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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

New Directions in Treatment of Metastatic or Advanced Pheochromocytomas and Sympathetic Paragangliomas: an American, Contemporary, Pragmatic Approach.

Jimenez C, Xu G, Varghese J, Graham PH, Campbell MT, Lu Y
Curr Oncol Rep 2022 Jan;24(1):89-98. Epub 2022 Jan 21 doi: 10.1007/s11912-022-01197-0. PMID: 35061191

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

See all (8)

Recent clinical studies

Etiology

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

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

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

Metastatic Cutaneous Paraganglioma: A Case Report and Review of the Literature.

de Leon D, Walton K, Sokumbi O
Am J Dermatopathol 2017 Sep;39(9):700-703. doi: 10.1097/DAD.0000000000000965. PMID: 28763336

See all (19)

Diagnosis

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 use of temozolomide in paediatric metastatic phaeochromocytoma/paraganglioma: A case report and literature review.

Urquhart C, Fleming B, Harper I, Aloj L, Armstrong R, Hook L, Long AM, Jackson C, Gallagher FA, McLean MA, Tarpey P, Kosmoliaptsis V, Nicholson J, Hendriks AEJ, Casey RT
Front Endocrinol (Lausanne) 2022;13:1066208. Epub 2022 Nov 9 doi: 10.3389/fendo.2022.1066208. PMID: 36440187 Free PMC Article

Metastatic Cutaneous Paraganglioma: A Case Report and Review of the Literature.

de Leon D, Walton K, Sokumbi O
Am J Dermatopathol 2017 Sep;39(9):700-703. doi: 10.1097/DAD.0000000000000965. PMID: 28763336

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 (45)

Therapy

The use of temozolomide in paediatric metastatic phaeochromocytoma/paraganglioma: A case report and literature review.

(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

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

Recent advances in the management of malignant pheochromocytoma and paraganglioma: focus on tyrosine kinase and hypoxia-inducible factor inhibitors.

Toledo R, Jimenez C
F1000Res 2018;7 Epub 2018 Jul 30 doi: 10.12688/f1000research.13995.1. PMID: 30109021 Free PMC Article

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

See all (29)

Prognosis

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

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.

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

Metastatic paraganglioma of the spine: case report and review of the literature.

Lau D, La Marca F, Camelo-Piragua S, Park P
Clin Neurol Neurosurg 2013 Sep;115(9):1571-4. Epub 2013 Feb 9 doi: 10.1016/j.clineuro.2013.01.006. PMID: 23398849

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

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

Glutamine Synthetase Immunoreactivity in Peritumoral Hyperplasia in Liver: Case Report of a Metastatic Paraganglioma With Focal Nodular Hyperplasia-Like Changes and Review of an Additional 54 Liver Masses.

Bryant BH, Zenali MJ, Swanson PE, Upton MP, Yeh MM, Cuevas C, Park JO, Westerhoff M
Am J Clin Pathol 2016 Aug;146(2):254-61. Epub 2016 May 31 doi: 10.1093/ajcp/aqw056. PMID: 27247379

Urinary bladder malignant paraganglioma with vertebral metastasis: a case report with literature review.

Feng N, Li X, Gao HD, Liu ZL, Shi LJ, Liu WZ
Chin J Cancer 2013 Nov;32(11):624-8. Epub 2013 May 14 doi: 10.5732/cjc.012.10317. PMID: 23668927 Free PMC Article

Sunitinib for the treatment of metastatic paraganglioma and vasoactive intestinal polypeptide-producing tumor (VIPoma).

Bourcier ME, Vinik AI
Pancreas 2013 Mar;42(2):348-52. doi: 10.1097/MPA.0b013e31825c53fa. PMID: 23407483

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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