Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline
Open accessAbstract
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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Minimally invasive cortical-sparing surgery for bilateral pheochromocytomas
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Diagnosis and localization of pheochromocytoma
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False Positive test Results for Pheochromocytoma from 2000 to 2008
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Pheochromocytoma and paraganglioma: imaging characteristics
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HIF2AMutations in Paraganglioma with Polycythemia
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Flagged first, then by citations
Research works printed but not linked: 5
- Lenders JW, Eisenhofer G, Mannelli M, Pacak K. Phaeochromo- cytoma. Lancet. 2005;366:665-675.
- Prejbisz A, Lenders JW, Eisenhofer G, Januszewicz A. Cardiovas- cular manifestations of phaeochromocytoma. J Hypertens. 2011; 29:2049 -2060.
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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
- Publisher
- The Endocrine Society
- 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
- hybrid
- doi.org
- Licence
- 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
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
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