Pubertal outcome in a female with virilizing adrenocortical carcinoma

J Pediatr Endocrinol Metab. 2016 Apr;29(4):503-9. doi: 10.1515/jpem-2015-0123.

Abstract

Adrenocortical tumors are neoplasms that rarely occur in pediatric patients. Adrenocortical carcinoma (ACC) is even more uncommon, and is an aggressive malignancy with 5-year survival of 55% in a registry series. There is a lack of information on long-term endocrine outcome in survivors. We describe a 10-year follow-up in a patient who presented at 3 years 5 months with a 1-year history of axillary odor and 6 months' history of pubic hair development with an increased clitoral size. Androgen levels were increased and a pelvic sonogram revealed a suprarenal mass of the left kidney. The tumor was successfully removed. At 6 years 11 months, androgen levels increased again. Workup for tumor recurrence was negative and the findings likely represented early adrenarche. The patient had menarche at an appropriate time and attained a height appropriate for her family.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural

MeSH terms

  • Adrenal Cortex Neoplasms / pathology*
  • Adrenal Cortex Neoplasms / surgery
  • Adrenalectomy
  • Adrenocortical Carcinoma / pathology*
  • Adrenocortical Carcinoma / surgery
  • Child, Preschool
  • Female
  • Humans
  • Menarche
  • Prognosis
  • Puberty / physiology*
  • Virilism / pathology*
  • Virilism / surgery