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Adrenal Tumors

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Pediatric Urology

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Abstract

Adrenal tumors are rare in childhood. Peripheral neuroblastic tumors are classified according to the prevalence of neural-type cells and Schwann-type cells; they show different degrees of differentiation and a broad spectrum of clinical phenotype, from benign to extremely aggressive. Among them neuroblastoma is the most frequent malignancy diagnosed in the first month of life (30–50 %), and it is the most common cancer among infants of less than 12 months of age. The adrenocortical tumors (ACTs) include both adrenocortical carcinoma and adenoma, in most cases hormonally active, causing virilization, hirsutism, and Cushing’s syndrome. Diagnosis and stadiation of these cancers are made by imaging studies and biochemical endocrine and histological evaluation. Treatment options include surgery, with preventive pharmacological control of hypertensive crises in patients with pheochromocytoma, and chemotherapy in selected cases as ACTs or inoperable masses. Long-term follow-up of patients with malignant cancers is mandatory.

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References

  1. Moore KL, Dalley AF (1999) Clinically oriented anatomy. Lippincott, Baltimore, p 279

    Google Scholar 

  2. Perrier ND, Boger MS (2005) Adrenal glands: diagnostic aspects and surgical therapy. In: Linos D, van Heerden JA (eds) Surgical anatomy. Springer, New York, p 7

    Google Scholar 

  3. Brunt LM, Halverson JD (2002) The endocrine system. In: O’Leary JP (ed) The physiologic basis of surgery. Lippincott, Philadelphia, p 351

    Google Scholar 

  4. Brodeur GM, Hogarty MD, Mosse YP et al (2011) Neuroblastoma. In: Pizzo PA, Poplack DG (eds) Principles and practice of pediatric oncology. Lippincott Williams & Wilkins, Philadelphia, p 886

    Google Scholar 

  5. Lonergan GJ, Schwab CM, Suarez ES et al (2002) Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation. Radiographics 22:911–934

    Article  PubMed  Google Scholar 

  6. Lenders JW, Eisenhofer G, Mannelli M et al (2005) Phaeochromocytoma. Lancet 366:665–675

    Article  PubMed  Google Scholar 

  7. Bhansali A, Rajput R, Behra A et al (2006) Childhood sporadic pheochromocytoma: clinical profile and outcome in 19 patients. J Pediatr Endocrinol Metab 19:749–756

    Article  CAS  PubMed  Google Scholar 

  8. British Society of Paediatric Endocrinology & Diabetes (2005) Paediatric endocrine tumours. A multidisciplinary consensus statement of best practice from a Working Group Convened Under the Auspices of the BSPED and UKCCSG. http://www.bsped.org.uk/clinical/docs/RareEndocrineTumour_final.pdf. Accessed 14 Sept 2013

  9. Agrons GA, Lonergan GJ, Dickey GE et al (1999) Adrenocortical neoplasms in children: radiologic-pathologic correlation. Radiographics 19:989–1008

    Article  CAS  PubMed  Google Scholar 

  10. Bonfig W, Bittmann I, Bechtold S et al (2003) Virilising adrenocortical tumours in children. Eur J Pediatr 162:623–628

    Article  PubMed  Google Scholar 

  11. Figueiredo BC, Sandrini R, Zambetti GP et al (2006) Penetrance of adrenocortical tumours associated with the germline TP53 R337H mutation. J Med Genet 43:91–96

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  12. Chow EJ, Friedman DL, Mueller BA (2007) Maternal and perinatal characteristics in relation to neuroblastoma. Cancer 109:983–992

    Article  PubMed  Google Scholar 

  13. French AE, Grant R, Weitzman S et al (2003) Folic acid food fortification is associated with a decline in neuroblastoma. Clin Pharmacol Ther 74:288–294

    Article  CAS  PubMed  Google Scholar 

  14. Cook MN, Olshan AF, Guess HA et al (2004) Maternal medication use and neuroblastoma in offspring. Am J Epidemiol 15:721–731

    Article  Google Scholar 

  15. Blatt J1, Olshan AF, Lee PA, Ross JL (1997) Neuroblastoma and related tumors in Turner’s syndrome. J Pediatr 131(5):666–70

    Google Scholar 

  16. Maris JM, Weiss MJ, Mosse Y et al (2002) Evidence for a hereditary neuroblastoma predisposition locus at chromosome 16p12-13. Cancer Res 22:6651–6658

    Google Scholar 

  17. Mossé YP, Laudenslager M, Longo L et al (2008) Identification of ALK as a major familial neuroblastoma predisposition gene. Nature 455:930–935

    Article  PubMed Central  PubMed  Google Scholar 

  18. Trochet D, Bourdeaut F, Janoueix-Lerosey I et al (2004) Germline mutations of the paired-like homeobox 2B (PHOX2B) gene in neuroblastoma. Am J Hum Genet 74:761–764

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  19. Berry T, Luther W, Bhatnagar N et al (2012) The ALK(F1174L) mutation potentiates the oncogenic activity of MYCN in neuroblastoma. Cancer Cell 22:117–130

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  20. Cohn SL, Tweddle DA (2004) MYCN amplification remains prognostically strong 20 years after its “clinical debut”. Eur J Cancer 40:2639–2642

    Article  CAS  PubMed  Google Scholar 

  21. Ize-Ludlow D, Gray JA, Sperling MA et al (2007) Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation presenting in childhood. Pediatrics 120:179–188

    Article  Google Scholar 

  22. Waguespack SG, Rich T, Grubbs E et al (2010) A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma. J Clin Endocrinol Metab 95:2023–2037

    Article  CAS  PubMed  Google Scholar 

  23. Hoyme HE, Seaver LH, Jones KL et al (1998) Isolated hemihyperplasia (hemihypertrophy): report of a prospective multicenter study of the incidence of neoplasia and review. Am J Med Genet 79:274–278

    Article  CAS  PubMed  Google Scholar 

  24. El Wakil A, Doghman M, Latre De Late P et al (2011) Genetics and genomics of childhood adrenocortical tumors. Mol Cell Endocrinol 336:169–173

    Article  PubMed  Google Scholar 

  25. De Bernardi B, Gambini C, Haupt R et al (2008) Retrospective study of childhood ganglioneuroma. J Clin Oncol 1:1710–1716

    Article  Google Scholar 

  26. Cohn SL, Pearson AD, London WB et al (2009) The International Neuroblastoma Risk Group (INRG) classification system: an INRG Task Force report. J Clin Oncol 27:289–297

    Article  PubMed Central  PubMed  Google Scholar 

  27. Maris JM (2010) Recent advances in neuroblastoma. N Engl J Med 362:2202–2211

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  28. Zage PE, Ater JL (2012) Neuroblastoma. In: Stanton B, St.Geme J, Schor N, Behrman RE, Kliegman RM (eds) Nelson textbook of pediatrics, 19th edn. Elsevier, Philadelphia, p 492

    Google Scholar 

  29. Quinn JJ, Altman AJ (1979) The multiple hematologic manifestations of neuroblastoma. Am J Pediatr Hematol Oncol 1:201–205

    Article  CAS  PubMed  Google Scholar 

  30. Geoerger B, Hero B, Harms D et al (2001) Metabolic activity and clinical features of primary ganglioneuromas. Cancer 15:1905–1913

    Article  Google Scholar 

  31. Schwab M, Shimada H, Joshi V et al (2000) Neuroblastic tumours of adrenal gland and sympathetic nervous system. In: Kleihues P, Cavenee WK (eds) World Health Organization classifications of tumors. Pathology and genetics of tumours of the nervous system. IARC Press, Lyon, France, p 153

    Google Scholar 

  32. Okamatsu C, London WB, Naranjo A et al (2009) Clinicopathological characteristics of ganglioneuroma and ganglioneuroblastoma: a report from the CCG and COG. Pediatr Blood Cancer 53:563–569

    Article  PubMed Central  PubMed  Google Scholar 

  33. Pham TH, Moir C, Thompson GB et al (2006) Pheochromocytoma and paraganglioma in children: a review of medical and surgical management at a tertiary care center. Pediatrics 118:1109–1117

    Article  PubMed  Google Scholar 

  34. Caty MG, Coran AG, Geagen M et al (1990) Current diagnosis and treatment of pheochromocytoma in children. Experience with 22 consecutive tumors in 14 patients. Arch Surg 125:978–981

    Article  CAS  PubMed  Google Scholar 

  35. Januszewicz P, Wieteska-Klimczak A, Wyszyńska T (1990) Pheochromocytoma in children: difficulties in diagnosis and localization. Clin Exp Hypertens A 12:571–579

    Article  CAS  PubMed  Google Scholar 

  36. Pattarino F, Bouloux PM (1996) The diagnosis of malignancy in phaeochromocytoma. Clin Endocrinol (Oxf) 44:239–241

    Article  CAS  Google Scholar 

  37. Wooten MD, King DK (1993) Adrenal cortical carcinoma. Epidemiology and treatment with mitotane and a review of the literature. Cancer 72:3145–3155

    Article  CAS  PubMed  Google Scholar 

  38. Michalkiewicz E, Sandrini R, Figueiredo B et al (2004) Clinical and outcome characteristics of children with adrenocortical tumors: a report from the International Pediatric Adrenocortical Tumor Registry. J Clin Oncol 22:838–845

    Article  CAS  PubMed  Google Scholar 

  39. Weiss LM (1984) Comparative histologic study of 43 metastasizing and nonmetastasizing adrenocortical tumors. Am J Surg Pathol 8:163–169

    Article  CAS  PubMed  Google Scholar 

  40. van Slooten H, Schaberg A, Smeenk D et al (1985) Morphologic characteristics of benign and malignant adrenocortical tumors. Cancer 55:766–773

    Article  PubMed  Google Scholar 

  41. Ribeiro RC, Sandrini Neto RS, Schell MJ et al (1990) Adrenocortical carcinoma in children: a study of 40 cases. J Clin Oncol 8:67–74

    CAS  PubMed  Google Scholar 

  42. Ciftici AO, Senocak ME, Tanyel FC et al (2001) Adrenocortical tumors in children. J Pediatr Surg 36:549–554

    Article  Google Scholar 

  43. Kleis M, Daldrup-Link H, Matthay K et al (2009) Diagnostic value of PET/CT for the staging and restaging of pediatric tumors. Eur J Nucl Med Mol Imaging 36:23–36

    Article  PubMed  Google Scholar 

  44. Voute PA, Hoefnagel CA, Marcuse HR et al (1985) Detection of neuroblastoma with 131I-meta-iodobenzylguanidine. Prog Clin Biol Res 175:389–398

    CAS  PubMed  Google Scholar 

  45. Ichikawa T, Ohtomo K, Araki T et al (1996) Ganglioneuroma: computed tomography and magnetic resonance features. Br J Radiol 69:114–121

    Article  CAS  PubMed  Google Scholar 

  46. Guo YK, Yang ZG, Li Y et al (2007) Uncommon adrenal masses: CT and MRI features with histopathologic correlation. Eur J Radiol 62:359–370

    Article  PubMed  Google Scholar 

  47. Domanski H (2005) Fine-needle aspiration of ganglioneuroma. Diagn Cytopathol 32:363–366

    Article  PubMed  Google Scholar 

  48. Gardet V, Gatta B, Simonnet G et al (2001) Lessons from an unpleasant surprise: a biochemical strategy for the diagnosis of pheochromocytoma. J Hypertens 19:1029–1035

    Article  CAS  PubMed  Google Scholar 

  49. McHugh K (2007) Renal and adrenal tumours in children. Cancer Imaging 7:41–51

    Article  PubMed Central  PubMed  Google Scholar 

  50. Grumbach MM, Biller BM, Braunstein GD et al (2003) Management of the clinically inapparent adrenal mass (“incidentaloma”). Ann Intern Med 138:424–429

    Article  PubMed  Google Scholar 

  51. Masiakos PT, Gerstle JT, Cheang T et al (2004) Is surgery necessary for incidentally discovered adrenal masses in children? J Pediatr Surg 39:754–758

    Article  PubMed  Google Scholar 

  52. Perez CA, Matthay KK, Atkinson JB et al (2000) Biologic variables in the outcome of stages I and II neuroblastoma treated with surgery as primary therapy: a children’s cancer group study. J Clin Oncol 18:18–26

    CAS  PubMed  Google Scholar 

  53. Nickerson HJ, Matthay KK, Seeger RC et al (2000) Favorable biology and outcome of stage IV-S neuroblastoma with supportive care or minimal therapy: a Children’s Cancer Group study. J Clin Oncol 18:477–486

    CAS  PubMed  Google Scholar 

  54. Maris JM, Hogarty MD, Bagatell R et al (2007) Neuroblastoma. Lancet 369:2106–2120

    Article  CAS  PubMed  Google Scholar 

  55. De Bernardi B, Nicolas B, Boni L et al (2003) Disseminated neuroblastoma in children older than one year at diagnosis: comparable results with three consecutive high-dose protocols adopted by the Italian Co-Operative Group for Neuroblastoma. J Clin Oncol 21:1592–1601

    Article  PubMed  Google Scholar 

  56. Cheung NK, Kushner BH, LaQuaglia M et al (2001) N7: a novel multi-modality therapy of high risk neuroblastoma (NB) in children diagnosed over 1 year of age. Med Pediatr Oncol 36:227–230

    Article  CAS  PubMed  Google Scholar 

  57. Matthay KK, George RE, Yu AL (2012) Promising therapeutic targets in neuroblastoma. Clin Cancer Res 18:2740–2753

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  58. Bravo EL, Tarazi RC, Gifford RW et al (1979) Circulating and urinary catecholamines in pheochromocytoma. Diagnostic and pathophysiologic implications. N Engl J Med 301:682–686

    Article  CAS  PubMed  Google Scholar 

  59. DeLellis RA, Lloyd RV, Heitz PU et al (2004) World health organization classification of tumours, pathology and genetics of tumours of endocrine organs. IARC Press, Lyon

    Google Scholar 

  60. Edmonds S, Fein DM, Gurtman A (2011) Pheochromocytoma. Pediatr Rev 32:308–310

    Article  PubMed  Google Scholar 

  61. Allolio B, Fassnacht M (2006) Adrenocortical carcinoma: clinical update. J Clin Endocrinol Metab 91:2027–2037

    Article  CAS  PubMed  Google Scholar 

  62. Rodriguez-Galindo C, Figueiredo BC, Zambetti GP et al (2005) Biology, clinical characteristics, and management of adrenocortical tumors in children. Pediatr Blood Cancer 45:265–273

    Article  PubMed  Google Scholar 

  63. Zancanella P, Pianovski MA, Oliveira BH et al (2006) Mitotane associated with cisplatin, etoposide, and doxorubicin in advanced childhood adrenocortical carcinoma: mitotane monitoring and tumor regression. J Pediatr Hematol Oncol 28:513–524

    Article  CAS  PubMed  Google Scholar 

  64. Gonzalez RJ1, Shapiro S, Sarlis N et al (2005) Laparoscopic resection of adrenal cortical carcinoma: a cautionary note. Surgery 2005;138(6):1078–85

    Google Scholar 

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Authors and Affiliations

  1. Department of Pediatrics, S. Orsola-Malpighi University Hospital, Via Massarenti 11, Bologna, 40138, Italy

    Andrea Pession & Federico Baronio

  2. Department of Pediatrics – Pediatric Hematology and Oncology “Lalla Seràgnoli”, S. Orsola-Malpighi University Hospital, Via Massarenti 11, Bologna, 40138, Italy

    Marco Togni & Arcangelo Prete

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  1. Andrea Pession
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  2. Federico Baronio
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  3. Marco Togni
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  4. Arcangelo Prete
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Correspondence to Andrea Pession .

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Editors and Affiliations

  1. Pediatric Surgery, S. Orsola-Malpighi University Hospital, Bologna, Bologna, Italy

    Mario Lima

  2. Pediatric Urology, Fondazione IRCCS Ca’ Granda – Ospedale Maggiore Policlinico, Milano, Italy

    Gianantonio Manzoni

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Pession, A., Baronio, F., Togni, M., Prete, A. (2015). Adrenal Tumors. In: Lima, M., Manzoni, G. (eds) Pediatric Urology. Springer, Milano. https://doi.org/10.1007/978-88-470-5693-0_24

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  • DOI: https://doi.org/10.1007/978-88-470-5693-0_24

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