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Metastatic Paraganglioma

MedGen UID:: 288558
•Concept ID:: C1533592
•: Neoplastic Process

Synonyms:	Malignant Neoplasm of Paraganglion; Malignant Neoplasm of the Paraganglion; Malignant paraganglioma; Malignant Paraganglioma; Malignant Paraganglion Neoplasm; Malignant Paraganglion Tumor; Malignant Paraganglionic Neoplasm; Malignant Paraganglionic Tumor; Malignant Tumor of Paraganglion; Malignant Tumor of the Paraganglion; Paraganglioma, malignant; Paraganglion Neoplasm, Malignant
SNOMED CT:	Malignant paraganglioma (9903002); Paraganglioma, malignant (9903002)

Definition

A paraganglioma that metastasizes to regional or distant anatomic sites. Extraadrenal paragangliomas have a higher tendency to metastasize, as compared to pheochromocytomas. Common sites of metastasis include the lymph nodes, lungs, bones, and liver. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVMetastatic Paraganglioma

Pathological Conditions, Signs and Symptoms
- Pathological process
  - Disease
    - Neoplasm
      - Malignant neoplastic disease
        Malignant neoplasm of endocrine gland
        Metastatic Paraganglioma
        Malignant adrenal gland pheochromocytoma
        Metastatic Adrenal Gland Composite Pheochromocytoma
        Metastatic Extra-Adrenal Paraganglioma
        Malignant carotid body paraganglioma
        Malignant jugulotympanic paraganglioma
        Metastatic Bladder Paraganglioma
        Metastatic Cardiac Paraganglioma
        Metastatic Intrathoracic Paravertebral Paraganglioma

Professional guidelines

PubMed

Metastatic pheochromocytoma and paraganglioma: Management of endocrine manifestations, surgery and ablative procedures, and systemic therapies.

Jasim S, Jimenez C
Best Pract Res Clin Endocrinol Metab 2020 Mar;34(2):101354. Epub 2019 Oct 24 doi: 10.1016/j.beem.2019.101354. PMID: 31685417

Treatment and outcome of metastatic paraganglioma of the spine.

Jia Q, Yin H, Yang J, Wu Z, Yan W, Zhou W, Yang X, Xiao J
Eur Spine J 2018 Apr;27(4):859-867. Epub 2017 Jun 26 doi: 10.1007/s00586-017-5140-5. PMID: 28653097

Treatment of metastatic carcinoid tumours, phaeochromocytoma, paraganglioma and medullary carcinoma of the thyroid with (131)I-meta-iodobenzylguanidine [(131)I-mIBG].

Mukherjee JJ, Kaltsas GA, Islam N, Plowman PN, Foley R, Hikmat J, Britton KE, Jenkins PJ, Chew SL, Monson JP, Besser GM, Grossman AB
Clin Endocrinol (Oxf) 2001 Jul;55(1):47-60. doi: 10.1046/j.1365-2265.2001.01309.x. PMID: 11453952

See all (9)

Recent clinical studies

Etiology

Treatment of metastatic paraganglioma: experience of a single center.

Veríssimo D, Regala C, Damásio I, Santos S, Donato S, Leite V
Endocrine 2024 Jun;84(3):1250-1257. Epub 2024 Feb 1 doi: 10.1007/s12020-024-03700-y. PMID: 38296912

Paraganglioma in pregnancy, a mimic of preeclampsia: a case report.

Lundholm MD, Marquard J, Rao PP
J Med Case Rep 2023 Apr 7;17(1):124. doi: 10.1186/s13256-023-03871-8. PMID: 37024931 Free PMC Article

Management of primary cardiac paraganglioma.

Chan EY, Ali A, Umana JP, Nguyen DT, Hamilton DJ, Graviss EA, Ravi V, MacGillivray TE, Reardon MJ
J Thorac Cardiovasc Surg 2022 Jul;164(1):158-166.e1. Epub 2020 Oct 3 doi: 10.1016/j.jtcvs.2020.09.100. PMID: 33148444

Efficacy of Peptide Receptor Radionuclide Therapy for Functional Metastatic Paraganglioma and Pheochromocytoma.

Kong G, Grozinsky-Glasberg S, Hofman MS, Callahan J, Meirovitz A, Maimon O, Pattison DA, Gross DJ, Hicks RJ
J Clin Endocrinol Metab 2017 Sep 1;102(9):3278-3287. doi: 10.1210/jc.2017-00816. PMID: 28605448

The phenotype of SDHB germline mutation carriers: a nationwide study.

Niemeijer ND, Rijken JA, Eijkelenkamp K, van der Horst-Schrivers ANA, Kerstens MN, Tops CMJ, van Berkel A, Timmers HJLM, Kunst HPM, Leemans CR, Bisschop PH, Dreijerink KMA, van Dooren MF, Bayley JP, Pereira AM, Jansen JC, Hes FJ, Hensen EF, Corssmit EPM
Eur J Endocrinol 2017 Aug;177(2):115-125. Epub 2017 May 10 doi: 10.1530/EJE-17-0074. PMID: 28490599

See all (20)

Diagnosis

Paraganglioma in pregnancy, a mimic of preeclampsia: a case report.

Lundholm MD, Marquard J, Rao PP
J Med Case Rep 2023 Apr 7;17(1):124. doi: 10.1186/s13256-023-03871-8. PMID: 37024931 Free PMC Article

Metastatic paraganglioma.

Harmsworth D, Mallia A, DeGaetano J, Cassar PJ
BMJ Case Rep 2023 Jan 17;16(1) doi: 10.1136/bcr-2022-252449. PMID: 36653043 Free PMC Article

Primary and metastatic paraganglioma of the cranial vault.

Kakodkar P, Houlihan LM, Preul M, Bermingham N, Lim C
Br J Neurosurg 2023 Oct;37(5):967-975. Epub 2021 Mar 19 doi: 10.1080/02688697.2021.1902477. PMID: 33739182

The phenotype of SDHB germline mutation carriers: a nationwide study.

Metastatic paraganglioma.

Fliedner SM, Lehnert H, Pacak K
Semin Oncol 2010 Dec;37(6):627-37. doi: 10.1053/j.seminoncol.2010.10.017. PMID: 21167381 Free PMC Article

See all (46)

Therapy

Paraganglioma in pregnancy, a mimic of preeclampsia: a case report.

Lundholm MD, Marquard J, Rao PP
J Med Case Rep 2023 Apr 7;17(1):124. doi: 10.1186/s13256-023-03871-8. PMID: 37024931 Free PMC Article

(131)I-MIBG negative progressive symptomatic metastatic paraganglioma: response and outcome with (177)Lu-DOTATATE peptide receptor radionuclide therapy.

Parghane RV, Talole S, Basu S
Ann Nucl Med 2021 Jan;35(1):92-101. Epub 2020 Nov 1 doi: 10.1007/s12149-020-01541-z. PMID: 33135123

Case Report: Primary Hypothyroidism Associated With Lutetium 177-DOTATATE Therapy for Metastatic Paraganglioma.

Gubbi S, Al-Jundi M, Del Rivero J, Jha A, Knue M, Zou J, Turkbey B, Carrasquillo JA, Lin E, Pacak K, Klubo-Gwiezdzinska J, Lin FI
Front Endocrinol (Lausanne) 2020;11:587065. Epub 2021 Jan 21 doi: 10.3389/fendo.2020.587065. PMID: 33551992 Free PMC Article

Metastatic pheochromocytoma and paraganglioma: Management of endocrine manifestations, surgery and ablative procedures, and systemic therapies.

Jasim S, Jimenez C
Best Pract Res Clin Endocrinol Metab 2020 Mar;34(2):101354. Epub 2019 Oct 24 doi: 10.1016/j.beem.2019.101354. PMID: 31685417

Efficacy of Peptide Receptor Radionuclide Therapy for Functional Metastatic Paraganglioma and Pheochromocytoma.

See all (29)

Prognosis

Treatment and outcome of metastatic paraganglioma of the spine.

Jia Q, Yin H, Yang J, Wu Z, Yan W, Zhou W, Yang X, Xiao J
Eur Spine J 2018 Apr;27(4):859-867. Epub 2017 Jun 26 doi: 10.1007/s00586-017-5140-5. PMID: 28653097

Efficacy of Peptide Receptor Radionuclide Therapy for Functional Metastatic Paraganglioma and Pheochromocytoma.

The phenotype of SDHB germline mutation carriers: a nationwide study.

Use of angioembolization as an effective technique for the management of pediatric solid tumors.

Krauel L, Albert A, Mora J, Sola T, Cruz O, Mortera C, Ribó JM
J Pediatr Surg 2009 Sep;44(9):1848-55. doi: 10.1016/j.jpedsurg.2009.04.037. PMID: 19735840

Treatment of metastatic carcinoid tumours, phaeochromocytoma, paraganglioma and medullary carcinoma of the thyroid with (131)I-meta-iodobenzylguanidine [(131)I-mIBG].

See all (26)

Clinical prediction guides

Fine-needle aspiration biopsy for suspected uveal metastases.

Bellerive C, Biscotti CV, Singh N, Singh AD
Can J Ophthalmol 2019 Dec;54(6):694-698. Epub 2019 May 31 doi: 10.1016/j.jcjo.2019.03.016. PMID: 31836102

Paraganglioma of the spine: A twenty-years clinical experience of a high volume tumor center.

Pipola V, Boriani S, Bandiera S, Righi A, Barbanti Bròdano G, Terzi S, Ghermandi R, Tedesco G, Evangelisti G, Girolami M, Gasbarrini A
J Clin Neurosci 2019 Aug;66:7-11. Epub 2019 May 29 doi: 10.1016/j.jocn.2019.05.037. PMID: 31153748

Germline Mutations in the Mitochondrial 2-Oxoglutarate/Malate Carrier SLC25A11 Gene Confer a Predisposition to Metastatic Paragangliomas.

Buffet A, Morin A, Castro-Vega LJ, Habarou F, Lussey-Lepoutre C, Letouzé E, Lefebvre H, Guilhem I, Haissaguerre M, Raingeard I, Padilla-Girola M, Tran T, Tchara L, Bertherat J, Amar L, Ottolenghi C, Burnichon N, Gimenez-Roqueplo AP, Favier J
Cancer Res 2018 Apr 15;78(8):1914-1922. Epub 2018 Feb 5 doi: 10.1158/0008-5472.CAN-17-2463. PMID: 29431636

The phenotype of SDHB germline mutation carriers: a nationwide study.

Arsenic Trioxide as a Radiation Sensitizer for 131I-Metaiodobenzylguanidine Therapy: Results of a Phase II Study.

Modak S, Zanzonico P, Carrasquillo JA, Kushner BH, Kramer K, Cheung NK, Larson SM, Pandit-Taskar N
J Nucl Med 2016 Feb;57(2):231-7. Epub 2016 Jan 7 doi: 10.2967/jnumed.115.161752. PMID: 26742708 Free PMC Article

See all (13)

Recent systematic reviews

Primary and metastatic paraganglioma of the cranial vault.

Kakodkar P, Houlihan LM, Preul M, Bermingham N, Lim C
Br J Neurosurg 2023 Oct;37(5):967-975. Epub 2021 Mar 19 doi: 10.1080/02688697.2021.1902477. PMID: 33739182

See all (1)

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Metastatic Paraganglioma

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Practice guidelines

Related information

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