International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents

Ruth T Casey^{1

2}, Emile Hendriks³, Cheri Deal⁴, Steven G Waguespack⁵, Verena Wiegering⁶, Antje Redlich⁷, Scott Akker⁸, Rathi Prasad⁹, Martin Fassnacht¹⁰, Roderick Clifton-Bligh¹¹, Laurence Amar^{12

13}, Stefan Bornstein¹⁴, Letizia Canu^{15

16}, Evangelia Charmandari¹⁷, Alexandra Chrisoulidou¹⁸, Maria Currás Freixes¹⁹, Ronald de Krijger^{20

21}, Luisa de Sanctis²², Antonio Fojo²³, Amol J Ghia²⁴, Angela Huebner²⁵, Vasilis Kosmoliaptsis^{26

27}, Michaela Kuhlen²⁸, Marco Raffaelli^{29

30}, Charlotte Lussey-Lepoutre³¹, Stephen D Marks³², Naris Nilubol³³, Mirko Parasiliti-Caprino³⁴, Henri H J L M Timmers³⁵, Anna Lena Zietlow³⁶, Mercedes Robledo¹⁹, Anne-Paule Gimenez-Roqueplo^{37

38}, Ashley B Grossman^{39

40

41}, David Taïeb⁴², Eamonn R Maher⁴³, Jacques W M Lenders³⁵, Graeme Eisenhofer¹⁴, Camilo Jimenez⁵, Karel Pacak⁴⁴, Christina Pamporaki⁴⁵

Affiliations

¹ Department of Medical Genetics, University of Cambridge and NIHR Cambridge Biomedical Research Centre, Cambridge, UK. rc674@cam.ac.uk.
² Department of Endocrinology, Cambridge Cancer Centre and Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK. rc674@cam.ac.uk.
³ Department of Paediatric Diabetes and Endocrinology, Cambridge Cancer Centre and Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.
⁴ Endocrine and Diabetes Service, CHU Sainte-Justine and University of Montreal, Montreal, Québec, Canada.
⁵ Department of Endocrine Neoplasia and Hormonal Disorders, University of Texas MD Anderson Cancer Center, Houston, TX, USA.
⁶ University Children's Hospital, Department of Paediatric Hematology, Oncology and Stem Cell Transplantation, University of Würzburg, Würzburg, Germany.
⁷ Paediatric Oncology Department, Otto von Guericke University Children's Hospital, Magdeburg, Germany.
⁸ St Bartholomew's Hospital, Barts Health NHS Trust, London, UK.
⁹ Centre for Endocrinology, William Harvey Research Institute, Queen Mary University of London, London, UK.
¹⁰ Department of Medicine, Division of Endocrinology and Diabetes, University Hospital, University of Würzburg, Würzburg, Germany.
¹¹ Department of Diabetes and Endocrinology, Royal North Shore Hospital, Sydney, New South Wales, Australia.
¹² Université de Paris, Paris, France.
¹³ Hypertension Unit, Hôpital Européen Georges Pompidou, Assistance Publique - Hôpitaux de Paris, Paris, France.
¹⁴ Department of Medicine III, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
¹⁵ Department of Experimental and Clinical Biomedical Sciences "Mario Serio", University of Florence, Florence, Italy.
¹⁶ Centro di Ricerca e Innovazione sulle Patologie Surrenaliche, Azienda Ospedaliera Universitaria (AOU) Careggi, Florence, Italy.
¹⁷ Division of Endocrinology, Metabolism and Diabetes, First Department of Paediatrics, National and Kapodistrian University of Athens Medical School, 'Aghia Sophia' Children's Hospital, Athens, Greece.
¹⁸ Unit of Endocrinology, Theagenio Hospital, Thessaloniki, Greece.
¹⁹ Hereditary Endocrine Cancer Group, Spanish National Cancer Research Centre (CNIO) and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Institute of Health Carlos III (ISCIII), Madrid, Spain.
²⁰ Princess Maxima Center for Paediatric Oncology, Utrecht, Netherlands.
²¹ Department of Pathology, University Medical Center Utrecht, Utrecht, Netherlands.
²² Department of Public Health and Paediatric Sciences, University of Turin, Turin, Italy.
²³ Division of Hematology/Oncology, Herbert Irving Comprehensive Cancer Center, Columbia University Irving Medical Center, New York, NY, USA.
²⁴ Department of Radiation Oncology, University Hospital of Texas, MD Anderson Cancer Center, Houston, TX, USA.
²⁵ Department of Paediatrics, Faculty of Medicine and University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
²⁶ Department of Surgery, University of Cambridge and National Institute for Health Research Cambridge Biomedical Research Centre, Addenbrooke's Hospital, Cambridge, UK.
²⁷ Blood and Transplant Research Unit in Organ Donation and Transplantation, National Institute for Health Research, University of Cambridge, Cambridge, UK.
²⁸ Paediatrics and Adolescent Medicine, Faculty of Medicine, University of Augsburg, Augsburg, Germany.
²⁹ U.O.C. Chirurgia Endocrina e Metabolica, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
³⁰ Istituto di Semeiotica Chirurgica, Università Cattolica del Sacro Cuore, Rome, Italy.
³¹ Service de médecine nucléaire, Inserm U970, Sorbonne université, Groupe hospitalier Pitié-Salpétrière, Paris, France.
³² Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust and NIHR GOSH Biomedical Research Centre, University College London Great Ormond Street Institute of Child Health, London, UK.
³³ Surgical Oncology Program, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
³⁴ Endocrinology, Diabetes and Metabolism, Department of Medical Sciences, University of Turin, Corso Dogliotti, Turin, Italy.
³⁵ Department of Internal Medicine, Radboud University Medical Centre, Nijmegen, Netherlands.
³⁶ Clinical Child and Adolescent Psychology, Institute of Clinical Psychology and Psychotherapy, Department of Psychology, TU Dresden, Dresden, Germany.
³⁷ Université Paris Cité, PARCC, INSERM, Paris, France.
³⁸ Service de Génétique, Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Paris, France.
³⁹ Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK.
⁴⁰ Centre for Endocrinology, Barts and the London School of Medicine, London, UK.
⁴¹ ENETS Centre of Excellence, Royal Free Hospital, London, UK.
⁴² Department of Nuclear Medicine, La Timone University Hospital, CERIMED, Aix-Marseille University, Marseille, France.
⁴³ Department of Medical Genetics, University of Cambridge and NIHR Cambridge Biomedical Research Centre, Cambridge, UK.
⁴⁴ Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Rockville, MD, USA.
⁴⁵ Department of Medicine III, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany. Christina.Pamporaki@ukdd.de.

PMID: 39147856
DOI: 10.1038/s41574-024-01024-5

Review

International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents

Ruth T Casey et al. Nat Rev Endocrinol. 2024 Dec.

. 2024 Dec;20(12):729-748.

doi: 10.1038/s41574-024-01024-5. Epub 2024 Aug 15.

Authors

Affiliations

¹ Department of Medical Genetics, University of Cambridge and NIHR Cambridge Biomedical Research Centre, Cambridge, UK. rc674@cam.ac.uk.
² Department of Endocrinology, Cambridge Cancer Centre and Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK. rc674@cam.ac.uk.
³ Department of Paediatric Diabetes and Endocrinology, Cambridge Cancer Centre and Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.
⁴ Endocrine and Diabetes Service, CHU Sainte-Justine and University of Montreal, Montreal, Québec, Canada.
⁵ Department of Endocrine Neoplasia and Hormonal Disorders, University of Texas MD Anderson Cancer Center, Houston, TX, USA.
⁶ University Children's Hospital, Department of Paediatric Hematology, Oncology and Stem Cell Transplantation, University of Würzburg, Würzburg, Germany.
⁷ Paediatric Oncology Department, Otto von Guericke University Children's Hospital, Magdeburg, Germany.
⁸ St Bartholomew's Hospital, Barts Health NHS Trust, London, UK.
⁹ Centre for Endocrinology, William Harvey Research Institute, Queen Mary University of London, London, UK.
¹⁰ Department of Medicine, Division of Endocrinology and Diabetes, University Hospital, University of Würzburg, Würzburg, Germany.
¹¹ Department of Diabetes and Endocrinology, Royal North Shore Hospital, Sydney, New South Wales, Australia.
¹² Université de Paris, Paris, France.
¹³ Hypertension Unit, Hôpital Européen Georges Pompidou, Assistance Publique - Hôpitaux de Paris, Paris, France.
¹⁴ Department of Medicine III, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
¹⁵ Department of Experimental and Clinical Biomedical Sciences "Mario Serio", University of Florence, Florence, Italy.
¹⁶ Centro di Ricerca e Innovazione sulle Patologie Surrenaliche, Azienda Ospedaliera Universitaria (AOU) Careggi, Florence, Italy.
¹⁷ Division of Endocrinology, Metabolism and Diabetes, First Department of Paediatrics, National and Kapodistrian University of Athens Medical School, 'Aghia Sophia' Children's Hospital, Athens, Greece.
¹⁸ Unit of Endocrinology, Theagenio Hospital, Thessaloniki, Greece.
¹⁹ Hereditary Endocrine Cancer Group, Spanish National Cancer Research Centre (CNIO) and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Institute of Health Carlos III (ISCIII), Madrid, Spain.
²⁰ Princess Maxima Center for Paediatric Oncology, Utrecht, Netherlands.
²¹ Department of Pathology, University Medical Center Utrecht, Utrecht, Netherlands.
²² Department of Public Health and Paediatric Sciences, University of Turin, Turin, Italy.
²³ Division of Hematology/Oncology, Herbert Irving Comprehensive Cancer Center, Columbia University Irving Medical Center, New York, NY, USA.
²⁴ Department of Radiation Oncology, University Hospital of Texas, MD Anderson Cancer Center, Houston, TX, USA.
²⁵ Department of Paediatrics, Faculty of Medicine and University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
²⁶ Department of Surgery, University of Cambridge and National Institute for Health Research Cambridge Biomedical Research Centre, Addenbrooke's Hospital, Cambridge, UK.
²⁷ Blood and Transplant Research Unit in Organ Donation and Transplantation, National Institute for Health Research, University of Cambridge, Cambridge, UK.
²⁸ Paediatrics and Adolescent Medicine, Faculty of Medicine, University of Augsburg, Augsburg, Germany.
²⁹ U.O.C. Chirurgia Endocrina e Metabolica, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
³⁰ Istituto di Semeiotica Chirurgica, Università Cattolica del Sacro Cuore, Rome, Italy.
³¹ Service de médecine nucléaire, Inserm U970, Sorbonne université, Groupe hospitalier Pitié-Salpétrière, Paris, France.
³² Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust and NIHR GOSH Biomedical Research Centre, University College London Great Ormond Street Institute of Child Health, London, UK.
³³ Surgical Oncology Program, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
³⁴ Endocrinology, Diabetes and Metabolism, Department of Medical Sciences, University of Turin, Corso Dogliotti, Turin, Italy.
³⁵ Department of Internal Medicine, Radboud University Medical Centre, Nijmegen, Netherlands.
³⁶ Clinical Child and Adolescent Psychology, Institute of Clinical Psychology and Psychotherapy, Department of Psychology, TU Dresden, Dresden, Germany.
³⁷ Université Paris Cité, PARCC, INSERM, Paris, France.
³⁸ Service de Génétique, Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Paris, France.
³⁹ Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK.
⁴⁰ Centre for Endocrinology, Barts and the London School of Medicine, London, UK.
⁴¹ ENETS Centre of Excellence, Royal Free Hospital, London, UK.
⁴² Department of Nuclear Medicine, La Timone University Hospital, CERIMED, Aix-Marseille University, Marseille, France.
⁴³ Department of Medical Genetics, University of Cambridge and NIHR Cambridge Biomedical Research Centre, Cambridge, UK.
⁴⁴ Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Rockville, MD, USA.
⁴⁵ Department of Medicine III, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany. Christina.Pamporaki@ukdd.de.

PMID: 39147856
DOI: 10.1038/s41574-024-01024-5

Erratum in

Author Correction: International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents.
Casey RT, Hendriks E, Deal C, Waguespack SG, Wiegering V, Redlich A, Akker S, Prasad R, Fassnacht M, Clifton-Bligh R, Amar L, Bornstein S, Canu L, Charmandari E, Chrisoulidou A, Freixes MC, de Krijger R, de Sanctis L, Fojo A, Ghia AJ, Huebner A, Kosmoliaptsis V, Kuhlen M, Raffaelli M, Lussey-Lepoutre C, Marks SD, Nilubol N, Parasiliti-Caprino M, Timmers HHJLM, Zietlow AL, Robledo M, Gimenez-Roqueplo AP, Grossman AB, Taïeb D, Maher ER, Lenders JWM, Eisenhofer G, Jimenez C, Pacak K, Pamporaki C. Casey RT, et al. Nat Rev Endocrinol. 2024 Dec;20(12):760. doi: 10.1038/s41574-024-01034-3. Nat Rev Endocrinol. 2024. PMID: 39191988 No abstract available.
Author Correction: International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents.
Casey RT, Hendriks E, Deal C, Waguespack SG, Wiegering V, Redlich A, Akker S, Prasad R, Fassnacht M, Clifton-Bligh R, Amar L, Bornstein S, Canu L, Charmandari E, Chrisoulidou A, Freixes MC, de Krijger R, de Sanctis L, Fojo A, Ghia AJ, Huebner A, Kosmoliaptsis V, Kuhlen M, Raffaelli M, Lussey-Lepoutre C, Marks SD, Nilubol N, Parasiliti-Caprino M, Timmers HHJLM, Zietlow AL, Robledo M, Gimenez-Roqueplo AP, Grossman AB, Taïeb D, Maher ER, Lenders JWM, Eisenhofer G, Jimenez C, Pacak K, Pamporaki C. Casey RT, et al. Nat Rev Endocrinol. 2025 Oct;21(10):656. doi: 10.1038/s41574-025-01167-z. Nat Rev Endocrinol. 2025. PMID: 40804506 No abstract available.

Abstract

Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumours that arise not only in adulthood but also in childhood and adolescence. Up to 70-80% of childhood PPGL are hereditary, accounting for a higher incidence of metastatic and/or multifocal PPGL in paediatric patients than in adult patients. Key differences in the tumour biology and management, together with rare disease incidence and therapeutic challenges in paediatric compared with adult patients, mandate close expert cross-disciplinary teamwork. Teams should ideally include adult and paediatric endocrinologists, oncologists, cardiologists, surgeons, geneticists, pathologists, radiologists, clinical psychologists and nuclear medicine physicians. Provision of an international Consensus Statement should improve care and outcomes for children and adolescents with these tumours.

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Conflict of interest statement

Competing interests R.T.C. has received a Novartis speaker honorarium and is in an editorial position in Clinical Endocrinology. C.J. has received funding to his institution from Lantheus, Progenics, Exelixis, Merck Sharpe and Dohme and is a clinical adviser for Lantheus and Merck Sharpe and Dohme. S.D.M. is the Director of the NIHR Clinical Research Facility at Great Ormond Street Hospital, London. D.T. has received speaker and attendance honoraria from AAA/NOVARTIS. M.F. is an unpaid member of the ExCo of the European Society of Endocrinology. J.W.M.L. is an unpaid member of the advisory board of the Phaeochromocytoma and Paraganglioma Alliance.

References

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International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents

Affiliations

International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents

Authors

Affiliations

Erratum in

Abstract

Conflict of interest statement

References

Publication types

MeSH terms

LinkOut - more resources

Full Text Sources

Medical