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Sympathetic paraganglioma

MedGen UID:: 137758
•Concept ID:: C0334415
•: Neoplastic Process

Synonyms:	Chromaffin Neoplasm; chromaffin neoplasm; Chromaffin Tumor; chromaffin tumor; chromaffin tumour; 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]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVSympathetic paraganglioma

Abnormality of the nervous system
- Abnormal nervous system morphology
  - Neoplasm of the nervous system
    - Neoplasm of the peripheral nervous system
      - Autonomic nervous system neoplasm
        Paraganglioma
        Sympathetic paraganglioma
        Adrenal Gland Pheochromocytoma and Sympathetic Paraganglioma by AJCC v8 Stage
        Stage I Adrenal Gland Pheochromocytoma and Sympathetic Paraganglioma AJCC v8
        Stage II Adrenal Gland Pheochromocytoma and Sympathetic Paraganglioma AJCC v8
        Stage III Adrenal Gland Pheochromocytoma and Sympathetic Paraganglioma AJCC v8
        Stage IV Adrenal Gland Pheochromocytoma and Sympathetic Paraganglioma AJCC v8
        Adrenal pheochromocytoma
        Adrenal Gland Composite Pheochromocytoma
        Metastatic Adrenal Gland Composite Pheochromocytoma
        Non-Metastatic Adrenal Gland Composite Pheochromocytoma
        Benign adrenal gland pheochromocytoma
        Childhood adrenal gland pheochromocytoma
        Hereditary Adrenal Gland Pheochromocytoma
        Malignant adrenal gland pheochromocytoma
        Recurrent Adrenal Gland Pheochromocytoma
        Refractory Adrenal Gland Pheochromocytoma
        Regional Adrenal Gland Pheochromocytoma
        Unresectable Adrenal Gland Pheochromocytoma
        Extraadrenal pheochromocytoma
        Bladder Paraganglioma
        Metastatic Bladder Paraganglioma
        Extra-Adrenal Retroperitoneal Paraganglioma
        Superior and Inferior Paraaortic Paraganglioma
        Intrathoracic Paravertebral Paraganglioma
        Metastatic Intrathoracic Paravertebral Paraganglioma

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

See all (2)

Recent clinical studies

Etiology

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

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: 31714582 Free 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

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

See all (25)

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.

Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: A nationwide study and systematic review.

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

See all (30)

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.

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

See all (10)

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.

Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: A nationwide study and systematic review.

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

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

See all (22)

Clinical prediction guides

Efficacy of α-Blockers on Hemodynamic Control during Pheochromocytoma Resection: A Randomized Controlled Trial.

Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: A nationwide study and systematic review.

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

See all (22)

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.

See all (2)

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Sympathetic paraganglioma

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

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