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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

Management and outcome of metastatic pheochromocytomas/paragangliomas: a monocentric experience.
De Filpo G, Cantini G, Rastrelli G, Vannini G, Ercolino T, Luconi M, Mannelli M, Maggi M, Canu L
J Endocrinol Invest 2022 Jan;45(1):149-157. Epub 2021 Jul 5 doi: 10.1007/s40618-021-01629-x. PMID: 34227051Free PMC Article
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

Recurrent Disease in Patients With Sporadic Pheochromocytoma and Paraganglioma.
Li M, Prodanov T, Meuter L, Kerstens MN, Bechmann N, Prejbisz A, Remde H, Timmers HJLM, Nölting S, Talvacchio S, Berends AMA, Fliedner S, Robledo M, Lenders JWM, Pacak K, Eisenhofer G, Pamporaki C
J Clin Endocrinol Metab 2023 Jan 17;108(2):397-404. doi: 10.1210/clinem/dgac563. PMID: 36190922Free PMC Article
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
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

Diagnosis

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
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
PHEOCHROMOCYTOMA: A GENETIC AND DIAGNOSTIC UPDATE.
Mercado-Asis LB, Wolf KI, Jochmanova I, Taïeb D
Endocr Pract 2018 Jan;24(1):78-90. Epub 2017 Nov 16 doi: 10.4158/EP-2017-0057. PMID: 29144820

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.
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

Prognosis

Recurrent Disease in Patients With Sporadic Pheochromocytoma and Paraganglioma.
Li M, Prodanov T, Meuter L, Kerstens MN, Bechmann N, Prejbisz A, Remde H, Timmers HJLM, Nölting S, Talvacchio S, Berends AMA, Fliedner S, Robledo M, Lenders JWM, Pacak K, Eisenhofer G, Pamporaki C
J Clin Endocrinol Metab 2023 Jan 17;108(2):397-404. doi: 10.1210/clinem/dgac563. PMID: 36190922Free 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.
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

Clinical prediction guides

Recurrent Disease in Patients With Sporadic Pheochromocytoma and Paraganglioma.
Li M, Prodanov T, Meuter L, Kerstens MN, Bechmann N, Prejbisz A, Remde H, Timmers HJLM, Nölting S, Talvacchio S, Berends AMA, Fliedner S, Robledo M, Lenders JWM, Pacak K, Eisenhofer G, Pamporaki C
J Clin Endocrinol Metab 2023 Jan 17;108(2):397-404. doi: 10.1210/clinem/dgac563. PMID: 36190922Free PMC Article
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
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 systematic reviews

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 clinical characteristics and outcomes of carotid body tumors in Chinese patients: A STROBE-compliant observational study.
Chen Y, Li Y, Liu J, Yang L
Medicine (Baltimore) 2020 Jan;99(3):e18824. doi: 10.1097/MD.0000000000018824. PMID: 32011493Free 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

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