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Rare adrenal tumors in children.

Abstract
Apart from neuroblastomas, adrenal tumors are exceedingly rare in children and young adults. In this age group, the vast majority of patients present with clinical signs associated with excess hormone production. The most common tumor to arise from the adrenal cortex is an adrenocortical carcinoma (ACC). Similar to the situation in adults, this tumor is frequently diagnosed at a late stage and carries a very poor prognosis. ACCs require extensive/aggressive local resection followed by mitotane chemotherapy. A multidisciplinary approach is essential, and these children should be referred to units that have previous experience in managing ACCs. International registries are an invaluable source for evidence-based care, and such collaborations should be further developed in the future. Pheochromocytomas are derived from the adrenal medulla and present with symptoms caused by high secretion of catecholamines. At least one-third of these children will be found to carry genetic mutations, most commonly the RET gene (MEN2 syndrome) or the VHL gene. Open radical adrenalectomy should be offered to children with adrenocortical cancers. For all other cases, laparoscopic adrenalectomy is the treatment of choice. It is possible that the retroperitoneoscopic approach will gain increasing favor. The role of robotic adrenalectomy remains controversial.
AuthorsRadu Mihai
JournalSeminars in pediatric surgery (Semin Pediatr Surg) Vol. 23 Issue 2 Pg. 71-5 (Apr 2014) ISSN: 1532-9453 [Electronic] United States
PMID24931351 (Publication Type: Journal Article, Review)
CopyrightCopyright © 2014 Elsevier Inc. All rights reserved.
Chemical References
  • Antineoplastic Agents, Hormonal
  • Mitotane
Topics
  • Adrenal Cortex Neoplasms (diagnosis, drug therapy, surgery)
  • Adrenal Gland Neoplasms (diagnosis, drug therapy, surgery)
  • Adrenal Hyperplasia, Congenital (diagnosis, surgery)
  • Adrenalectomy (methods)
  • Adrenocortical Carcinoma (diagnosis, drug therapy, surgery)
  • Antineoplastic Agents, Hormonal (therapeutic use)
  • Chemotherapy, Adjuvant
  • Child
  • Humans
  • Hyperaldosteronism (diagnosis, surgery)
  • Mitotane (therapeutic use)
  • Pheochromocytoma (diagnosis, drug therapy, surgery)
  • Prognosis
  • Treatment Outcome

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