The North American Neuroendocrine Tumor Society Consensus Guidelines for Surveillance and Management of Metastatic and/or Unresectable Pheochromocytoma and Paraganglioma.
Journal
Pancreas
ISSN: 1536-4828
Titre abrégé: Pancreas
Pays: United States
ID NLM: 8608542
Informations de publication
Date de publication:
01 04 2021
01 04 2021
Historique:
entrez:
3
5
2021
pubmed:
4
5
2021
medline:
20
1
2022
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 33939658
doi: 10.1097/MPA.0000000000001792
pii: 00006676-202104000-00001
doi:
Types de publication
Consensus Development Conference
Journal Article
Practice Guideline
Langues
eng
Sous-ensembles de citation
IM
Pagination
469-493Subventions
Organisme : NIGMS NIH HHS
ID : R01 GM114102
Pays : United States
Informations de copyright
Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.
Déclaration de conflit d'intérêts
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.
Références
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.
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.
Fishbein L. Pheochromocytoma and paraganglioma: genetics, diagnosis, and treatment. Hematol Oncol Clin North Am . 2016;30:135–150.
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.
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.
Hamidi O, Young WF Jr, Gruber L, et al. Outcomes of patients with metastatic phaeochromocytoma and paraganglioma: a systematic review and meta-analysis. Clin Endocrinol (Oxf) . 2017;87:440–450.
Hescot S, Leboulleux S, Amar L, et al. One-year progression-free survival of therapy-naive patients with malignant pheochromocytoma and paraganglioma. J Clin Endocrinol Metab . 2013;98:4006–4012.
Favier J, Amar L, Gimenez-Roqueplo AP. Paraganglioma and phaeochromocytoma: from genetics to personalized medicine. Nat Rev Endocrinol . 2015;11:101–111.
Gruber LM, Erickson D, Babovic-Vuksanovic D, et al. Pheochromocytoma and paraganglioma in patients with neurofibromatosis type 1. Clin Endocrinol (Oxf) . 2017;86:141–149.
Else T, Greenberg S, Fishbein L. Hereditary paraganglioma-pheochromocytoma syndromes. In: Adam MP, Ardinger HH, Pagon RA, et al, eds. GeneReviews [Internet] . Seattle, WA: University of Washington, Seattle; 2008. [Updated 2018 Oct 4].
Greenberg SE, Jacobs MF, Wachtel H, et al. Tumor detection rates in screening of individuals with SDHx -related hereditary paraganglioma-pheochromocytoma syndrome. Genet Med . 2020;22:2101–2107.
Andrews KA, Ascher DB, Pires DEV, et al. Tumour risks and genotype-phenotype correlations associated with germline variants in succinate dehydrogenase subunit genes SDHB , SDHC and SDHD . J Med Genet . 2018;55:384–394.
van der Tuin K, Mensenkamp AR, Tops CMJ, et al. Clinical aspects of SDHA-related pheochromocytoma and paraganglioma: a nationwide study. J Clin Endocrinol Metab . 2018;103:438–445.
Buffet A, Morin A, Castro-Vega LJ, et al. Germline mutations in the mitochondrial 2-oxoglutarate/malate carrier SLC25A11 gene confer a predisposition to metastatic paragangliomas. Cancer Res . 2018;78:1914–1922.
Buffet A, Smati S, Mansuy L, et al. Mosaicism in HIF2A -related polycythemia-paraganglioma syndrome. J Clin Endocrinol Metab . 2014;99:E369–E373.
Calsina B, Currás-Freixes M, Buffet A, et al. Role of MDH2 pathogenic variant in pheochromocytoma and paraganglioma patients. Genet Med . 2018;20:1652–1662.
Cascón A, Comino-Méndez I, Currás-Freixes M, et al. Whole-exome sequencing identifies MDH2 as a new familial paraganglioma gene. J Natl Cancer Inst . 2015;107:djv053.
Lorenzo FR, Yang C, Ng Tang Fui M, et al. A novel EPAS1 / HIF2A germline mutation in a congenital polycythemia with paraganglioma. J Mol Med (Berl) . 2013;91:507–512.
Remacha L, Comino-Méndez I, Richter S, et al. Targeted exome sequencing of Krebs cycle genes reveals candidate cancer-predisposing mutations in pheochromocytomas and paragangliomas. Clin Cancer Res . 2017;23:6315–6324.
Remacha L, Currás-Freixes M, Torres-Ruiz R, et al. Gain-of-function mutations in DNMT3A in patients with paraganglioma. Genet Med . 2018;20:1644–1651.
Remacha L, Pirman D, Mahoney CE, et al. Recurrent germline DLST mutations in individuals with multiple pheochromocytomas and paragangliomas. Am J Hum Genet . 2019;104:651–664.
Yang C, Sun MG, Matro J, et al. Novel HIF2A mutations disrupt oxygen sensing, leading to polycythemia, paragangliomas, and somatostatinomas. Blood . 2013;121:2563–2566.
Else T, Fishbein L. Discovery of new susceptibility genes: proceed cautiously. Genet Med . 2018;20:1512–1514.
Fishbein L, Merrill S, Fraker DL, et al. Inherited mutations in pheochromocytoma and paraganglioma: why all patients should be offered genetic testing. Ann Surg Oncol . 2013;20:1444–1450.
Neumann HPH, Young WF Jr, Eng C. Pheochromocytoma and paraganglioma. N Engl J Med . 2019;381:552–565.
VHL Alliance. The VHL Handbook . 5th ed. Boston, MA: VHL Alliance; 2015.
Ferner RE, Huson SM, Thomas N, et al. Guidelines for the diagnosis and management of individuals with neurofibromatosis 1. J Med Genet . 2007;44:81–88.
Rednam SP, Erez A, Druker H, et al. Von Hippel–Lindau and hereditary pheochromocytoma/paraganglioma syndromes: clinical features, genetics, and surveillance recommendations in childhood. Clin Cancer Res . 2017;23:e68–e75.
Wells SA Jr, Asa SL, Dralle H, et al. Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma. Thyroid . 2015;25:567–610.
Asa SL, Ezzat S, Mete O. The diagnosis and clinical significance of paragangliomas in unusual locations. J Clin Med . 2018;7:280.
Tischler AS, Asa SL. Paraganglia. In: Mills SE, ed. Histology for Pathologists . 5th ed. Philadelphia, PA: Wolters Kluwer; 2020:1274.
Asari R, Scheuba C, Kaczirek K, et al. Estimated risk of pheochromocytoma recurrence after adrenal-sparing surgery in patients with multiple endocrine neoplasia type 2A. Arch Surg . 2006;141:1199–1205; discussion 1205.
Benhammou JN, Boris RS, Pacak K, et al. Functional and oncologic outcomes of partial adrenalectomy for pheochromocytoma in patients with von Hippel–Lindau syndrome after at least 5 years of followup. J Urol . 2010;184:1855–1859.
Castinetti F, Qi XP, Walz MK, et al. Outcomes of adrenal-sparing surgery or total adrenalectomy in phaeochromocytoma associated with multiple endocrine neoplasia type 2: an international retrospective population-based study. Lancet Oncol . 2014;15:648–655.
Grubbs EG, Rich TA, Ng C, et al. Long-term outcomes of surgical treatment for hereditary pheochromocytoma. J Am Coll Surg . 2013;216:280–289.
Volkin D, Yerram N, Ahmed F, et al. Partial adrenalectomy minimizes the need for long-term hormone replacement in pediatric patients with pheochromocytoma and von Hippel–Lindau syndrome. J Pediatr Surg . 2012;47:2077–2082.
Koch CA, Mauro D, Walther MM, et al. Pheochromocytoma in von Hippel–Lindau disease: distinct histopathologic phenotype compared to pheochromocytoma in multiple endocrine neoplasia type 2. Endocr Pathol . 2002;13:17–27.
Białas M, Okoń K, Dyduch G, et al. Neuroendocrine markers and sustentacular cell count in benign and malignant pheochromocytomas—a comparative study. Pol J Pathol . 2013;64:129–135.
Elder EE, Xu D, Höög A, et al. KI-67 AND hTERT expression can aid in the distinction between malignant and benign pheochromocytoma and paraganglioma. Mod Pathol . 2003;16:246–255.
Fishbein L, Leshchiner I, Walter V, et al. Comprehensive molecular characterization of pheochromocytoma and paraganglioma. Cancer Cell . 2017;31:181–193.
Thompson LD. Pheochromocytoma of the adrenal gland scaled score (PASS) to separate benign from malignant neoplasms: a clinicopathologic and immunophenotypic study of 100 cases. Am J Surg Pathol . 2002;26:551–566.
Wu D, Tischler AS, Lloyd RV, et al. Observer variation in the application of the pheochromocytoma of the adrenal gland scaled score. Am J Surg Pathol . 2009;33:599–608.
Kimura N, Takayanagi R, Takizawa N, et al. Pathological grading for predicting metastasis in phaeochromocytoma and paraganglioma. Endocr Relat Cancer 2014;21:405–414.
Wachtel H, Hutchens T, Baraban E, et al. Predicting metastatic potential in pheochromocytoma and paraganglioma: a comparison of PASS and GAPP scoring systems. J Clin Endocrinol Metab . 2020;105:e4661–e4670.
Assadipour Y, Sadowski SM, Alimchandani M, et al. SDHB mutation status and tumor size but not tumor grade are important predictors of clinical outcome in pheochromocytoma and abdominal paraganglioma. Surgery . 2017;161:230–239.
Koh JM, Ahn SH, Kim H, et al. Validation of pathological grading systems for predicting metastatic potential in pheochromocytoma and paraganglioma. PLoS One . 2017;12:e0187398.
Udager AM, Magers MJ, Goerke DM, et al. The utility of SDHB and FH immunohistochemistry in patients evaluated for hereditary paraganglioma-pheochromocytoma syndromes. Hum Pathol . 2018;71:47–54.
Nockel P, El Lakis M, Gaitanidis A, et al. Preoperative 18 F-FDG PET/CT in pheochromocytomas and paragangliomas allows for precision surgery. Ann Surg . 2019;269:741–747.
Dhir M, Li W, Hogg ME, et al. Clinical predictors of malignancy in patients with pheochromocytoma and paraganglioma. Ann Surg Oncol . 2017;24:3624–3630.
Pierre C, Agopiantz M, Brunaud L, et al. COPPS, a composite score integrating pathological features, PS100 and SDHB losses, predicts the risk of metastasis and progression-free survival in pheochromocytomas/paragangliomas. Virchows Arch . 2019;474:721–734.
Zhong X, Ye L, Su T, et al. Establishment and evaluation of a novel biomarker-based nomogram for malignant phaeochromocytomas and paragangliomas. Clin Endocrinol (Oxf) . 2017;87:127–135.
Eisenhofer G, Lenders JW, Siegert G, et al. Plasma methoxytyramine: a novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumour size, location and SDHB mutation status. Eur J Cancer . 2012;48:1739–1749.
Asai S, Katabami T, Tsuiki M, et al. Controlling tumor progression with cyclophosphamide, vincristine, and dacarbazine treatment improves survival in patients with metastatic and unresectable malignant pheochromocytomas/paragangliomas. Horm Cancer . 2017;8:108–118.
Handforth C, Clegg A, Young C, et al. The prevalence and outcomes of frailty in older cancer patients: a systematic review. Ann Oncol . 2015;26:1091–1101.
Roman-Gonzalez A, Zhou S, Ayala-Ramirez M, et al. Impact of surgical resection of the primary tumor on overall survival in patients with metastatic pheochromocytoma or sympathetic paraganglioma. Ann Surg . 2018;268:172–178.
Sethi RV, Sethi RK, Herr MW, et al. Malignant head and neck paragangliomas: treatment efficacy and prognostic indicators. Am J Otolaryngol . 2013;34:431–438.
Amin MB, Edge SB, Green FL, et al, eds. AJCC Cancer Staging Manual . 8th ed. New York, NY: Springer International Publishing; 2017.
McCrary HC, Babajanian E, Calquin M, et al. Characterization of malignant head and neck paragangliomas at a single institution across multiple decades. JAMA Otolaryngol Head Neck Surg . 2019;145:641–646.
Lenders JW, Duh QY, Eisenhofer G, et al. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab . 2014;99:1915–1942.
Bílek R, Vlček P, Šafařík L, et al. Chromogranin A in the laboratory diagnosis of pheochromocytoma and paraganglioma. Cancers (Basel) . 2019;11:586.
Kidd M, Bodei L, Modlin IM. Chromogranin A: any relevance in neuroendocrine tumors? Curr Opin Endocrinol Diabetes Obes . 2016;23:28–37.
Dromain C, de Baere T, Baudin E, et al. MR imaging of hepatic metastases caused by neuroendocrine tumors: comparing four techniques. AJR Am J Roentgenol . 2003;180:121–128.
Timmers HJ, Chen CC, Carrasquillo JA, et al. Staging and functional characterization of pheochromocytoma and paraganglioma by 18 F-fluorodeoxyglucose ( 18 F-FDG) positron emission tomography. J Natl Cancer Inst . 2012;104:700–708.
Timmers HJ, Chen CC, Carrasquillo JA, et al. Comparison of 18 F-fluoro- l -DOPA, 18 F-fluoro-deoxyglucose, and 18 F-fluorodopamine PET and 123 I-MIBG scintigraphy in the localization of pheochromocytoma and paraganglioma. J Clin Endocrinol Metab . 2009;94:4757–4767.
Rao D, van Berkel A, Piscaer I, et al. Impact of 123 I-MIBG scintigraphy on clinical decision making in pheochromocytoma and paraganglioma. J Clin Endocrinol Metab . 2019;104:3812–3820.
Koopmans KP, Jager PL, Kema IP, et al. 111 In-octreotide is superior to 123 I-metaiodobenzylguanidine for scintigraphic detection of head and neck paragangliomas. J Nucl Med . 2008;49:1232–1237.
Janssen I, Blanchet EM, Adams K, et al. Superiority of [ 68 Ga]-DOTATATE PET/CT to other functional imaging modalities in the localization of SDHB-associated metastatic pheochromocytoma and paraganglioma. Clin Cancer Res . 2015;21:3888–3895.
Jha A, Ling A, Millo C, et al. Superiority of 68 Ga-DOTATATE over 18 F-FDG and anatomic imaging in the detection of succinate dehydrogenase mutation ( SDHx )-related pheochromocytoma and paraganglioma in the pediatric population. Eur J Nucl Med Mol Imaging . 2018;45:787–797.
Janssen I, Chen CC, Millo CM, et al. PET/CT comparing 68 Ga-DOTATATE and other radiopharmaceuticals and in comparison with CT/MRI for the localization of sporadic metastatic pheochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging . 2016;43:1784–1791.
Janssen I, Chen CC, Zhuang Z, et al. Functional imaging signature of patients presenting with polycythemia/paraganglioma syndromes. J Nucl Med . 2017;58:1236–1242.
Jimenez C, Rohren E, Habra MA, et al. Current and future treatments for malignant pheochromocytoma and sympathetic paraganglioma. Curr Oncol Rep . 2013;15:356–371.
Roman-Gonzalez A, Jimenez C. Malignant pheochromocytoma-paraganglioma: pathogenesis, TNM staging, and current clinical trials. Curr Opin Endocrinol Diabetes Obes . 2017;24:174–183.
Taïeb D, Hicks RJ, Hindié E, et al. European Association of Nuclear Medicine Practice Guideline/Society of Nuclear Medicine and Molecular Imaging procedure standard 2019 for radionuclide imaging of phaeochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging . 2019;46:2112–2137.
NCCN guidelines for neuroendocrine and adrenal tumors. 2019. Available at: https://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf . Accessed March 5, 2019.
Bruynzeel H, Feelders RA, Groenland TH, et al. Risk factors for hemodynamic instability during surgery for pheochromocytoma. J Clin Endocrinol Metab . 2010;95:678–685.
Pappachan JM, Tun NN, Arunagirinathan G, et al. Pheochromocytomas and hypertension. Curr Hypertens Rep . 2018;20:3.
Munro J, Hurlbert BJ, Hill GE. Calcium channel blockade and uncontrolled blood pressure during phaeochromocytoma surgery. Can J Anaesth . 1995;42:228–230.
Butz JJ, Weingarten TN, Cavalcante AN, et al. Perioperative hemodynamics and outcomes of patients on metyrosine undergoing resection of pheochromocytoma or paraganglioma. Int J Surg . 2017;46:1–6.
Wachtel H, Kennedy EH, Zaheer S, et al. Preoperative metyrosine improves cardiovascular outcomes for patients undergoing surgery for pheochromocytoma and paraganglioma. Ann Surg Oncol . 2015;22(suppl 3):S646–S654.
Groeben H, Nottebaum BJ, Alesina PF, et al. Perioperative α-receptor blockade in phaeochromocytoma surgery: an observational case series. Br J Anaesth . 2017;118:182–189.
Isaacs M, Lee P. Preoperative alpha-blockade in phaeochromocytoma and paraganglioma: is it always necessary? Clin Endocrinol (Oxf) . 2017;86:309–314.
Weingarten TN, Cata JP, O'Hara JF, et al. Comparison of two preoperative medical management strategies for laparoscopic resection of pheochromocytoma. Urology . 2010;76:508.e6–e11.
Buitenwerf E, Osinga TE, Timmers HJLM, et al. Efficacy of alpha-blockers on hemodynamic control during pheochromocytoma resection: a randomized controlled trial. J Clin Endocrinol Metab . 2020;105:2381–2391.
Dubois LA, Gray DK. Dopamine-secreting pheochromocytomas: in search of a syndrome. World J Surg . 2005;29:909–913.
Kohlenberg J, Welch B, Hamidi O, et al. Efficacy and safety of ablative therapy in the treatment of patients with metastatic pheochromocytoma and paraganglioma. Cancers (Basel) . 2019;11:195.
Venkatesan AM, Locklin J, Lai EW, et al. Radiofrequency ablation of metastatic pheochromocytoma. J Vasc Interv Radiol . 2009;20:1483–1490.
Zheng L, Zhou F, Yu X, et al. Hypertensive crisis during microwave ablation of adrenal neoplasms: a retrospective analysis of predictive factors. J Vasc Interv Radiol . 2019;30:1343–1350.
Frenk NE, Sebastianes F, Lerario AM, et al. Long-term results after CT-guided percutaneous ethanol ablation for the treatment of hyperfunctioning adrenal disorders. Clinics (Sao Paulo) . 2016;71:600–605.
Texakalidis P, Charisis N, Giannopoulos S, et al. Role of preoperative embolization in carotid body tumor surgery: a systematic review and meta-analysis. World Neurosurg . 2019;129:503–513.e2.
Watanabe D, Tanabe A, Naruse M, et al. Transcatheter arterial embolization for the treatment of liver metastases in a patient with malignant pheochromocytoma. Endocr J . 2006;53:59–66.
Nakano S, Tsushima Y, Taketomi-Takahashi A, et al. Hypertensive crisis due to contrast-enhanced computed tomography in a patient with malignant pheochromocytoma. Jpn J Radiol . 2011;29:449–451.
Breen W, Bancos I, Young WF Jr, et al. External beam radiation therapy for advanced/unresectable malignant paraganglioma and pheochromocytoma. Adv Radiat Oncol . 2017;3:25–29.
Teno S, Tanabe A, Nomura K, et al. Acutely exacerbated hypertension and increased inflammatory signs due to radiation treatment for metastatic pheochromocytoma. Endocr J . 1996;43:511–516.
Mazza A, Armigliato M, Marzola MC, et al. Anti-hypertensive treatment in pheochromocytoma and paraganglioma: current management and therapeutic features. Endocrine . 2014;45:469–478.
Thosani S, Ayala-Ramirez M, Román-González A, et al. Constipation: an overlooked, unmanaged symptom of patients with pheochromocytoma and sympathetic paraganglioma. Eur J Endocrinol . 2015;173:377–387.
American Diabetes Association. 9. Pharmacologic approaches to glycemic treatment: standards of medical care in diabetes—2020. Diabetes Care . 2020;43(suppl 1):S98–S110.
Matsui H, Ikeuchi S, Onoda N, et al. Malignant paraganglioma of the retroperitoneum with lung metastases: a 13-year survivor after radical surgery. Asian J Surg . 2007;30:75–79.
Khan JH, McElhinney DB, Rahman SB, et al. Pulmonary metastases of endocrine origin: the role of surgery. Chest . 1998;114:526–534.
Prejbisz A, Lenders JW, Eisenhofer G, et al. Mortality associated with phaeochromocytoma. Horm Metab Res . 2013;45:154–158.
Ayala-Ramirez M, Feng L, Habra MA, et al. Clinical benefits of systemic chemotherapy for patients with metastatic pheochromocytomas or sympathetic extra-adrenal paragangliomas: insights from the largest single-institutional experience. Cancer . 2012;118:2804–2812.
Letizia C, De Toma G, Caliumi C, et al. Plasma adrenomedullin concentrations in patients with adrenal pheochromocytoma. Horm Metab Res . 2001;33:290–294.
Larráyoz IM, Martínez-Herrero S, García-Sanmartín J, et al. Adrenomedullin and tumour microenvironment. J Transl Med . 2014;12:339.
Thouennon E, Pierre A, Yon L, et al. Expression of trophic peptides and their receptors in chromaffin cells and pheochromocytoma. Cell Mol Neurobiol . 2010;30:1383–1389.
Zografos GN, Vasiliadis G, Farfaras AN, et al. Laparoscopic surgery for malignant adrenal tumors. JSLS . 2009;13:196–202.
Li ML, Fitzgerald PA, Price DC, et al. Iatrogenic pheochromocytomatosis: a previously unreported result of laparoscopic adrenalectomy. Surgery . 2001;130:1072–1077.
Merklin RJ. Suprarenal gland lymphatic drainage. Am J Anat . 1966;119:359–374.
Gerry JM, Tran TB, Postlewait LM, et al. Lymphadenectomy for adrenocortical carcinoma: is there a therapeutic benefit? Ann Surg Oncol . 2016;23(suppl 5):708–713.
Reibetanz J, Jurowich C, Erdogan I, et al. Impact of lymphadenectomy on the oncologic outcome of patients with adrenocortical carcinoma. Ann Surg . 2012;255:363–369.
Mahoney EM, Harrison JH. Malignant pheochromocytoma: clinical course and treatment. J Urol . 1977;118:225–229.
Mittal J, Manikandan R, Dorairajan LN, et al. Recurrent malignant pheochromocytoma with lymph nodal metastasis in a child: a rare case. J Indian Assoc Pediatr Surg . 2017;22:242–244.
Plouin PF, Chatellier G, Fofol I, et al. Tumor recurrence and hypertension persistence after successful pheochromocytoma operation. Hypertension . 1997;29:1133–1139.
Howe JR. Radioguided surgery with gallium for neuroendocrine tumors. JAMA Surg . 2019;154:45–46.
Filippi L, Valentini FB, Gossetti B, et al. Intraoperative gamma probe detection of head and neck paragangliomas with 111 In-pentetreotide: a pilot study. Tumori . 2005;91:173–176.
Buhl T, Mortensen J, Kjaer A. I-123 MIBG imaging and intraoperative localization of metastatic pheochromocytoma: a case report. Clin Nucl Med . 2002;27:183–185.
Javid M, Callender GG, Baregamian N, et al. Pheochromocytomatosis treated by radio-guided surgery. AACE Clin Case Rep . 2017;3:e170–e175.
El Lakis M, Gianakou A, Nockel P, et al. Radioguided surgery with gallium 68 Dotatate for patients with neuroendocrine tumors. JAMA Surg . 2019;154:40–45.
Povoski SP, Neff RL, Mojzisik CM, et al. A comprehensive overview of radioguided surgery using gamma detection probe technology. World J Surg Oncol . 2009;7:11.
Vanderveen KA, Thompson SM, Callstrom MR, et al. Biopsy of pheochromocytomas and paragangliomas: potential for disaster. Surgery . 2009;146:1158–1166.
American College of Radiology. ACR–SIR–SPR practice parameter for the performance of image-guided percutaneous needle biopsy (PNB). 2018 Published 2018 revised. Available at: https://www.acr.org/-/media/ACR/Files/Practice-Parameters/PNB.pdf . Accessed August 12, 2019.
Jackson RS, Myhill JA, Padhya TA, et al. The effects of preoperative embolization on carotid body paraganglioma surgery: a systematic review and meta-analysis. Otolaryngol Head Neck Surg . 2015;153:943–950.
Abu-Ghanem S, Yehuda M, Carmel NN, et al. Impact of preoperative embolization on the outcomes of carotid body tumor surgery: a meta-analysis and review of the literature. Head Neck . 2016;38(suppl 1):E2386–E2394.
Averbuch SD, Steakley CS, Young RC, et al. Malignant pheochromocytoma: effective treatment with a combination of cyclophosphamide, vincristine, and dacarbazine. Ann Intern Med . 1988;109:267–273.
Huang H, Abraham J, Hung E, et al. Treatment of malignant pheochromocytoma/paraganglioma with cyclophosphamide, vincristine, and dacarbazine: recommendation from a 22-year follow-up of 18 patients. Cancer . 2008;113:2020–2028.
Niemeijer ND, Alblas G, van Hulsteijn LT, et al. Chemotherapy with cyclophosphamide, vincristine and dacarbazine for malignant paraganglioma and pheochromocytoma: systematic review and meta-analysis. Clin Endocrinol (Oxf) . 2014;81:642–651.
Szalat A, Fraenkel M, Doviner V, et al. Malignant pheochromocytoma: predictive factors of malignancy and clinical course in 16 patients at a single tertiary medical center. Endocrine . 2011;39:160–166.
Tanabe A, Naruse M, Nomura K, et al. Combination chemotherapy with cyclophosphamide, vincristine, and dacarbazine in patients with malignant pheochromocytoma and paraganglioma. Horm Cancer . 2013;4:103–110.
Kunz PL, Catalano PJ, Nimeiri H, et al. A randomized study of temozolomide or temozolomide and capecitabine in patients with advanced pancreatic neuroendocrine tumors: a trial of the ECOG-ACRIN cancer research group (E2211). J Clin Oncol . 2018;36(suppl 15):4004.abstract.
Hadoux J, Favier J, Scoazec JY, et al. SDHB mutations are associated with response to temozolomide in patients with metastatic pheochromocytoma or paraganglioma. Int J Cancer . 2014;135:2711–2720.
Halperin DM, Brais L, Ramaiya NH, et al. Clinical presentation and outcomes in patients with advanced pheochromocytoma/paraganglioma: evidence of temozolomide efficacy. J Clin Oncol . 2014;32(suppl 15):e15157.abstract.
Kulke MH, Stuart K, Enzinger PC, et al. Phase II study of temozolomide and thalidomide in patients with metastatic neuroendocrine tumors. J Clin Oncol . 2006;24:401–406.
Favier J, Igaz P, Burnichon N, et al. Rationale for anti-angiogenic therapy in pheochromocytoma and paraganglioma. Endocr Pathol . 2012;23:34–42.
Jimenez C, Fazeli S, Román-Gonzalez A. Antiangiogenic therapies for pheochromocytoma and paraganglioma. Endocr Relat Cancer . 2020;27:R239–R254.
Jimenez C, Cabanillas ME, Santarpia L, et al. Use of the tyrosine kinase inhibitor sunitinib in a patient with von Hippel–Lindau disease: targeting angiogenic factors in pheochromocytoma and other von Hippel–Lindau disease-related tumors. J Clin Endocrinol Metab . 2009;94:386–391.
Joshua AM, Ezzat S, Asa SL, et al. Rationale and evidence for sunitinib in the treatment of malignant paraganglioma/pheochromocytoma. J Clin Endocrinol Metab . 2009;94:5–9.
Jasim S, Suman VJ, Jimenez C, et al. Phase II trial of pazopanib in advanced/progressive malignant pheochromocytoma and paraganglioma. Endocrine . 2017;57:220–225.
Jimenez P, Tatsui C, Jessop A, et al. Treatment for malignant pheochromocytomas and paragangliomas: 5 years of progress. Curr Oncol Rep . 2017;19:83.
O’Kane GM, Ezzat S, Joshua AM, et al. A phase 2 trial of sunitinib in patients with progressive paraganglioma or pheochromocytoma: the SNIPP trial. Br J Cancer . 2019;120:1113–1119.
Ayala-Ramirez M, Chougnet CN, Habra MA, et al. Treatment with sunitinib for patients with progressive metastatic pheochromocytomas and sympathetic paragangliomas. J Clin Endocrinol Metab . 2012;97:4040–4050.
Yokomoto-Umakoshi M, Umakoshi H, Tsuiki M, et al. Paraganglioma as a risk factor for bone metastasis. Endocr J . 2018;65:253–260.
Kim BJ, Kwak MK, Kim JS, et al. Higher sympathetic activity as a risk factor for skeletal deterioration in pheochromocytoma. Bone . 2018;116:1–7.
Wang Z, Qiao D, Lu Y, et al. Systematic literature review and network meta-analysis comparing bone-targeted agents for the prevention of skeletal-related events in cancer patients with bone metastasis. Oncologist . 2015;20:440–449.
von Moos R, Costa L, Ripamonti CI, et al. Improving quality of life in patients with advanced cancer: targeting metastatic bone pain. Eur J Cancer . 2017;71:80–94.
Southcott D, Awan A, Ghate K, et al. Practical update for the use of bone-targeted agents in patients with bone metastases from metastatic breast cancer or castration-resistant prostate cancer. Curr Oncol . 2020;27:220–224.
Tsourdi E, Zillikens MC, Meier C, et al. Fracture risk and management of discontinuation of denosumab therapy: a systematic review and position statement by ECTS. J Clin Endocrinol Metab . 2020;dgaa756.
Gravel G, Leboulleux S, Tselikas L, et al. Prevention of serious skeletal-related events by interventional radiology techniques in patients with malignant paraganglioma and pheochromocytoma. Endocrine . 2018;59:547–554.
Zelinka T, Timmers HJ, Kozupa A, et al. Role of positron emission tomography and bone scintigraphy in the evaluation of bone involvement in metastatic pheochromocytoma and paraganglioma: specific implications for succinate dehydrogenase enzyme subunit B gene mutations. Endocr Relat Cancer . 2008;15:311–323.
Kline RC, Swanson DP, Wieland DM, et al. Myocardial imaging in man with I-123 meta-iodobenzylguanidine. J Nucl Med . 1981;22:129–132.
Wieland DM, Wu J, Brown LE, et al. Radiolabeled adrenergi neuron-blocking agents: adrenomedullary imaging with [ 131 I]iodobenzylguanidine. J Nucl Med . 1980;21:349–353.
Valk TW, Frager MS, Gross MD, et al. Spectrum of pheochromocytoma in multiple endocrine neoplasia. A scintigraphic portrayal using 131 I-metaiodobenzylguanidine. Ann Intern Med . 1981;94:762–767.
Wieland DM, Brown LE, Tobes MC, et al. Imaging the primate adrenal medulla with [ 123 I] and [ 131 I] meta-iodobenzylguanidine: concise communication. J Nucl Med . 1981;22:358–364.
Sisson J, Shapiro B, Beierwaltes WH, et al. Treatment of malignant pheochromocytoma with a new radiopharmaceutical. Trans Assoc Am Physicians . 1983;96:209–217.
Castellani MR, Seghezzi S, Chiesa C, et al. ( 131 )I-MIBG treatment of pheochromocytoma: low versus intermediate activity regimens of therapy. Q J Nucl Med Mol Imaging . 2010;54:100–113.
French S, DuBois SG, Horn B, et al. 131 I-MIBG followed by consolidation with busulfan, melphalan and autologous stem cell transplantation for refractory neuroblastoma. Pediatr Blood Cancer . 2013;60:879–884.
Gonias S, Goldsby R, Matthay KK, et al. Phase II study of high-dose [ 131 I]metaiodobenzylguanidine therapy for patients with metastatic pheochromocytoma and paraganglioma. J Clin Oncol . 2009;27:4162–4168.
Matthay KK, Quach A, Huberty J, et al. Iodine-131—metaiodobenzylguanidine double infusion with autologous stem-cell rescue for neuroblastoma: a new approaches to neuroblastoma therapy phase I study. J Clin Oncol . 2009;27:1020–1025.
Navalkissoor S, Alhashimi DM, Quigley AM, et al. Efficacy of using a standard activity of ( 131 )I-MIBG therapy in patients with disseminated neuroendocrine tumours. Eur J Nucl Med Mol Imaging . 2010;37:904–912.
Noto RB, Pryma DA, Jensen J, et al. Phase 1 study of high-specific-activity I-131 MIBG for metastatic and/or recurrent pheochromocytoma or paraganglioma. J Clin Endocrinol Metab . 2018;103:213–220.
Pryma DA, Chin BB, Noto RB, et al. Efficacy and safety of high-specific-activity 131 I-MIBG therapy in patients with advanced pheochromocytoma or paraganglioma. J Nucl Med . 2019;60:623–630.
Rose B, Matthay KK, Price D, et al. High-dose 131 I-metaiodobenzylguanidine therapy for 12 patients with malignant pheochromocytoma. Cancer . 2003;98:239–248.
Shilkrut M, Bar-Deroma R, Bar-Sela G, et al. Low-dose iodine-131 metaiodobenzylguanidine therapy for patients with malignant pheochromocytoma and paraganglioma: single center experience. Am J Clin Oncol . 2010;33:79–82.
Suh JK, Koh KN, Min SY, et al. Feasibility and effectiveness of treatment strategy of tandem high-dose chemotherapy and autologous stem cell transplantation in combination with 131 I-MIBG therapy for high-risk neuroblastoma. Pediatr Transplant . 2020;24:e13658.
Wakabayashi H, Inaki A, Yoshimura K, et al. A phase I clinical trial for [ 131 I]meta-iodobenzylguanidine therapy in patients with refractory pheochromocytoma and paraganglioma. Sci Rep . 2019;9:7625.
Wakabayashi H, Taki J, Inaki A, et al. Prognostic values of initial responses to low-dose ( 131 )I-MIBG therapy in patients with malignant pheochromocytoma and paraganglioma. Ann Nucl Med . 2013;27:839–846.
Progenics Pharmaceuticals I. Azedra prescribing information. Published 2018. Updated 7.2018. Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2018/209607s000lbl.pdf . Accessed February 13, 2020.
Porzig A, Matthay KK, Dubois S, et al. Proteinuria in metastatic pheochromocytoma is associated with an increased risk of acute respiratory distress syndrome, spontaneously or after therapy with 131 I-meta-iodobenzylguanidine ( 131 I-MIBG). Horm Metab Res . 2012;44:539–542.
Advanced Accelerator Applications USA I. Lutathera prescribing information. Published 2018. Updated 1.2018. Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2018/208700s000lbl.pdf . Accessed December 13, 2020.
Forrer F, Riedweg I, Maecke HR, et al. Radiolabeled DOTATOC in patients with advanced paraganglioma and pheochromocytoma. Q J Nucl Med Mol Imaging . 2008;52:334–340.
Kong G, Grozinsky-Glasberg S, Hofman MS, et al. Efficacy of peptide receptor radionuclide therapy for functional metastatic paraganglioma and pheochromocytoma. J Clin Endocrinol Metab . 2017;102:3278–3287.
Vyakaranam AR, Crona J, Norlén O, et al. Favorable outcome in patients with pheochromocytoma and paraganglioma treated with 177 Lu-DOTATATE. Cancers (Basel) . 2019;11:909.
Zandee WT, Feelders RA, Smit Duijzentkunst DA, et al. Treatment of inoperable or metastatic paragangliomas and pheochromocytomas with peptide receptor radionuclide therapy using 177 Lu-DOTATATE. Eur J Endocrinol . 2019;181:45–53.
Spranger S, Luke JJ, Bao R, et al. Density of immunogenic antigens does not explain the presence or absence of the T-cell-inflamed tumor microenvironment in melanoma. Proc Natl Acad Sci U S A . 2016;113:E7759–E7768.
Jimenez C, Subbiah V, Stephen B, et al. Phase II clinical trial of pembrolizumab in patients with progressive metastatic pheochromocytomas and paragangliomas. Cancers (Basel) . 2020;12:2307.
Williams HL, Childs DS Jr, Parkhill EM, et al. Chemodectomas of the glomus jugulare (nonchromaffin paragangliomas) with especial reference to their response to roentgen therapy. Ann Otol Rhinol Laryngol . 1955;64:546–566.
Suárez C, Rodrigo JP, Bödeker CC, et al. Jugular and vagal paragangliomas: systematic study of management with surgery and radiotherapy. Head Neck . 2013;35:1195–1204.
van Hulsteijn LT, Corssmit EP, Coremans IE, et al. Regression and local control rates after radiotherapy for jugulotympanic paragangliomas: systematic review and meta-analysis. Radiother Oncol . 2013;106:161–168.
Fishbein L, Bonner L, Torigian DA, et al. External beam radiation therapy (EBRT) for patients with malignant pheochromocytoma and non-head and -neck paraganglioma: combination with 131 I-MIBG. Horm Metab Res . 2012;44:405–410.
Vogel J, Atanacio AS, Prodanov T, et al. External beam radiation therapy in treatment of malignant pheochromocytoma and paraganglioma. Front Oncol . 2014;4:166.
Caplin ME, Pavel M, Ćwikła JB, et al. Lanreotide in metastatic enteropancreatic neuroendocrine tumors. N Engl J Med . 2014;371:224–233.
Lamarre-Cliche M, Gimenez-Roqueplo AP, Billaud E, et al. Effects of slow-release octreotide on urinary metanephrine excretion and plasma chromogranin A and catecholamine levels in patients with malignant or recurrent phaeochromocytoma. Clin Endocrinol (Oxf) . 2002;57:629–634.
Plouin PF, Bertherat J, Chatellier G, et al. Short-term effects of octreotide on blood pressure and plasma catecholamines and neuropeptide Y levels in patients with phaeochromocytoma: a placebo-controlled trial. Clin Endocrinol (Oxf) . 1995;42:289–294.
Cass ND, Schopper MA, Lubin JA, et al. The changing paradigm of head and neck paragangliomas: what every otolaryngologist needs to know. Ann Otol Rhinol Laryngol . 2020;129:1135–1143.
Lee JH, Barich F, Karnell LH, et al. National Cancer Data Base report on malignant paragangliomas of the head and neck. Cancer 2002;94:730–737.
Hu K, Persky MS. Treatment of head and neck paragangliomas. Cancer Control . 2016;23:228–241.
Hamidi O, Young WF Jr, Iñiguez-Ariza NM, et al. Malignant pheochromocytoma and paraganglioma: 272 patients over 55 years. J Clin Endocrinol Metab . 2017;102:3296–3305.
Hinerman RW, Amdur RJ, Morris CG, et al. Definitive radiotherapy in the management of paragangliomas arising in the head and neck: a 35-year experience. Head Neck . 2008;30:1431–1438.
Ivan ME, Sughrue ME, Clark AJ, et al. A meta-analysis of tumor control rates and treatment-related morbidity for patients with glomus jugulare tumors. J Neurosurg . 2011;114:1299–1305.
Chun SG, Nedzi LA, Choe KS, et al. A retrospective analysis of tumor volumetric responses to five-fraction stereotactic radiotherapy for paragangliomas of the head and neck (glomus tumors). Stereotact Funct Neurosurg . 2014;92:153–159.
Janssen I, Chen CC, Taieb D, et al. 68 Ga-DOTATATE PET/CT in the localization of head and neck paragangliomas compared with other functional imaging modalities and CT/MRI. J Nucl Med . 2016;57:186–191.
Duet M, Guichard JP, Rizzo N, et al. Are somatostatin analogs therapeutic alternatives in the management of head and neck paragangliomas? Laryngoscope . 2005;115:1381–1384.
Kau R, Arnold W. Somatostatin receptor scintigraphy and therapy of neuroendocrine (APUD) tumors of the head and neck. Acta Otolaryngol . 1996;116:345–349.
Koyama H, Wada T, Nishizawa Y, et al. Cyclophosphamide-induced ovarian failure and its therapeutic significance in patients with breast cancer. Cancer . 1977;39:1403–1409.
Rivkees SA, Crawford JD. The relationship of gonadal activity and chemotherapy-induced gonadal damage. JAMA . 1988;259:2123–2125.
Blumenfeld Z. Chemotherapy and fertility. Best Pract Res Clin Obstet Gynaecol . 2012;26:379–390.
Muñoz M, Santaballa A, Seguí MA, et al. SEOM clinical guideline of fertility preservation and reproduction in cancer patients (2016). Clin Transl Oncol . 2016;18:1229–1236.
Berthaut I, Montjean D, Dessolle L, et al. Effect of temozolomide on male gametes: an epigenetic risk to the offspring? J Assist Reprod Genet . 2013;30:827–833.
Strowd RE, Blackwood R, Brown M, et al. Impact of temozolomide on gonadal function in patients with primary malignant brain tumors. J Oncol Pharm Pract . 2013;19:321–327.
Larson RA. Etiology and management of therapy-related myeloid leukemia. Hematology Am Soc Hematol Educ Program . 2007;453–459.
Leone G, Pagano L, Ben-Yehuda D, et al. Therapy-related leukemia and myelodysplasia: susceptibility and incidence. Haematologica . 2007;92:1389–1398.
Smith SM, Le Beau MM, Huo D, et al. Clinical-cytogenetic associations in 306 patients with therapy-related myelodysplasia and myeloid leukemia: the University of Chicago series. Blood . 2003;102:43–52.
Bergsma H, van Lom K, Raaijmakers MHGP, et al. Persistent hematologic dysfunction after peptide receptor radionuclide therapy with 177 Lu-DOTATATE: incidence, course, and predicting factors in patients with gastroenteropancreatic neuroendocrine tumors. J Nucl Med . 2018;59:452–458.
Brabander T, van der Zwan WA, Teunissen JJM, et al. Long-term efficacy, survival, and safety of [ 177 Lu-DOTA 0 ,Tyr 3 ]octreotate in patients with gastroenteropancreatic and bronchial neuroendocrine tumors. Clin Cancer Res . 2017;23:4617–4624.
Strosberg J, El-Haddad G, Wolin E, et al. Phase 3 trial of (177)Lu-Dotatate for midgut neuroendocrine tumors. N Engl J Med . 2017;376:125–135.
Ezziddin S, Sabet A, Logvinski T, et al. Long-term outcome and toxicity after dose-intensified treatment with 131 I-MIBG for advanced metastatic carcinoid tumors. J Nucl Med . 2013;54:2032–2038.
Fitzgerald PA, Goldsby RE, Huberty JP, et al. Malignant pheochromocytomas and paragangliomas: a phase II study of therapy with high-dose 131 I-metaiodobenzylguanidine ( 131 I-MIBG). Ann N Y Acad Sci . 2006;1073:465–490.
Gedik GK, Hoefnagel CA, Bais E, et al. 131 I-MIBG therapy in metastatic phaeochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging . 2008;35:725–733.
Mukherjee JJ, Kaltsas GA, Islam N, et al. Treatment of metastatic carcinoid tumours, phaeochromocytoma, paraganglioma and medullary carcinoma of the thyroid with ( 131 )I-meta-iodobenzylguanidine [( 131 )I-mIBG]. Clin Endocrinol (Oxf) . 2001;55:47–60.
Safford SD, Coleman RE, Gockerman JP, et al. Iodine-131 metaiodobenzylguanidine is an effective treatment for malignant pheochromocytoma and paraganglioma. Surgery . 2003;134:956–962; discussion 962–963.
Castro-Vega LJ, Letouzé E, Burnichon N, et al. Multi-omics analysis defines core genomic alterations in pheochromocytomas and paragangliomas. Nat Commun . 2015;6:6044.
Currás-Freixes M, Inglada-Pérez L, Mancikova V, et al. Recommendations for somatic and germline genetic testing of single pheochromocytoma and paraganglioma based on findings from a series of 329 patients. J Med Genet . 2015;52:647–656.
Ben Aim L, Pigny P, Castro-Vega LJ, et al. Targeted next-generation sequencing detects rare genetic events in pheochromocytoma and paraganglioma. J Med Genet . 2019;56:513–520.
Currás-Freixes M, Piñeiro-Yañez E, Montero-Conde C, et al. PheoSeq: a targeted next-generation sequencing assay for pheochromocytoma and paraganglioma diagnostics. J Mol Diagn . 2017;19:575–588.
Barthel FP, Wei W, Tang M, et al. Systematic analysis of telomere length and somatic alterations in 31 cancer types. Nat Genet . 2017;49:349–357.
Fishbein L, Khare S, Wubbenhorst B, et al. Whole-exome sequencing identifies somatic ATRX mutations in pheochromocytomas and paragangliomas. Nat Commun . 2015;6:6140.
Toledo RA, Qin Y, Cheng ZM, et al. Recurrent mutations of chromatin-remodeling genes and kinase receptors in pheochromocytomas and paragangliomas. Clin Cancer Res . 2016;22:2301–2310.
Dwight T, Flynn A, Amarasinghe K, et al. TERT structural rearrangements in metastatic pheochromocytomas. Endocr Relat Cancer . 2018;25:1–9.
Job S, Draskovic I, Burnichon N, et al. Telomerase activation and ATRX mutations are independent risk factors for metastatic pheochromocytoma and paraganglioma. Clin Cancer Res . 2019;25:760–770.
Calsina B, Castro-Vega LJ, Torres-Pérez R, et al. Integrative multi-omics analysis identifies a prognostic miRNA signature and a targetable miR-21-3p/TSC2/mTOR axis in metastatic pheochromocytoma/paraganglioma. Theranostics . 2019;9:4946–4958.
de Cubas AA, Korpershoek E, Inglada-Pérez L, et al. DNA methylation profiling in pheochromocytoma and paraganglioma reveals diagnostic and prognostic markers. Clin Cancer Res . 2015;21:3020–3030.
Toledo RA, Burnichon N, et alNGS in PPGL (NGSnPPGL) Study Group. Consensus statement on next-generation-sequencing-based diagnostic testing of hereditary phaeochromocytomas and paragangliomas. Nat Rev Endocrinol . 2017;13:233–247.
Crona J, Lamarca A, Ghosal S, et al. Genotype-phenotype correlations in pheochromocytoma and paraganglioma: a systematic review and individual patient meta-analysis. Endocr Relat Cancer . 2019;26:539–550.
Gill AJ, Benn DE, Chou A, et al. Immunohistochemistry for SDHB triages genetic testing of SDHB, SDHC, and SDHD in paraganglioma-pheochromocytoma syndromes. Hum Pathol . 2010;41:805–814.
Papathomas TG, Oudijk L, Persu A, et al. SDHB/SDHA immunohistochemistry in pheochromocytomas and paragangliomas: a multicenter interobserver variation analysis using virtual microscopy: a multinational study of the European Network for the Study of Adrenal Tumors (ENS@T). Mod Pathol . 2015;28:807–821.
Castro-Vega LJ, Buffet A, De Cubas AA, et al. Germline mutations in FH confer predisposition to malignant pheochromocytomas and paragangliomas. Hum Mol Genet . 2014;23:2440–2446.
Mattox AK, Bettegowda C, Zhou S, et al. Applications of liquid biopsies for cancer. Sci Transl Med . 2019;11:eaay1984.
Wang L, Li Y, Guan X, et al. Exosomal double-stranded DNA as a biomarker for the diagnosis and preoperative assessment of pheochromocytoma and paraganglioma. Mol Cancer . 2018;17:128.
Eisenhofer G, Lenders JW, Goldstein DS, et al. Pheochromocytoma catecholamine phenotypes and prediction of tumor size and location by use of plasma free metanephrines. Clin Chem . 2005;51:735–744.
Tischler AS, Favier J. Models of pheochromocytoma: what's on the horizon? Int J Endo Oncol . 2015;2:171–174.
Powers JF, Cochran B, Baleja JD, et al. A xenograft and cell line model of SDH-deficient pheochromocytoma derived from Sdhb+/− rats. Endocr Relat Cancer . 2020;27:337–354.