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Pheochromocytoma-paraganglioma

MedGen UID:
1826130
Concept ID:
C5681712
Neoplastic Process
Synonym: pheochromocytoma-paraganglioma
 
Monarch Initiative: MONDO:0035540
Orphanet: ORPHA573163

Definition

A rare neuroendocrine tumor arising from chromaffin cells of the adrenal medulla (pheochromocytoma) or from sympathetic and parasympathetic ganglia (paraganglioma). These tumors are most often benign and may produce catecholamines in excess causing hypertension and sometimes severe acute cardiovascular complications. [from ORDO]

Professional guidelines

PubMed

Sharma S, Fishbein L
Endocr Pract 2023 Dec;29(12):999-1006. Epub 2023 Aug 15 doi: 10.1016/j.eprac.2023.07.027. PMID: 37586639
Nölting S, Bechmann N, Taieb D, Beuschlein F, Fassnacht M, Kroiss M, Eisenhofer G, Grossman A, Pacak K
Endocr Rev 2022 Mar 9;43(2):199-239. doi: 10.1210/endrev/bnab019. PMID: 34147030Free PMC Article
Rossi GP, Bisogni V, Rossitto G, Maiolino G, Cesari M, Zhu R, Seccia TM
High Blood Press Cardiovasc Prev 2020 Dec;27(6):547-560. Epub 2020 Nov 7 doi: 10.1007/s40292-020-00415-9. PMID: 33159664Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Neuroendocrine and Adrenal Tumors, 2023

Recent clinical studies

Etiology

Sharma S, Fishbein L
Endocr Pract 2023 Dec;29(12):999-1006. Epub 2023 Aug 15 doi: 10.1016/j.eprac.2023.07.027. PMID: 37586639
Calsina B, Piñeiro-Yáñez E, Martínez-Montes ÁM, Caleiras E, Fernández-Sanromán Á, Monteagudo M, Torres-Pérez R, Fustero-Torre C, Pulgarín-Alfaro M, Gil E, Letón R, Jiménez S, García-Martín S, Martin MC, Roldán-Romero JM, Lanillos J, Mellid S, Santos M, Díaz-Talavera A, Rubio Á, González P, Hernando B, Bechmann N, Dona M, Calatayud M, Guadalix S, Álvarez-Escolá C, Regojo RM, Aller J, Del Olmo-Garcia MI, López-Fernández A, Fliedner SMJ, Rapizzi E, Fassnacht M, Beuschlein F, Quinkler M, Toledo RA, Mannelli M, Timmers HJ, Eisenhofer G, Rodríguez-Perales S, Domínguez O, Macintyre G, Currás-Freixes M, Rodríguez-Antona C, Cascón A, Leandro-García LJ, Montero-Conde C, Roncador G, García-García JF, Pacak K, Al-Shahrour F, Robledo M
Nat Commun 2023 Feb 28;14(1):1122. doi: 10.1038/s41467-023-36769-6. PMID: 36854674Free PMC Article
Nölting S, Bechmann N, Taieb D, Beuschlein F, Fassnacht M, Kroiss M, Eisenhofer G, Grossman A, Pacak K
Endocr Rev 2022 Mar 9;43(2):199-239. doi: 10.1210/endrev/bnab019. PMID: 34147030Free PMC Article
Jha A, Taïeb D, Carrasquillo JA, Pryma DA, Patel M, Millo C, de Herder WW, Del Rivero J, Crona J, Shulkin BL, Virgolini I, Chen AP, Mittal BR, Basu S, Dillon JS, Hope TA, Mari Aparici C, Iagaru AH, Hicks RJ, Avram AM, Strosberg JR, Civelek AC, Lin FI, Pandit-Taskar N, Pacak K
Clin Cancer Res 2021 Jun 1;27(11):2989-2995. Epub 2021 Mar 8 doi: 10.1158/1078-0432.CCR-20-3703. PMID: 33685867Free PMC Article
Rossi GP, Bisogni V, Rossitto G, Maiolino G, Cesari M, Zhu R, Seccia TM
High Blood Press Cardiovasc Prev 2020 Dec;27(6):547-560. Epub 2020 Nov 7 doi: 10.1007/s40292-020-00415-9. PMID: 33159664Free PMC Article

Diagnosis

Sharma S, Fishbein L
Endocr Pract 2023 Dec;29(12):999-1006. Epub 2023 Aug 15 doi: 10.1016/j.eprac.2023.07.027. PMID: 37586639
Lima JV Júnior, Kater CE
Int Braz J Urol 2023 May-Jun;49(3):307-319. doi: 10.1590/S1677-5538.IBJU.2023.0038. PMID: 37115176Free PMC Article
Nölting S, Bechmann N, Taieb D, Beuschlein F, Fassnacht M, Kroiss M, Eisenhofer G, Grossman A, Pacak K
Endocr Rev 2022 Mar 9;43(2):199-239. doi: 10.1210/endrev/bnab019. PMID: 34147030Free PMC Article
Granberg D, Juhlin CC, Falhammar H
J Clin Endocrinol Metab 2021 Apr 23;106(5):e1937-e1952. doi: 10.1210/clinem/dgaa982. PMID: 33462603Free PMC Article
Rossi GP, Bisogni V, Rossitto G, Maiolino G, Cesari M, Zhu R, Seccia TM
High Blood Press Cardiovasc Prev 2020 Dec;27(6):547-560. Epub 2020 Nov 7 doi: 10.1007/s40292-020-00415-9. PMID: 33159664Free PMC Article

Therapy

Reel PS, Reel S, van Kralingen JC, Langton K, Lang K, Erlic Z, Larsen CK, Amar L, Pamporaki C, Mulatero P, Blanchard A, Kabat M, Robertson S, MacKenzie SM, Taylor AE, Peitzsch M, Ceccato F, Scaroni C, Reincke M, Kroiss M, Dennedy MC, Pecori A, Monticone S, Deinum J, Rossi GP, Lenzini L, McClure JD, Nind T, Riddell A, Stell A, Cole C, Sudano I, Prehn C, Adamski J, Gimenez-Roqueplo AP, Assié G, Arlt W, Beuschlein F, Eisenhofer G, Davies E, Zennaro MC, Jefferson E
EBioMedicine 2022 Oct;84:104276. Epub 2022 Sep 27 doi: 10.1016/j.ebiom.2022.104276. PMID: 36179553Free PMC Article
Jha A, Taïeb D, Carrasquillo JA, Pryma DA, Patel M, Millo C, de Herder WW, Del Rivero J, Crona J, Shulkin BL, Virgolini I, Chen AP, Mittal BR, Basu S, Dillon JS, Hope TA, Mari Aparici C, Iagaru AH, Hicks RJ, Avram AM, Strosberg JR, Civelek AC, Lin FI, Pandit-Taskar N, Pacak K
Clin Cancer Res 2021 Jun 1;27(11):2989-2995. Epub 2021 Mar 8 doi: 10.1158/1078-0432.CCR-20-3703. PMID: 33685867Free PMC Article
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
Jimenez C, Fazeli S, Román-Gonzalez A
Endocr Relat Cancer 2020 Jul;27(7):R239-R254. doi: 10.1530/ERC-20-0043. PMID: 32369773
Simó M, Navarro X, Yuste VJ, Bruna J
Clin Auton Res 2018 Jun;28(3):301-314. Epub 2018 Mar 28 doi: 10.1007/s10286-018-0523-1. PMID: 29594605

Prognosis

Szatko A, Glinicki P, Gietka-Czernel M
Front Endocrinol (Lausanne) 2023;14:1204851. Epub 2023 Jul 13 doi: 10.3389/fendo.2023.1204851. PMID: 37522121Free PMC Article
Calsina B, Piñeiro-Yáñez E, Martínez-Montes ÁM, Caleiras E, Fernández-Sanromán Á, Monteagudo M, Torres-Pérez R, Fustero-Torre C, Pulgarín-Alfaro M, Gil E, Letón R, Jiménez S, García-Martín S, Martin MC, Roldán-Romero JM, Lanillos J, Mellid S, Santos M, Díaz-Talavera A, Rubio Á, González P, Hernando B, Bechmann N, Dona M, Calatayud M, Guadalix S, Álvarez-Escolá C, Regojo RM, Aller J, Del Olmo-Garcia MI, López-Fernández A, Fliedner SMJ, Rapizzi E, Fassnacht M, Beuschlein F, Quinkler M, Toledo RA, Mannelli M, Timmers HJ, Eisenhofer G, Rodríguez-Perales S, Domínguez O, Macintyre G, Currás-Freixes M, Rodríguez-Antona C, Cascón A, Leandro-García LJ, Montero-Conde C, Roncador G, García-García JF, Pacak K, Al-Shahrour F, Robledo M
Nat Commun 2023 Feb 28;14(1):1122. doi: 10.1038/s41467-023-36769-6. PMID: 36854674Free PMC Article
Nölting S, Bechmann N, Taieb D, Beuschlein F, Fassnacht M, Kroiss M, Eisenhofer G, Grossman A, Pacak K
Endocr Rev 2022 Mar 9;43(2):199-239. doi: 10.1210/endrev/bnab019. PMID: 34147030Free PMC Article
Granberg D, Juhlin CC, Falhammar H
J Clin Endocrinol Metab 2021 Apr 23;106(5):e1937-e1952. doi: 10.1210/clinem/dgaa982. PMID: 33462603Free PMC Article
Rossi GP, Bisogni V, Rossitto G, Maiolino G, Cesari M, Zhu R, Seccia TM
High Blood Press Cardiovasc Prev 2020 Dec;27(6):547-560. Epub 2020 Nov 7 doi: 10.1007/s40292-020-00415-9. PMID: 33159664Free PMC Article

Clinical prediction guides

Fischer A, Kloos S, Maccio U, Friemel J, Remde H, Fassnacht M, Pamporaki C, Eisenhofer G, Timmers HJLM, Robledo M, Fliedner SMJ, Wang K, Maurer J, Reul A, Zitzmann K, Bechmann N, Žygienė G, Richter S, Hantel C, Vetter D, Lehmann K, Mohr H, Pellegata NS, Ullrich M, Pietzsch J, Ziegler CG, Bornstein SR, Kroiss M, Reincke M, Pacak K, Grossman AB, Beuschlein F, Nölting S
J Clin Endocrinol Metab 2023 Sep 18;108(10):2676-2685. doi: 10.1210/clinem/dgad166. PMID: 36946182Free PMC Article
Reel PS, Reel S, van Kralingen JC, Langton K, Lang K, Erlic Z, Larsen CK, Amar L, Pamporaki C, Mulatero P, Blanchard A, Kabat M, Robertson S, MacKenzie SM, Taylor AE, Peitzsch M, Ceccato F, Scaroni C, Reincke M, Kroiss M, Dennedy MC, Pecori A, Monticone S, Deinum J, Rossi GP, Lenzini L, McClure JD, Nind T, Riddell A, Stell A, Cole C, Sudano I, Prehn C, Adamski J, Gimenez-Roqueplo AP, Assié G, Arlt W, Beuschlein F, Eisenhofer G, Davies E, Zennaro MC, Jefferson E
EBioMedicine 2022 Oct;84:104276. Epub 2022 Sep 27 doi: 10.1016/j.ebiom.2022.104276. PMID: 36179553Free PMC Article
Granberg D, Juhlin CC, Falhammar H
J Clin Endocrinol Metab 2021 Apr 23;106(5):e1937-e1952. doi: 10.1210/clinem/dgaa982. PMID: 33462603Free PMC Article
Rossi GP, Bisogni V, Rossitto G, Maiolino G, Cesari M, Zhu R, Seccia TM
High Blood Press Cardiovasc Prev 2020 Dec;27(6):547-560. Epub 2020 Nov 7 doi: 10.1007/s40292-020-00415-9. PMID: 33159664Free PMC Article
Bausch B, Schiavi F, Ni Y, Welander J, Patocs A, Ngeow J, Wellner U, Malinoc A, Taschin E, Barbon G, Lanza V, Söderkvist P, Stenman A, Larsson C, Svahn F, Chen JL, Marquard J, Fraenkel M, Walter MA, Peczkowska M, Prejbisz A, Jarzab B, Hasse-Lazar K, Petersenn S, Moeller LC, Meyer A, Reisch N, Trupka A, Brase C, Galiano M, Preuss SF, Kwok P, Lendvai N, Berisha G, Makay Ö, Boedeker CC, Weryha G, Racz K, Januszewicz A, Walz MK, Gimm O, Opocher G, Eng C, Neumann HPH; European-American-Asian Pheochromocytoma-Paraganglioma Registry Study Group
JAMA Oncol 2017 Sep 1;3(9):1204-1212. doi: 10.1001/jamaoncol.2017.0223. PMID: 28384794Free PMC Article

Recent systematic reviews

Zhang L, Åkerström T, Mollazadegan K, Beuschlein F, Pacak K, Skogseid B, Crona J
Endocr Relat Cancer 2023 Jul 1;30(7) Epub 2023 Jun 2 doi: 10.1530/ERC-22-0354. PMID: 37185155Free PMC Article
Wang J, Liu Q, Jiang S, Zhang J, He J, Li Y, Wang D
Int J Surg 2023 May 1;109(5):1470-1480. doi: 10.1097/JS9.0000000000000390. PMID: 37037514Free PMC Article
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
Brito JP, Asi N, Bancos I, Gionfriddo MR, Zeballos-Palacios CL, Leppin AL, Undavalli C, Wang Z, Domecq JP, Prustsky G, Elraiyah TA, Prokop LJ, Montori VM, Murad MH
Clin Endocrinol (Oxf) 2015 Mar;82(3):338-45. Epub 2014 Jul 7 doi: 10.1111/cen.12530. PMID: 24954084
Beilan JA, Lawton A, Hajdenberg J, Rosser CJ
BMC Urol 2013 Apr 29;13:22. doi: 10.1186/1471-2490-13-22. PMID: 23627260Free PMC Article

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    Clinical resources

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      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.

    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Neuroendocrine and Adrenal Tumors, 2023

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