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Childhood adrenal gland pheochromocytoma

MedGen UID:
859271
•Concept ID:
C3899677
•
Neoplastic Process
Synonyms: Childhood Adrenal Gland Pheochromocytoma; childhood adrenal gland pheochromocytoma; Childhood Pheochromocytoma; malignant childhood adrenal gland pheochromocytoma; Pediatric Adrenal Gland Pheochromocytoma; Pediatric Pheochromocytoma; pheochromocytoma, childhood
 
Monarch Initiative: MONDO:0044767

Definition

A rare pheochromocytoma of the adrenal gland that occurs during childhood. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood adrenal gland pheochromocytoma

Professional guidelines

PubMed

Extra-adrenal pheochromocytoma: diagnosis and management.
Disick GI, Palese MA
Curr Urol Rep 2007 Jan;8(1):83-8. doi: 10.1007/s11934-007-0025-5. PMID: 17239321
Current diagnosis and treatment of pheochromocytoma in children. Experience with 22 consecutive tumors in 14 patients.
Caty MG, Coran AG, Geagen M, Thompson NW
Arch Surg 1990 Aug;125(8):978-81. doi: 10.1001/archsurg.1990.01410200036004. PMID: 2378563
Diagnosis, management, and pathogenetic studies in medullary thyroid carcinoma syndrome.
Samaan NA, Yang KP, Schultz P, Hickey RC
Henry Ford Hosp Med J 1989;37(3-4):132-7. PMID: 2576946

Recent clinical studies

Etiology

Endocrine morbidity in neurofibromatosis 1: a nationwide, register-based cohort study.
Kenborg L, Ebbehoj A, Ejerskov C, Handrup MM, Østergaard JR, Hove H, Doser K, Krøyer A, Mulvihill JJ, Winther JF, Stochholm K
Eur J Endocrinol 2023 Aug 2;189(2):190-198. doi: 10.1093/ejendo/lvad101. PMID: 37542520
Cancer Stem Cells in Pheochromocytoma and Paraganglioma.
Scriba LD, Bornstein SR, Santambrogio A, Mueller G, Huebner A, Hauer J, Schedl A, Wielockx B, Eisenhofer G, Andoniadou CL, Steenblock C
Front Endocrinol (Lausanne) 2020;11:79. Epub 2020 Feb 25 doi: 10.3389/fendo.2020.00079. PMID: 32158431Free PMC Article
Von Hippel-Lindau and Hereditary Pheochromocytoma/Paraganglioma Syndromes: Clinical Features, Genetics, and Surveillance Recommendations in Childhood.
Rednam SP, Erez A, Druker H, Janeway KA, Kamihara J, Kohlmann WK, Nathanson KL, States LJ, Tomlinson GE, Villani A, Voss SD, Schiffman JD, Wasserman JD
Clin Cancer Res 2017 Jun 15;23(12):e68-e75. doi: 10.1158/1078-0432.CCR-17-0547. PMID: 28620007
Neuroendocrine tumors and tumor syndromes in childhood.
Gaal J, de Krijger RR
Pediatr Dev Pathol 2010 Nov-Dec;13(6):427-41. Epub 2009 Aug 26 doi: 10.2350/09-04-0635-CP.1. PMID: 19708762
Extra-adrenal pheochromocytoma: diagnosis and management.
Disick GI, Palese MA
Curr Urol Rep 2007 Jan;8(1):83-8. doi: 10.1007/s11934-007-0025-5. PMID: 17239321

Diagnosis

Biochemical Diagnosis of Catecholamine-Producing Tumors of Childhood: Neuroblastoma, Pheochromocytoma and Paraganglioma.
Eisenhofer G, Peitzsch M, Bechmann N, Huebner A
Front Endocrinol (Lausanne) 2022;13:901760. Epub 2022 Jul 26 doi: 10.3389/fendo.2022.901760. PMID: 35957826Free PMC Article
Adrenal Tumors in Childhood.
Fuqua JS
Adv Pediatr 2021 Aug;68:227-244. doi: 10.1016/j.yapd.2021.05.015. PMID: 34243855
Von Hippel-Lindau and Hereditary Pheochromocytoma/Paraganglioma Syndromes: Clinical Features, Genetics, and Surveillance Recommendations in Childhood.
Rednam SP, Erez A, Druker H, Janeway KA, Kamihara J, Kohlmann WK, Nathanson KL, States LJ, Tomlinson GE, Villani A, Voss SD, Schiffman JD, Wasserman JD
Clin Cancer Res 2017 Jun 15;23(12):e68-e75. doi: 10.1158/1078-0432.CCR-17-0547. PMID: 28620007
Malignant adrenal tumors.
Rescorla FJ
Semin Pediatr Surg 2006 Feb;15(1):48-56. doi: 10.1053/j.sempedsurg.2005.11.008. PMID: 16458846
Pheochromocytoma.
Ram CV
Cardiol Clin 1988 Nov;6(4):517-35. PMID: 3067844

Therapy

Endocrine morbidity in neurofibromatosis 1: a nationwide, register-based cohort study.
Kenborg L, Ebbehoj A, Ejerskov C, Handrup MM, Østergaard JR, Hove H, Doser K, Krøyer A, Mulvihill JJ, Winther JF, Stochholm K
Eur J Endocrinol 2023 Aug 2;189(2):190-198. doi: 10.1093/ejendo/lvad101. PMID: 37542520
Late diagnosis of metastatic pheochromocytoma in multiple endocrine neoplasia 2B with rapid clinical decline.
Jester G, Hassanein H, El-Far A
BMJ Case Rep 2021 Feb 4;14(2) doi: 10.1136/bcr-2020-240488. PMID: 33541961Free PMC Article
Childhood sporadic pheochromocytoma: clinical profile and outcome in 19 patients.
Bhansali A, Rajput R, Behra A, Rao KL, Khandelwal N, Radotra BD
J Pediatr Endocrinol Metab 2006 May;19(5):749-56. doi: 10.1515/jpem.2006.19.5.749. PMID: 16789642
[(131)I] and [(125)I] metaiodobenzylguanidine therapy in macroscopic and microscopic tumors: a comparative study in SK-N-SH human neuroblastoma and PC12 rat pheochromocytoma xenografts.
Rutgers M, Buitenhuis CK, van der Valk MA, Hoefnagel CA, Voûte PA, Smets LA
Int J Cancer 2000 Dec 20;90(6):312-25. PMID: 11180134
Phaeochromocytoma and functioning paraganglioma in childhood and adolescence: role of iodine 131 metaiodobenzylguanidine.
Khafagi FA, Shapiro B, Fischer M, Sisson JC, Hutchinson R, Beierwaltes WH
Eur J Nucl Med 1991;18(3):191-8. doi: 10.1007/BF02262730. PMID: 1645665

Prognosis

Renal and adrenal tumours in children.
McHugh K
Cancer Imaging 2007 Mar 5;7(1):41-51. doi: 10.1102/1470-7330.2007.0007. PMID: 17339140Free PMC Article
Extra-adrenal pheochromocytoma: diagnosis and management.
Disick GI, Palese MA
Curr Urol Rep 2007 Jan;8(1):83-8. doi: 10.1007/s11934-007-0025-5. PMID: 17239321
Malignant adrenal tumors.
Rescorla FJ
Semin Pediatr Surg 2006 Feb;15(1):48-56. doi: 10.1053/j.sempedsurg.2005.11.008. PMID: 16458846
Pheochromocytoma in children.
Ciftci AO, Tanyel FC, Senocak ME, Büyükpamukçu N
J Pediatr Surg 2001 Mar;36(3):447-52. doi: 10.1053/jpsu.2001.21612. PMID: 11226993
Pheochromocytoma.
Ram CV
Cardiol Clin 1988 Nov;6(4):517-35. PMID: 3067844

Clinical prediction guides

Endocrine morbidity in neurofibromatosis 1: a nationwide, register-based cohort study.
Kenborg L, Ebbehoj A, Ejerskov C, Handrup MM, Østergaard JR, Hove H, Doser K, Krøyer A, Mulvihill JJ, Winther JF, Stochholm K
Eur J Endocrinol 2023 Aug 2;189(2):190-198. doi: 10.1093/ejendo/lvad101. PMID: 37542520
VHL-Related Neuroendocrine Neoplasms And Beyond: An Israeli Specialized Center Real-Life Report.
Szalat A, Oleinikov K, Nahmias A, Meiner V, Ben-Haim S, Atlan K, Lev-Cohain N, Appelbaum L, Gomori M, Mazeh H, Khalaileh A, Pe'er J, Lossos A, Shoshan Y, Grozinsky-Glasberg S, Gross DJ
Endocr Pract 2020 Oct;26(10):1131-1142. doi: 10.4158/EP-2020-0220. PMID: 33471715
Malignant pheochromocytoma in a 16-year-old patient with neurofibromatosis type 1.
Giovannoni I, Callea F, Boldrini R, Inserra A, Cozza R, Francalanci P
Pediatr Dev Pathol 2014 Mar-Apr;17(2):126-9. Epub 2014 Feb 20 doi: 10.2350/13-10-1397-CR.1. PMID: 24555864
Metastatic pheochromocytoma/paraganglioma related to primary tumor development in childhood or adolescence: significant link to SDHB mutations.
King KS, Prodanov T, Kantorovich V, Fojo T, Hewitt JK, Zacharin M, Wesley R, Lodish M, Raygada M, Gimenez-Roqueplo AP, McCormack S, Eisenhofer G, Milosevic D, Kebebew E, Stratakis CA, Pacak K
J Clin Oncol 2011 Nov 1;29(31):4137-42. Epub 2011 Oct 3 doi: 10.1200/JCO.2011.34.6353. PMID: 21969497Free PMC Article
Pheochromocytoma in children.
Ciftci AO, Tanyel FC, Senocak ME, Büyükpamukçu N
J Pediatr Surg 2001 Mar;36(3):447-52. doi: 10.1053/jpsu.2001.21612. PMID: 11226993

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