The North American Neuroendocrine Tumor Society Consensus Guidelines for Surveillance and Management of Metastatic and/or Unresectable Pheochromocytoma and Paraganglioma

doi:10.1097/MPA.0000000000001792

Practice Guideline

. 2021 Apr 1;50(4):469-493.

doi: 10.1097/MPA.0000000000001792.

The North American Neuroendocrine Tumor Society Consensus Guidelines for Surveillance and Management of Metastatic and/or Unresectable Pheochromocytoma and Paraganglioma

Lauren Fishbein¹, Jaydira Del Rivero², Tobias Else³, James R Howe⁴, Sylvia L Asa⁵, Debbie L Cohen⁶, Patricia L M Dahia⁷, Douglas L Fraker⁸, Karyn A Goodman⁹, Thomas A Hope¹⁰, Pamela L Kunz¹¹, Kimberly Perez¹², Nancy D Perrier¹³, Daniel A Pryma¹⁴, Mabel Ryder¹⁵, Aaron R Sasson¹⁶, Michael C Soulen¹⁷, Camilo Jimenez¹⁸

Affiliations

¹ From the Division of Endocrinology, Metabolism, and Diabetes, Department of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO.
² Developmental Therapeutics Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD.
³ Division of Metabolism, Endocrinology, and Diabetes, Department of Internal Medicine, University of Michigan, Ann Arbor, MI.
⁴ Division of Surgical Oncology and Endocrine Surgery, Department of Surgery, University of Iowa Carver College of Medicine, Iowa City, IA.
⁵ Department of Pathology, University Hospitals Cleveland Medical Center and University Health Network, Toronto, Case Western Reserve University, Cleveland, OH.
⁶ Renal Division, Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
⁷ Division of Hematology and Medical Oncology, Department of Medicine, University of Texas Health San Antonio, San Antonio, TX.
⁸ Division of Endocrine and Oncologic Surgery, Department of Surgery, University of Pennsylvania and Abramson Cancer Center, Philadelphia, PA.
⁹ Department of Radiation Oncology, Icahn School of Medicine at Mount Sinai, New York, NY.
¹⁰ Department of Radiology and Biomedical Imaging, University of California San Francisco, San Francisco, CA.
¹¹ Division of Oncology, Department of Medicine, Yale School of Medicine, New Haven, CT.
¹² Department of Medical Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA.
¹³ Division of Surgery, Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX.
¹⁴ Department of Radiology and Abramson Cancer Center, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
¹⁵ Endocrine Oncology Tumor Group, Division of Medical Oncology, Mayo Clinic, Rochester, MN.
¹⁶ Division of Surgical Oncology, Department of Surgery, Stony Brook University Medical Center, Stony Brook, NY.
¹⁷ Department of Radiology, University of Pennsylvania, Philadelphia, PA.
¹⁸ Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, TX.

PMID: 33939658
DOI: 10.1097/MPA.0000000000001792

Practice Guideline

The North American Neuroendocrine Tumor Society Consensus Guidelines for Surveillance and Management of Metastatic and/or Unresectable Pheochromocytoma and Paraganglioma

Lauren Fishbein et al. Pancreas. 2021.

. 2021 Apr 1;50(4):469-493.

doi: 10.1097/MPA.0000000000001792.

Authors

Affiliations

¹ From the Division of Endocrinology, Metabolism, and Diabetes, Department of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO.
² Developmental Therapeutics Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD.
³ Division of Metabolism, Endocrinology, and Diabetes, Department of Internal Medicine, University of Michigan, Ann Arbor, MI.
⁴ Division of Surgical Oncology and Endocrine Surgery, Department of Surgery, University of Iowa Carver College of Medicine, Iowa City, IA.
⁵ Department of Pathology, University Hospitals Cleveland Medical Center and University Health Network, Toronto, Case Western Reserve University, Cleveland, OH.
⁶ Renal Division, Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
⁷ Division of Hematology and Medical Oncology, Department of Medicine, University of Texas Health San Antonio, San Antonio, TX.
⁸ Division of Endocrine and Oncologic Surgery, Department of Surgery, University of Pennsylvania and Abramson Cancer Center, Philadelphia, PA.
⁹ Department of Radiation Oncology, Icahn School of Medicine at Mount Sinai, New York, NY.
¹⁰ Department of Radiology and Biomedical Imaging, University of California San Francisco, San Francisco, CA.
¹¹ Division of Oncology, Department of Medicine, Yale School of Medicine, New Haven, CT.
¹² Department of Medical Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA.
¹³ Division of Surgery, Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX.
¹⁴ Department of Radiology and Abramson Cancer Center, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
¹⁵ Endocrine Oncology Tumor Group, Division of Medical Oncology, Mayo Clinic, Rochester, MN.
¹⁶ Division of Surgical Oncology, Department of Surgery, Stony Brook University Medical Center, Stony Brook, NY.
¹⁷ Department of Radiology, University of Pennsylvania, Philadelphia, PA.
¹⁸ Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, TX.

PMID: 33939658
DOI: 10.1097/MPA.0000000000001792

Abstract

This manuscript is the result of the North American Neuroendocrine Tumor Society consensus conference on the medical management and surveillance of metastatic and unresectable pheochromocytoma and paraganglioma held on October 2 and 3, 2019. The panelists consisted of endocrinologists, medical oncologists, surgeons, radiologists/nuclear medicine physicians, nephrologists, pathologists, and radiation oncologists. The panelists performed a literature review on a series of questions regarding the medical management of metastatic and unresectable pheochromocytoma and paraganglioma as well as questions regarding surveillance after resection. The panelists voted on controversial topics, and final recommendations were sent to all panel members for final approval.

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Conflict of interest statement

T.E. discloses consulting on an advisory board for HRA Pharma and Corcept Therapeutics, and participates in institutional contracted clinical study for Merck and Co, Inc, Corcept Therapeutics, and Strongbridge Biopharma. J.R.H. discloses National Institutes of Health grant funding unrelated to this project and royalties for a book on endocrine surgery unrelated to this project. S.L.A. discloses board membership for Leica Biosystems, consults for PathAI, and has received speaker payment from Med Learning Group. P.L.M.D. discloses support to travel to North American Neuroendocrine Tumor Society, is a full-time faculty member at the University of Texas Health San Antonio, and received grant funding for other projects from the National Institutes of Health and Alex's Lemonade Stand Foundation. T.A.H. discloses consulting for Ipsen and pending grant from Advanced Accelerator Applications. P.L.K. discloses consulting for Advanced Accelerator Applications and Ipsen and has grants from Advanced Accelerator Applications, Ipsen, Brahms (Thermo Fisher Scientific), Lexicon Pharmaceuticals, and Xencor. K.P. discloses serving one time on an advisory board panel for Celgene and Eisai, both unrelated to this manuscript. D.A.P. discloses consulting honoraria from Siemens, Progenics, Bayer, Ipsen, Fusion, 511 Pharma, and Actinium, and receiving research funding from Siemens, Fusion, Nordic Nanovector, 511 Pharma, and Progenics. A.R.S. discloses being part owner of a start-up called Sanguine Diagnostics and Therapeutics and also lecturing for Novartis pending. M.C.S. discloses consulting fees from Guerbet LLC, Genentech, and Instylla, and grant funding from Guerbet LLC and Boston Scientific. The other authors declare no conflict of interest.

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Molecular Genetics of Pheochromocytoma/Paraganglioma.
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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 Aug 15. doi: 10.1038/s41574-024-01024-5. Online ahead of print. Nat Rev Endocrinol. 2024. PMID: 39147856 Review.
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References

1. Lloyd RV, Osamura RY, Kloppel G, et al., eds. WHO Classification of Tumours: Pathology and Genetics of Tumours of Endocrine Organs . Vol 10, 4th ed. Lyon, France: IARC; 2017.
1. Ayala-Ramirez M, Palmer JL, Hofmann MC, et al. Bone metastases and skeletal-related events in patients with malignant pheochromocytoma and sympathetic paraganglioma. J Clin Endocrinol Metab . 2013;98:1492–1497.
1. Fishbein L. Pheochromocytoma and paraganglioma: genetics, diagnosis, and treatment. Hematol Oncol Clin North Am . 2016;30:135–150.
1. Ayala-Ramirez M, Feng L, Johnson MM, et al. Clinical risk factors for malignancy and overall survival in patients with pheochromocytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators. J Clin Endocrinol Metab . 2011;96:717–725.
1. Fishbein L, Ben-Maimon S, Keefe S, et al. SDHB mutation carriers with malignant pheochromocytoma respond better to CVD. Endocr Relat Cancer . 2017;24:L51–L55.

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[1] Lloyd RV, Osamura RY, Kloppel G, et al., eds. WHO Classification of Tumours: Pathology and Genetics of Tumours of Endocrine Organs . Vol 10, 4th ed. Lyon, France: IARC; 2017.

[2] Lloyd RV, Osamura RY, Kloppel G, et al., eds. WHO Classification of Tumours: Pathology and Genetics of Tumours of Endocrine Organs . Vol 10, 4th ed. Lyon, France: IARC; 2017.

[3] Ayala-Ramirez M, Palmer JL, Hofmann MC, et al. Bone metastases and skeletal-related events in patients with malignant pheochromocytoma and sympathetic paraganglioma. J Clin Endocrinol Metab . 2013;98:1492–1497.

[4] Ayala-Ramirez M, Palmer JL, Hofmann MC, et al. Bone metastases and skeletal-related events in patients with malignant pheochromocytoma and sympathetic paraganglioma. J Clin Endocrinol Metab . 2013;98:1492–1497.

[5] Fishbein L. Pheochromocytoma and paraganglioma: genetics, diagnosis, and treatment. Hematol Oncol Clin North Am . 2016;30:135–150.

[6] Fishbein L. Pheochromocytoma and paraganglioma: genetics, diagnosis, and treatment. Hematol Oncol Clin North Am . 2016;30:135–150.

[7] Ayala-Ramirez M, Feng L, Johnson MM, et al. Clinical risk factors for malignancy and overall survival in patients with pheochromocytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators. J Clin Endocrinol Metab . 2011;96:717–725.

[8] Ayala-Ramirez M, Feng L, Johnson MM, et al. Clinical risk factors for malignancy and overall survival in patients with pheochromocytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators. J Clin Endocrinol Metab . 2011;96:717–725.

[9] Fishbein L, Ben-Maimon S, Keefe S, et al. SDHB mutation carriers with malignant pheochromocytoma respond better to CVD. Endocr Relat Cancer . 2017;24:L51–L55.

[10] Fishbein L, Ben-Maimon S, Keefe S, et al. SDHB mutation carriers with malignant pheochromocytoma respond better to CVD. Endocr Relat Cancer . 2017;24:L51–L55.

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The North American Neuroendocrine Tumor Society Consensus Guidelines for Surveillance and Management of Metastatic and/or Unresectable Pheochromocytoma and Paraganglioma

Affiliations

The North American Neuroendocrine Tumor Society Consensus Guidelines for Surveillance and Management of Metastatic and/or Unresectable Pheochromocytoma and Paraganglioma

Authors

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Abstract

Conflict of interest statement

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