U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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]

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: 33329393Free 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

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: 32877928Free 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

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
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: 36653043Free 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
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: 33588146Free PMC Article
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: 32877928Free PMC Article

Therapy

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: 33588146Free PMC Article
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: 33329393Free 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
Complications of mediastinal neural tumours.
Parish C
Thorax 1971 Jul;26(4):392-5. doi: 10.1136/thx.26.4.392. PMID: 4327710Free PMC Article

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: 37033265Free 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
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: 32877928Free PMC Article
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

Clinical prediction guides

Preoperative prediction of metastatic pheochromocytoma and paraganglioma using clinical, genetic, and biochemical markers: A cohort study.
Park SS, Ahn CH, Lee S, Lee W, Kim WW, Lee YM, Kim SJ, Sung TY, Lee KE, Kim JH, Lee SH, Koh JM
J Intern Med 2024 Jul;296(1):68-79. Epub 2024 Apr 24 doi: 10.1111/joim.13791. PMID: 38659304
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: 35903274Free 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.
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: 32877928Free PMC Article
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

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: 36339441Free 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: 34021421Free 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

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...