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Sympathetic paraganglioma

MedGen UID:
137758
•Concept ID:
C0334415
•
Neoplastic Process
Synonyms: Chromaffin Neoplasm; chromaffin neoplasm; Chromaffin Tumor; chromaffin tumor; Chromaffinoma; paraganglioma of sympathetic nervous system; sympathetic nervous system paraganglioma; Sympathetic Paraganglioma; sympathetic paraganglioma; Sympathetic Paraganglionic Neoplasm; sympathetic Paraganglionic neoplasm
SNOMED CT: Sympathetic paraganglioma (31794002)
 
Monarch Initiative: MONDO:0021072

Definition

A paraganglioma arising from the chromaffin cells of the paraganglia that are located along the sympathetic nerves. It includes extra-adrenal paragangliomas and paragangliomas that arise from the adrenal medulla. The latter are commonly referred to as pheochromocytomas. Representative examples of extra-adrenal sympathetic paragangliomas include the bladder, and superior and inferior paraaortic paragangliomas. Clinical signs are related to the secretion of catecholamines resulting in hypertension. [from NCI]

Professional guidelines

PubMed

Treatment for Malignant Pheochromocytomas and Paragangliomas: 5 Years of Progress.
Jimenez P, Tatsui C, Jessop A, Thosani S, Jimenez C
Curr Oncol Rep 2017 Oct 28;19(12):83. doi: 10.1007/s11912-017-0643-0. PMID: 29081018
Clinical management of paragangliomas.
Corssmit EP, Romijn JA
Eur J Endocrinol 2014 Dec;171(6):R231-43. Epub 2014 Jul 25 doi: 10.1530/EJE-14-0396. PMID: 25063320

Recent clinical studies

Etiology

Incidence and Clinical Presentation of Pheochromocytoma and Sympathetic Paraganglioma: A Population-based Study.
Ebbehoj A, Stochholm K, Jacobsen SF, Trolle C, Jepsen P, Robaczyk MG, Rasmussen ÅK, Feldt-Rasmussen U, Thomsen RW, Søndergaard E, Poulsen PL
J Clin Endocrinol Metab 2021 Apr 23;106(5):e2251-e2261. doi: 10.1210/clinem/dgaa965. PMID: 33479747
Efficacy of α-Blockers on Hemodynamic Control during Pheochromocytoma Resection: A Randomized Controlled Trial.
Buitenwerf E, Osinga TE, Timmers HJLM, Lenders JWM, Feelders RA, Eekhoff EMW, Haak HR, Corssmit EPM, Bisschop PHLT, Valk GD, Veldman RG, Dullaart RPF, Links TP, Voogd MF, Wietasch GJKG, Kerstens MN
J Clin Endocrinol Metab 2020 Jul 1;105(7):2381-91. doi: 10.1210/clinem/dgz188. PMID: 31714582Free PMC Article
Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: A nationwide study and systematic review.
Berends AMA, Buitenwerf E, de Krijger RR, Veeger NJGM, van der Horst-Schrivers ANA, Links TP, Kerstens MN
Eur J Intern Med 2018 May;51:68-73. Epub 2018 Feb 1 doi: 10.1016/j.ejim.2018.01.015. PMID: 29361475
Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours.
Lam AK
Endocr Pathol 2017 Sep;28(3):213-227. doi: 10.1007/s12022-017-9484-5. PMID: 28477311
Clinical management of paragangliomas.
Corssmit EP, Romijn JA
Eur J Endocrinol 2014 Dec;171(6):R231-43. Epub 2014 Jul 25 doi: 10.1530/EJE-14-0396. PMID: 25063320

Diagnosis

TNM Staging and Overall Survival in Patients With Pheochromocytoma and Sympathetic Paraganglioma.
Jimenez C, Ma J, Roman Gonzalez A, Varghese J, Zhang M, Perrier N, Habra MA, Graham P, Waguespack SG
J Clin Endocrinol Metab 2023 Apr 13;108(5):1132-1142. doi: 10.1210/clinem/dgac677. PMID: 36433823
Incidence and Clinical Presentation of Pheochromocytoma and Sympathetic Paraganglioma: A Population-based Study.
Ebbehoj A, Stochholm K, Jacobsen SF, Trolle C, Jepsen P, Robaczyk MG, Rasmussen ÅK, Feldt-Rasmussen U, Thomsen RW, Søndergaard E, Poulsen PL
J Clin Endocrinol Metab 2021 Apr 23;106(5):e2251-e2261. doi: 10.1210/clinem/dgaa965. PMID: 33479747
Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: A nationwide study and systematic review.
Berends AMA, Buitenwerf E, de Krijger RR, Veeger NJGM, van der Horst-Schrivers ANA, Links TP, Kerstens MN
Eur J Intern Med 2018 May;51:68-73. Epub 2018 Feb 1 doi: 10.1016/j.ejim.2018.01.015. PMID: 29361475
Clinical management of paragangliomas.
Corssmit EP, Romijn JA
Eur J Endocrinol 2014 Dec;171(6):R231-43. Epub 2014 Jul 25 doi: 10.1530/EJE-14-0396. PMID: 25063320
Current and future treatments for malignant pheochromocytoma and sympathetic paraganglioma.
Jimenez C, Rohren E, Habra MA, Rich T, Jimenez P, Ayala-Ramirez M, Baudin E
Curr Oncol Rep 2013 Aug;15(4):356-71. doi: 10.1007/s11912-013-0320-x. PMID: 23674235

Therapy

Selective vs non-selective alpha-blockade prior to adrenalectomy for pheochromocytoma: systematic review and meta-analysis.
Zawadzka K, Więckowski K, Małczak P, Wysocki M, Major P, Pędziwiatr M, Pisarska-Adamczyk M
Eur J Endocrinol 2021 May 4;184(6):751-760. doi: 10.1530/EJE-20-1301. PMID: 33769959
Efficacy of α-Blockers on Hemodynamic Control during Pheochromocytoma Resection: A Randomized Controlled Trial.
Buitenwerf E, Osinga TE, Timmers HJLM, Lenders JWM, Feelders RA, Eekhoff EMW, Haak HR, Corssmit EPM, Bisschop PHLT, Valk GD, Veldman RG, Dullaart RPF, Links TP, Voogd MF, Wietasch GJKG, Kerstens MN
J Clin Endocrinol Metab 2020 Jul 1;105(7):2381-91. doi: 10.1210/clinem/dgz188. PMID: 31714582Free PMC Article
Treatment for Malignant Pheochromocytomas and Paragangliomas: 5 Years of Progress.
Jimenez P, Tatsui C, Jessop A, Thosani S, Jimenez C
Curr Oncol Rep 2017 Oct 28;19(12):83. doi: 10.1007/s11912-017-0643-0. PMID: 29081018
Constipation: an overlooked, unmanaged symptom of patients with pheochromocytoma and sympathetic paraganglioma.
Thosani S, Ayala-Ramirez M, Román-González A, Zhou S, Thosani N, Bisanz A, Jimenez C
Eur J Endocrinol 2015 Sep;173(3):377-87. Epub 2015 Jun 9 doi: 10.1530/EJE-15-0456. PMID: 26060051
Current and future treatments for malignant pheochromocytoma and sympathetic paraganglioma.
Jimenez C, Rohren E, Habra MA, Rich T, Jimenez P, Ayala-Ramirez M, Baudin E
Curr Oncol Rep 2013 Aug;15(4):356-71. doi: 10.1007/s11912-013-0320-x. PMID: 23674235

Prognosis

TNM Staging and Overall Survival in Patients With Pheochromocytoma and Sympathetic Paraganglioma.
Jimenez C, Ma J, Roman Gonzalez A, Varghese J, Zhang M, Perrier N, Habra MA, Graham P, Waguespack SG
J Clin Endocrinol Metab 2023 Apr 13;108(5):1132-1142. doi: 10.1210/clinem/dgac677. PMID: 36433823
Incidence and Clinical Presentation of Pheochromocytoma and Sympathetic Paraganglioma: A Population-based Study.
Ebbehoj A, Stochholm K, Jacobsen SF, Trolle C, Jepsen P, Robaczyk MG, Rasmussen ÅK, Feldt-Rasmussen U, Thomsen RW, Søndergaard E, Poulsen PL
J Clin Endocrinol Metab 2021 Apr 23;106(5):e2251-e2261. doi: 10.1210/clinem/dgaa965. PMID: 33479747
Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: A nationwide study and systematic review.
Berends AMA, Buitenwerf E, de Krijger RR, Veeger NJGM, van der Horst-Schrivers ANA, Links TP, Kerstens MN
Eur J Intern Med 2018 May;51:68-73. Epub 2018 Feb 1 doi: 10.1016/j.ejim.2018.01.015. PMID: 29361475
Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours.
Lam AK
Endocr Pathol 2017 Sep;28(3):213-227. doi: 10.1007/s12022-017-9484-5. PMID: 28477311
Current and future treatments for malignant pheochromocytoma and sympathetic paraganglioma.
Jimenez C, Rohren E, Habra MA, Rich T, Jimenez P, Ayala-Ramirez M, Baudin E
Curr Oncol Rep 2013 Aug;15(4):356-71. doi: 10.1007/s11912-013-0320-x. PMID: 23674235

Clinical prediction guides

Efficacy of α-Blockers on Hemodynamic Control during Pheochromocytoma Resection: A Randomized Controlled Trial.
Buitenwerf E, Osinga TE, Timmers HJLM, Lenders JWM, Feelders RA, Eekhoff EMW, Haak HR, Corssmit EPM, Bisschop PHLT, Valk GD, Veldman RG, Dullaart RPF, Links TP, Voogd MF, Wietasch GJKG, Kerstens MN
J Clin Endocrinol Metab 2020 Jul 1;105(7):2381-91. doi: 10.1210/clinem/dgz188. PMID: 31714582Free PMC Article
Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: A nationwide study and systematic review.
Berends AMA, Buitenwerf E, de Krijger RR, Veeger NJGM, van der Horst-Schrivers ANA, Links TP, Kerstens MN
Eur J Intern Med 2018 May;51:68-73. Epub 2018 Feb 1 doi: 10.1016/j.ejim.2018.01.015. PMID: 29361475
Treatment for Malignant Pheochromocytomas and Paragangliomas: 5 Years of Progress.
Jimenez P, Tatsui C, Jessop A, Thosani S, Jimenez C
Curr Oncol Rep 2017 Oct 28;19(12):83. doi: 10.1007/s11912-017-0643-0. PMID: 29081018
Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours.
Lam AK
Endocr Pathol 2017 Sep;28(3):213-227. doi: 10.1007/s12022-017-9484-5. PMID: 28477311
Current and future treatments for malignant pheochromocytoma and sympathetic paraganglioma.
Jimenez C, Rohren E, Habra MA, Rich T, Jimenez P, Ayala-Ramirez M, Baudin E
Curr Oncol Rep 2013 Aug;15(4):356-71. doi: 10.1007/s11912-013-0320-x. PMID: 23674235

Recent systematic reviews

Selective vs non-selective alpha-blockade prior to adrenalectomy for pheochromocytoma: systematic review and meta-analysis.
Zawadzka K, Więckowski K, Małczak P, Wysocki M, Major P, Pędziwiatr M, Pisarska-Adamczyk M
Eur J Endocrinol 2021 May 4;184(6):751-760. doi: 10.1530/EJE-20-1301. PMID: 33769959
Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: A nationwide study and systematic review.
Berends AMA, Buitenwerf E, de Krijger RR, Veeger NJGM, van der Horst-Schrivers ANA, Links TP, Kerstens MN
Eur J Intern Med 2018 May;51:68-73. Epub 2018 Feb 1 doi: 10.1016/j.ejim.2018.01.015. PMID: 29361475

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