Cushing's syndrome due to a non-adrenal ectopic adrenocorticotropin-secreting Ewing's sarcoma in a child

J Pediatr Endocrinol Metab. 2009 Apr;22(4):363-8. doi: 10.1515/jpem.2009.22.4.363.

Abstract

Ectopic ACTH syndrome (EAS) is extremely rare in the pediatric age group. Sarcomatous tumors causing EAS are even rarer. We report a 9 year-old girl presenting with Cushing's syndrome caused by ectopic ACTH production by a Ewing's sarcoma. This case illustrates that rapid appearance of Cushingoid symptoms, absence of growth retardation and the presence of hypokalemia are suggestive clues for ectopic ACTH production as the source of Cushing's syndrome.

Publication types

  • Case Reports

MeSH terms

  • ACTH Syndrome, Ectopic / complications*
  • Adrenocorticotropic Hormone / metabolism*
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Child
  • Cushing Syndrome / drug therapy
  • Cushing Syndrome / etiology*
  • Cyclophosphamide / therapeutic use
  • Doxorubicin / therapeutic use
  • Female
  • Humans
  • Retroperitoneal Neoplasms / complications
  • Retroperitoneal Neoplasms / drug therapy
  • Sarcoma, Ewing / complications*
  • Sarcoma, Ewing / drug therapy
  • Vincristine / therapeutic use

Substances

  • Vincristine
  • Doxorubicin
  • Cyclophosphamide
  • Adrenocorticotropic Hormone

Supplementary concepts

  • CAV protocol