Evidence map›Paper›PMID 34021277›Full record

ReviewNature reviews. Endocrinology2021

International consensus on initial screening and follow-up of asymptomatic SDHx mutation carriers.

Laurence Amar, Karel Pacak, Olivier Steichen, Scott A Akker, Simon J B Aylwin, Eric Baudin, Alexandre Buffet, Nelly Burnichon, Roderick J Clifton-Bligh, Patricia L M Dahia and 20 more

Open access · hybridAbstract readReviewConsensus Statement
In one paragraph

Review in Nature reviews. Endocrinology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 91 papers, 4 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
91citing papers in PubMed, 4 pooled it
25.1field-weighted citation impact, top 1% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

91 citing papers in PubMed, 4 syntheses or guidelines pooled it, 156 citations in OpenAlex.

  1. Clinical characteristics and prognosis ofJournal of medical genetics · 2026
    Pooled it
  2. Guideline
  3. Outcomes of SDHB Pathogenic Variant Carriers.The Journal of clinical endocrinology and metabolism · 2024
    Pooled it
  4. Pooled it
  5. Review
  6. Review
  7. Review
  8. Journal of the Endocrine Society · 2026
    Article
  9. Pheochromocytomas and Paragangliomas.Endocrinology and metabolism clinics of North America · 2026
    Review
  10. Article
  11. Article
  12. Article
  13. Review
  14. Tumor risks and surveillance outcomes inEndocrine oncology (Bristol, England) · 2026
    Article
  15. Article
  16. Review
  17. Review
  18. Article
  19. Article
  20. Approach to the Patient With Metastatic Pheochromocytoma and Paraganglioma.The Journal of clinical endocrinology and metabolism · 2025
    Article

31 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

30 authors at 20 institutions in 11 countries.

Laurence AmarParis University, Hypertension unit, Hôpital Européen Georges Pompidou, AP-HP, Paris, France. laurence.amar@aphp.fr.ORCID http://orcid.org/0000-0003-3942-4276
Karel PacakEunice Kennedy Shriver NICHD, NIH, Bethesda, MD, USA.ORCID http://orcid.org/0000-0002-3541-3767
Olivier SteichenSorbonne University, Department of Internal Medicine, Hôpital Tenon, AP-HP, Paris, France.ORCID http://orcid.org/0000-0002-1185-0372
Scott A AkkerSt Bartholomew's Hospital, Barts Health NHS Trust, London, UK.
Simon J B AylwinKing's College Hospital NHS Foundation Trust, London, UK.
Eric BaudinGustave Roussy Institute and Paris Saclay University, Villejuif, France.
Alexandre BuffetINSERM, PARCC, Equipe Labellisée par la Ligue contre le Cancer, Paris, France.
Nelly BurnichonINSERM, PARCC, Equipe Labellisée par la Ligue contre le Cancer, Paris, France.
Roderick J Clifton-BlighDepartment of Endocrinology, Royal North Shore Hospital, St Leonards, NSW, Australia.ORCID http://orcid.org/0000-0002-1545-0368
Patricia L M DahiaDepartment of Medicine, Mays Cancer Center, University of Texas Health Science Center at San Antonio, San Antonio, TX, USA.ORCID http://orcid.org/0000-0002-7757-370X
Martin FassnachtDepartment of Internal Medicine, Division of Endocrinology and Diabetes, University Hospital, University of Würzburg, Würzburg, Germany.ORCID http://orcid.org/0000-0001-6170-6398
Ashley B GrossmanOxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, Oxford, UK.
Philippe HermanENT unit, Lariboisière Hospital, AP-HP, University of Paris, Paris, France.
Rodney J HicksPeter MacCallum Cancer Centre, The University of Melbourne, Melbourne, VIC, Australia.ORCID http://orcid.org/0000-0002-0758-0824
Andrzej JanuszewiczDepartment of Hypertension, National Institute of Cardiology, Warsaw, Poland.
Camilo JimenezDepartment of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Henricus P M KunstDepartment of ENT, Radboud University Medical Center, Nijmegen, Netherlands.
Dylan LewisKing's College Hospital NHS Foundation Trust, London, UK.
Massimo MannelliDepartment of Experimental and Clinical Biomedical Sciences, University of Florence, Florence, Italy.
Mitsuhide NaruseEndocrine Center, Ijinkai Takeda General Hospital and Clinical Research Institute, NHO Kyoto Medical Center, Kyoto, Japan.ORCID http://orcid.org/0000-0002-8977-4090
Mercedes RobledoHereditary Endocrine Cancer Group. Spanish National Cancer Research Center (CNIO), Madrid, Spain.ORCID http://orcid.org/0000-0001-6256-5902
David TaïebAix-Marseille University, La Timone university hospital, European Center for Research in Medical Imaging, Marseille, France.
David R TaylorKing's College Hospital NHS Foundation Trust, London, UK.
Henri J L M TimmersDepartment of Internal Medicine, Radboud University Medical Center, Nijmegen, Netherlands.
Giorgio TregliaEnte Ospedaliero Cantonale, Bellinzona, Switzerland.
Nicola TuftonSt Bartholomew's Hospital, Barts Health NHS Trust, London, UK.
William F YoungDivision of Endocrinology, Diabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, MN, USA.
Jacques W M LendersDepartment of Internal Medicine, Radboud University Medical Center, Nijmegen, Netherlands.
Anne-Paule Gimenez-RoqueploINSERM, PARCC, Equipe Labellisée par la Ligue contre le Cancer, Paris, France.ORCID http://orcid.org/0000-0002-4816-670X
Charlotte Lussey-LepoutreINSERM, PARCC, Equipe Labellisée par la Ligue contre le Cancer, Paris, France. charlotte.lussey@inserm.fr.ORCID http://orcid.org/0000-0003-2228-0106
Inserm · FRKing's College Hospital NHS Foundation Trust · GBRadboud University Nijmegen · NLSt Bartholomew's Hospital · GBAix-Marseille Université · FREnte Ospedaliero Cantonale · CHEunice Kennedy Shriver National Institute of Child Health and Human Development · USInstitute of Cardiology · PLKyoto Medical Center · JPMayo Clinic in Arizona · USSorbonne Université · FRSpanish National Cancer Research Centre · ESThe Royal Free Hospital · GBThe University of Melbourne · AUThe University of Sydney · AUThe University of Texas Health Science Center at San Antonio · USThe University of Texas MD Anderson Cancer Center · USUniversité Paris Cité · FRUniversité Paris-Saclay · FRUniversity of Florence · IT

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Approximately 20% of patients diagnosed with a phaeochromocytoma or paraganglioma carry a germline mutation in one of the succinate dehydrogenase (SDHx) genes (SDHA, SDHB, SDHC and SDHD), which encode the four subunits of the SDH enzyme. When a pathogenic SDHx mutation is identified in an affected patient, genetic counselling is proposed for first-degree relatives. Optimal initial evaluation and follow-up of people who are asymptomatic but might carry SDHx mutations have not yet been agreed. Thus, we established an international consensus algorithm of clinical, biochemical and imaging screening at diagnosis and during surveillance for both adults and children. An international panel of 29 experts from 12 countries was assembled, and the Delphi method was used to reach a consensus on 41 statements. This Consensus Statement covers a range of topics, including age of first genetic testing, appropriate biochemical and imaging tests for initial tumour screening and follow-up, screening for rare SDHx-related tumours and management of elderly people who have an SDHx mutation. This Consensus Statement focuses on the management of asymptomatic SDHx mutation carriers and provides clinicians with much-needed guidance. The standardization of practice will enable prospective studies in the near future.

Indexed as

AdultAgedAlgorithmsAsymptomatic DiseasesChildConsensusGenetic Carrier ScreeningGenetic TestingGerm-Line MutationHeterozygoteHumansInternationalityMass ScreeningMonitoring, PhysiologicSuccinate DehydrogenaseSuccinate Dehydrogenase

Identifiers

PMID34021277
PMCPMC8205850
OpenAlexW3162562470

What OpenQuestion holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.