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Regional Adrenal Gland Pheochromocytoma

MedGen UID:
318115
•Concept ID:
C1709884
•
Neoplastic Process
Synonyms: Regional Adrenal Gland Chromaffin Neoplasm; Regional Adrenal Gland Chromaffin Paraganglioma; Regional Adrenal Gland Chromaffin Tumor; Regional Adrenal Gland Chromaffinoma

Definition

A pheochromocytoma that has not spread to other regions. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRegional Adrenal Gland Pheochromocytoma

Professional guidelines

PubMed

Diagnosis and Management of Pheochromocytomas and Paragangliomas: A Guide for the Clinician.
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
Multidisciplinary practice guidelines for the diagnosis, genetic counseling and treatment of pheochromocytomas and paragangliomas.
Garcia-Carbonero R, Matute Teresa F, Mercader-Cidoncha E, Mitjavila-Casanovas M, Robledo M, Tena I, Alvarez-Escola C, Arístegui M, Bella-Cueto MR, Ferrer-Albiach C, Hanzu FA
Clin Transl Oncol 2021 Oct;23(10):1995-2019. Epub 2021 May 6 doi: 10.1007/s12094-021-02622-9. PMID: 33959901Free PMC Article
Pheochromocytoma and paraganglioma-an update on diagnosis, evaluation, and management.
Jain A, Baracco R, Kapur G
Pediatr Nephrol 2020 Apr;35(4):581-594. Epub 2019 Jan 2 doi: 10.1007/s00467-018-4181-2. PMID: 30603807

Recent clinical studies

Etiology

Diagnosis and Management of Pheochromocytomas and Paragangliomas: A Guide for the Clinician.
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
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
Genetics, diagnosis, management and future directions of research of phaeochromocytoma and paraganglioma: a position statement and consensus of the Working Group on Endocrine Hypertension of the European Society of Hypertension.
Lenders JWM, Kerstens MN, Amar L, Prejbisz A, Robledo M, Taieb D, Pacak K, Crona J, Zelinka T, Mannelli M, Deutschbein T, Timmers HJLM, Castinetti F, Dralle H, Widimský J, Gimenez-Roqueplo AP, Eisenhofer G
J Hypertens 2020 Aug;38(8):1443-1456. doi: 10.1097/HJH.0000000000002438. PMID: 32412940Free PMC Article
Pheochromocytoma and paraganglioma-an update on diagnosis, evaluation, and management.
Jain A, Baracco R, Kapur G
Pediatr Nephrol 2020 Apr;35(4):581-594. Epub 2019 Jan 2 doi: 10.1007/s00467-018-4181-2. PMID: 30603807
Clinical Characterization of the Pheochromocytoma and Paraganglioma Susceptibility Genes SDHA, TMEM127, MAX, and SDHAF2 for Gene-Informed Prevention.
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

Diagnosis

Adrenal Pathologies.
Johnson K
Prim Care 2024 Sep;51(3):417-430. Epub 2024 May 23 doi: 10.1016/j.pop.2024.03.005. PMID: 39067968
Diagnosis and Management of Pheochromocytomas and Paragangliomas: A Guide for the Clinician.
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
Multidisciplinary practice guidelines for the diagnosis, genetic counseling and treatment of pheochromocytomas and paragangliomas.
Garcia-Carbonero R, Matute Teresa F, Mercader-Cidoncha E, Mitjavila-Casanovas M, Robledo M, Tena I, Alvarez-Escola C, Arístegui M, Bella-Cueto MR, Ferrer-Albiach C, Hanzu FA
Clin Transl Oncol 2021 Oct;23(10):1995-2019. Epub 2021 May 6 doi: 10.1007/s12094-021-02622-9. PMID: 33959901Free PMC Article
Genetics, diagnosis, management and future directions of research of phaeochromocytoma and paraganglioma: a position statement and consensus of the Working Group on Endocrine Hypertension of the European Society of Hypertension.
Lenders JWM, Kerstens MN, Amar L, Prejbisz A, Robledo M, Taieb D, Pacak K, Crona J, Zelinka T, Mannelli M, Deutschbein T, Timmers HJLM, Castinetti F, Dralle H, Widimský J, Gimenez-Roqueplo AP, Eisenhofer G
J Hypertens 2020 Aug;38(8):1443-1456. doi: 10.1097/HJH.0000000000002438. PMID: 32412940Free PMC Article
Pheochromocytoma and paraganglioma-an update on diagnosis, evaluation, and management.
Jain A, Baracco R, Kapur G
Pediatr Nephrol 2020 Apr;35(4):581-594. Epub 2019 Jan 2 doi: 10.1007/s00467-018-4181-2. PMID: 30603807

Therapy

Prediction of metastatic pheochromocytoma and paraganglioma: a machine learning modelling study using data from a cross-sectional cohort.
Pamporaki C, Berends AMA, Filippatos A, Prodanov T, Meuter L, Prejbisz A, Beuschlein F, Fassnacht M, Timmers HJLM, Nölting S, Abhyankar K, Constantinescu G, Kunath C, de Haas RJ, Wang K, Remde H, Bornstein SR, Januszewicz A, Robledo M, Lenders JWM, Kerstens MN, Pacak K, Eisenhofer G
Lancet Digit Health 2023 Sep;5(9):e551-e559. Epub 2023 Jul 18 doi: 10.1016/S2589-7500(23)00094-8. PMID: 37474439Free PMC Article
Predictors of Hypotension after Adrenalectomy for Pheochromocytoma.
Koyama K, Miura N, Watanabe R, Sawada Y, Noda T, Nishimura K, Asai S, Fukumoto T, Yanagihara Y, Miyauchi Y, Kikugawa T, Saika T
Acta Med Okayama 2021 Jun;75(3):345-349. doi: 10.18926/AMO/62229. PMID: 34176938
Adrenal morphology and associated comorbidities in congenital adrenal hyperplasia.
El-Maouche D, Hannah-Shmouni F, Mallappa A, Hargreaves CJ, Avila NA, Merke DP
Clin Endocrinol (Oxf) 2019 Aug;91(2):247-255. Epub 2019 May 7 doi: 10.1111/cen.13996. PMID: 31001843Free PMC Article
Local anesthesia reversal.
Malamed SF
Dent Today 2010 Mar;29(3):65-6, 68, 71-2 passim; quiz 74. PMID: 20333856
Treatment of resistant hypertension.
Czarina Acelajado M, Calhoun DA
Minerva Cardioangiol 2009 Dec;57(6):787-812. PMID: 19942848

Prognosis

Prediction of metastatic pheochromocytoma and paraganglioma: a machine learning modelling study using data from a cross-sectional cohort.
Pamporaki C, Berends AMA, Filippatos A, Prodanov T, Meuter L, Prejbisz A, Beuschlein F, Fassnacht M, Timmers HJLM, Nölting S, Abhyankar K, Constantinescu G, Kunath C, de Haas RJ, Wang K, Remde H, Bornstein SR, Januszewicz A, Robledo M, Lenders JWM, Kerstens MN, Pacak K, Eisenhofer G
Lancet Digit Health 2023 Sep;5(9):e551-e559. Epub 2023 Jul 18 doi: 10.1016/S2589-7500(23)00094-8. PMID: 37474439Free PMC Article
Local-Regional Recurrence of Pheochromocytoma/Paraganglioma: Characteristics, Risk Factors and Outcomes.
Cui Y, Ma X, Gao Y, Chang X, Chen S, Lu L, Tong A
Front Endocrinol (Lausanne) 2021;12:762548. Epub 2021 Oct 19 doi: 10.3389/fendo.2021.762548. PMID: 34899602Free 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
Tumour risks and genotype-phenotype correlations associated with germline variants in succinate dehydrogenase subunit genes SDHB, SDHC and SDHD.
Andrews KA, Ascher DB, Pires DEV, Barnes DR, Vialard L, Casey RT, Bradshaw N, Adlard J, Aylwin S, Brennan P, Brewer C, Cole T, Cook JA, Davidson R, Donaldson A, Fryer A, Greenhalgh L, Hodgson SV, Irving R, Lalloo F, McConachie M, McConnell VPM, Morrison PJ, Murday V, Park SM, Simpson HL, Snape K, Stewart S, Tomkins SE, Wallis Y, Izatt L, Goudie D, Lindsay RS, Perry CG, Woodward ER, Antoniou AC, Maher ER
J Med Genet 2018 Jun;55(6):384-394. Epub 2018 Jan 31 doi: 10.1136/jmedgenet-2017-105127. PMID: 29386252Free PMC Article
Clinical Characterization of the Pheochromocytoma and Paraganglioma Susceptibility Genes SDHA, TMEM127, MAX, and SDHAF2 for Gene-Informed Prevention.
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

Clinical prediction guides

Prediction of metastatic pheochromocytoma and paraganglioma: a machine learning modelling study using data from a cross-sectional cohort.
Pamporaki C, Berends AMA, Filippatos A, Prodanov T, Meuter L, Prejbisz A, Beuschlein F, Fassnacht M, Timmers HJLM, Nölting S, Abhyankar K, Constantinescu G, Kunath C, de Haas RJ, Wang K, Remde H, Bornstein SR, Januszewicz A, Robledo M, Lenders JWM, Kerstens MN, Pacak K, Eisenhofer G
Lancet Digit Health 2023 Sep;5(9):e551-e559. Epub 2023 Jul 18 doi: 10.1016/S2589-7500(23)00094-8. PMID: 37474439Free PMC Article
Local-Regional Recurrence of Pheochromocytoma/Paraganglioma: Characteristics, Risk Factors and Outcomes.
Cui Y, Ma X, Gao Y, Chang X, Chen S, Lu L, Tong A
Front Endocrinol (Lausanne) 2021;12:762548. Epub 2021 Oct 19 doi: 10.3389/fendo.2021.762548. PMID: 34899602Free 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
Tumour risks and genotype-phenotype correlations associated with germline variants in succinate dehydrogenase subunit genes SDHB, SDHC and SDHD.
Andrews KA, Ascher DB, Pires DEV, Barnes DR, Vialard L, Casey RT, Bradshaw N, Adlard J, Aylwin S, Brennan P, Brewer C, Cole T, Cook JA, Davidson R, Donaldson A, Fryer A, Greenhalgh L, Hodgson SV, Irving R, Lalloo F, McConachie M, McConnell VPM, Morrison PJ, Murday V, Park SM, Simpson HL, Snape K, Stewart S, Tomkins SE, Wallis Y, Izatt L, Goudie D, Lindsay RS, Perry CG, Woodward ER, Antoniou AC, Maher ER
J Med Genet 2018 Jun;55(6):384-394. Epub 2018 Jan 31 doi: 10.1136/jmedgenet-2017-105127. PMID: 29386252Free PMC Article
Clinical Characterization of the Pheochromocytoma and Paraganglioma Susceptibility Genes SDHA, TMEM127, MAX, and SDHAF2 for Gene-Informed Prevention.
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

Diagnosis, Management, and Follow-Up of the Incidentally Discovered Adrenal Mass: CUA Guideline Endorsed by the AUA.
Rowe NE, Kumar R, Schieda N, Siddiqi F, McGregor T, McAlpine K, Violette P, Bathini V, Eng M, Izard J
J Urol 2023 Oct;210(4):590-599. Epub 2023 Aug 9 doi: 10.1097/JU.0000000000003644. PMID: 37556768
The association of genitourinary cancer among Saudi patients with gastrointestinal stromal tumors and a systematic literature review.
Seyam R, Chaer KA, Abouelkhair A, Almouh A, Alzahrani O, Sayed AG, Alghafees M, Alhamidi RA, Al-Gonaim A, Al-Hussain T, Amin TM, Altaweel W
Sci Rep 2023 Mar 15;13(1):4325. doi: 10.1038/s41598-023-28060-x. PMID: 36922517Free PMC Article
Surgical therapy of adrenal tumors: guidelines from the German Association of Endocrine Surgeons (CAEK).
Lorenz K, Langer P, Niederle B, Alesina P, Holzer K, Nies C, Musholt T, Goretzki PE, Rayes N, Quinkler M, Waldmann J, Simon D, Trupka A, Ladurner R, Hallfeldt K, Zielke A, Saeger D, Pöppel T, Kukuk G, Hötker A, Schabram P, Schopf S, Dotzenrath C, Riss P, Steinmüller T, Kopp I, Vorländer C, Walz MK, Bartsch DK
Langenbecks Arch Surg 2019 Jun;404(4):385-401. Epub 2019 Apr 1 doi: 10.1007/s00423-019-01768-z. PMID: 30937523
Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: A nationwide study and systematic review.
Berends AMA, Buitenwerf E, de Krijger RR, Veeger NJGM, van der Horst-Schrivers ANA, Links TP, Kerstens MN
Eur J Intern Med 2018 May;51:68-73. Epub 2018 Feb 1 doi: 10.1016/j.ejim.2018.01.015. PMID: 29361475
European Society of Endocrinology Clinical Practice Guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma.
Plouin PF, Amar L, Dekkers OM, Fassnacht M, Gimenez-Roqueplo AP, Lenders JW, Lussey-Lepoutre C, Steichen O; Guideline Working Group
Eur J Endocrinol 2016 May;174(5):G1-G10. doi: 10.1530/EJE-16-0033. PMID: 27048283

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