Full Report

Format

Full Report
Summary (Text)
Summary (XML)

Send to:

Choose Destination

File
Clipboard
Collections

Metastatic Extra-Adrenal Paraganglioma

MedGen UID:: 87237
•Concept ID:: C0334418
•: Neoplastic Process

Synonyms:	Extra-adrenal paraganglioma, malignant; Malignant extra-adrenal paraganglioma; Malignant Extra-Adrenal Paraganglioma; Malignant non-chromaffin paraganglioma; Nonchromaffin paraganglioma, malignant
SNOMED CT:	Malignant non-chromaffin paraganglioma (32512003); Malignant extra-adrenal paraganglioma (32512003); Extra-adrenal paraganglioma, malignant (32512003); Nonchromaffin paraganglioma, malignant (32512003)

Definition

An extra-adrenal paraganglioma that metastasizes to regional or distant anatomic sites. 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 Extra-Adrenal Paraganglioma

Pathological Conditions, Signs and Symptoms
- Pathological process
  - Disease
    - Neoplasm
      - Extra-Adrenal Paraganglioma
        Metastatic Extra-Adrenal Paraganglioma
        Malignant carotid body paraganglioma
        Malignant jugulotympanic paraganglioma
        Metastatic Bladder Paraganglioma
        Metastatic Cardiac Paraganglioma
        Metastatic Intrathoracic Paravertebral Paraganglioma

Professional guidelines

PubMed

A Case for Genetic Testing in Isolated Tympanic Paragangliomas.

Shrivastava MK, Curran JF, Sheerin F, Mohammadi BJ, Halliday D, MacKeith SAC
Otol Neurotol 2022 Aug 1;43(7):840-844. Epub 2022 Jul 4 doi: 10.1097/MAO.0000000000003557. PMID: 35802032

The VHL/HIF Axis in the Development and Treatment of Pheochromocytoma/Paraganglioma.

Peng S, Zhang J, Tan X, Huang Y, Xu J, Silk N, Zhang D, Liu Q, Jiang J
Front Endocrinol (Lausanne) 2020;11:586857. Epub 2020 Nov 24 doi: 10.3389/fendo.2020.586857. PMID: 33329393 Free PMC Article

Recent advances in the management of pheochromocytoma and paraganglioma.

Tanabe A, Naruse M
Hypertens Res 2020 Nov;43(11):1141-1151. Epub 2020 Aug 11 doi: 10.1038/s41440-020-0531-0. PMID: 32778780

See all (16)

Recent clinical studies

Etiology

Biochemical Assessment of Pheochromocytoma and Paraganglioma.

Eisenhofer G, Pamporaki C, Lenders JWM
Endocr Rev 2023 Sep 15;44(5):862-909. doi: 10.1210/endrev/bnad011. PMID: 36996131

Advances in Adrenal and Extra-adrenal Paraganglioma: Practical Synopsis for Pathologists.

Juhlin CC, Mete O
Adv Anat Pathol 2023 Jan 1;30(1):47-57. Epub 2022 Sep 23 doi: 10.1097/PAP.0000000000000365. PMID: 36136370

Overview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas.

Mete O, Asa SL, Gill AJ, Kimura N, de Krijger RR, Tischler A
Endocr Pathol 2022 Mar;33(1):90-114. Epub 2022 Mar 13 doi: 10.1007/s12022-022-09704-6. PMID: 35285002

Predicting Metastatic Potential in Pheochromocytoma and Paraganglioma: A Comparison of PASS and GAPP Scoring Systems.

Wachtel H, Hutchens T, Baraban E, Schwartz LE, Montone K, Baloch Z, LiVolsi V, Krumeich L, Fraker DL, Nathanson KL, Cohen DL, Fishbein L
J Clin Endocrinol Metab 2020 Dec 1;105(12):e4661-70. doi: 10.1210/clinem/dgaa608. PMID: 32877928 Free PMC Article

Pheochromocytoma/Paraganglioma: Is This a Genetic Disorder?

Fishbein L
Curr Cardiol Rep 2019 Jul 31;21(9):104. doi: 10.1007/s11886-019-1184-y. PMID: 31367972

See all (129)

Diagnosis

Biochemical Assessment of Pheochromocytoma and Paraganglioma.

Eisenhofer G, Pamporaki C, Lenders JWM
Endocr Rev 2023 Sep 15;44(5):862-909. doi: 10.1210/endrev/bnad011. PMID: 36996131

Overview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas.

Mete O, Asa SL, Gill AJ, Kimura N, de Krijger RR, Tischler A
Endocr Pathol 2022 Mar;33(1):90-114. Epub 2022 Mar 13 doi: 10.1007/s12022-022-09704-6. PMID: 35285002

Consensus on molecular imaging and theranostics in neuroendocrine neoplasms.

Ambrosini V, Kunikowska J, Baudin E, Bodei L, Bouvier C, Capdevila J, Cremonesi M, de Herder WW, Dromain C, Falconi M, Fani M, Fanti S, Hicks RJ, Kabasakal L, Kaltsas G, Lewington V, Minozzi S, Cinquini M, Öberg K, Oyen WJG, O'Toole D, Pavel M, Ruszniewski P, Scarpa A, Strosberg J, Sundin A, Taïeb D, Virgolini I, Wild D, Herrmann K, Yao J
Eur J Cancer 2021 Mar;146:56-73. Epub 2021 Feb 12 doi: 10.1016/j.ejca.2021.01.008. PMID: 33588146 Free PMC Article

Predicting Metastatic Potential in Pheochromocytoma and Paraganglioma: A Comparison of PASS and GAPP Scoring Systems.

Pheochromocytomas and Paragangliomas.

Tevosian SG, Ghayee HK
Endocrinol Metab Clin North Am 2019 Dec;48(4):727-750. doi: 10.1016/j.ecl.2019.08.006. PMID: 31655773

See all (244)

Therapy

Hypoxia-inducible Factor 2α: A Key Player in Tumorigenesis and Metastasis of Pheochromocytoma and Paraganglioma?

Bechmann N, Eisenhofer G
Exp Clin Endocrinol Diabetes 2022 May;130(5):282-289. Epub 2021 Jul 28 doi: 10.1055/a-1526-5263. PMID: 34320663

Consensus on molecular imaging and theranostics in neuroendocrine neoplasms.

131I-metaiodobenzylguanidine and peptide receptor radionuclide therapy in pheochromocytoma and paraganglioma.

Jungels C, Karfis I
Curr Opin Oncol 2021 Jan;33(1):33-39. doi: 10.1097/CCO.0000000000000691. PMID: 33093336

The VHL/HIF Axis in the Development and Treatment of Pheochromocytoma/Paraganglioma.

Peng S, Zhang J, Tan X, Huang Y, Xu J, Silk N, Zhang D, Liu Q, Jiang J
Front Endocrinol (Lausanne) 2020;11:586857. Epub 2020 Nov 24 doi: 10.3389/fendo.2020.586857. PMID: 33329393 Free PMC Article

Gastrointestinal stromal tumour.

Joensuu H, Hohenberger P, Corless CL
Lancet 2013 Sep 14;382(9896):973-83. Epub 2013 Apr 24 doi: 10.1016/S0140-6736(13)60106-3. PMID: 23623056

See all (48)

Prognosis

Tumour microenvironment in pheochromocytoma and paraganglioma.

Martinelli S, Amore F, Canu L, Maggi M, Rapizzi E
Front Endocrinol (Lausanne) 2023;14:1137456. Epub 2023 Mar 22 doi: 10.3389/fendo.2023.1137456. PMID: 37033265 Free PMC Article

Advances in Adrenal and Extra-adrenal Paraganglioma: Practical Synopsis for Pathologists.

Juhlin CC, Mete O
Adv Anat Pathol 2023 Jan 1;30(1):47-57. Epub 2022 Sep 23 doi: 10.1097/PAP.0000000000000365. PMID: 36136370

Overview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas.

Mete O, Asa SL, Gill AJ, Kimura N, de Krijger RR, Tischler A
Endocr Pathol 2022 Mar;33(1):90-114. Epub 2022 Mar 13 doi: 10.1007/s12022-022-09704-6. PMID: 35285002

Epigenetic and metabolic reprogramming of SDH-deficient paragangliomas.

Moog S, Lussey-Lepoutre C, Favier J
Endocr Relat Cancer 2020 Dec;27(12):R451-R463. doi: 10.1530/ERC-20-0346. PMID: 33112834

Predicting Metastatic Potential in Pheochromocytoma and Paraganglioma: A Comparison of PASS and GAPP Scoring Systems.

See all (118)

Clinical prediction guides

Pediatric Metastatic Pheochromocytoma and Paraganglioma: Clinical Presentation and Diagnosis, Genetics, and Therapeutic Approaches.

Kuo MJM, Nazari MA, Jha A, Pacak K
Front Endocrinol (Lausanne) 2022;13:936178. Epub 2022 Jul 12 doi: 10.3389/fendo.2022.936178. PMID: 35903274 Free PMC Article

Overview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas.

Mete O, Asa SL, Gill AJ, Kimura N, de Krijger RR, Tischler A
Endocr Pathol 2022 Mar;33(1):90-114. Epub 2022 Mar 13 doi: 10.1007/s12022-022-09704-6. PMID: 35285002

Predicting Metastatic Potential in Pheochromocytoma and Paraganglioma: A Comparison of PASS and GAPP Scoring Systems.

Recent advances in the management of pheochromocytoma and paraganglioma.

Tanabe A, Naruse M
Hypertens Res 2020 Nov;43(11):1141-1151. Epub 2020 Aug 11 doi: 10.1038/s41440-020-0531-0. PMID: 32778780

Nonparaganglioma jugular foramen tumors.

Thomas AJ, Wiggins RH 3rd, Gurgel RK
Otolaryngol Clin North Am 2015 Apr;48(2):343-59. Epub 2015 Feb 4 doi: 10.1016/j.otc.2014.12.008. PMID: 25659512

See all (76)

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

Response to targeted radionuclide therapy with [(131)I]MIBG AND [(177)Lu]Lu-DOTA-TATE according to adrenal vs. extra-adrenal primary location in metastatic paragangliomas and pheochromocytomas: A systematic review.

Prado-Wohlwend S, Del Olmo-García MI, Bello-Arques P, Merino-Torres JF
Front Endocrinol (Lausanne) 2022;13:957172. Epub 2022 Oct 20 doi: 10.3389/fendo.2022.957172. PMID: 36339441 Free PMC Article

Paraganglioma of the cauda equina: a tertiary centre experience and scoping review of the current literature.

Shtaya A, Iorga R, Hettige S, Bridges LR, Stapleton S, Johnston FG
Neurosurg Rev 2022 Feb;45(1):103-118. Epub 2021 May 21 doi: 10.1007/s10143-021-01565-7. PMID: 34021421 Free PMC Article

The incremental benefit of functional imaging in pheochromocytoma/paraganglioma: a systematic review.

Brito JP, Asi N, Gionfriddo MR, Norman C, Leppin AL, Zeballos-Palacios C, Undavalli C, Wang Z, Domecq JP, Prustsky G, Elraiyah TA, Prokop LJ, Montori VM, Murad MH
Endocrine 2015 Sep;50(1):176-86. Epub 2015 Feb 6 doi: 10.1007/s12020-015-0544-7. PMID: 25663601

Comparison of metaiodobenzylguanidine scintigraphy with positron emission tomography in the diagnostic work-up of pheochromocytoma and paraganglioma: a systematic review.

Rufini V, Treglia G, Castaldi P, Perotti G, Giordano A
Q J Nucl Med Mol Imaging 2013 Jun;57(2):122-33. PMID: 23822989

See all (6)

MedGen

National Center for Biotechnology Information

Send to:

Metastatic Extra-Adrenal 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

Recent activity