
Language
en
Open access
Locations
  • The Journal of Clinical Endocrinology & Metabolism (publishedVersion, cc-by-nc-nd)
  • PubMed (publishedVersion)
  • eScholarship (California Digital Library) (submittedVersion)
  • Radboud Repository (Radboud University) (submittedVersion)
Funders
  • European Society of Endocrinology
  • Society for Endocrinology
Topics
  • Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
  • Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Renal cell carcinoma treatment
Keywords
pheochromocytoma, paraganglioma, metanephrine, pre-analytical variables, 123I-MIBG scintigraphy, genetic testing, SDHB mutation, SDHx mutations, adrenalectomy, partial adrenalectomy, metastatic disease, multidisciplinary management, lifelong follow-up
MeSH terms
Adrenal Gland Neoplasms, Adrenalectomy, Combined Modality Therapy, Diagnostic Techniques, Endocrine, Diagnostic Imaging, Endocrinology, Humans, Paraganglioma, Pheochromocytoma, Evidence-Based Medicine, Perioperative Care, Consensus, Precision Medicine

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