Feminizing adrenocortical oncocytoma presenting as precocious puberty: a case report and literature review

J Pediatr Endocrinol Metab. 2022 Mar 3;35(6):827-830. doi: 10.1515/jpem-2021-0707. Print 2022 Jun 27.

Abstract

Objectives: Feminizing adrenal tumors are rare in childhood. We present a case of a special category of adrenal tumor, an oncocytoma, causing isosexual peripheral precocity.

Case presentation: A 4-year old girl presented with breast development and menstrual bleeding over a period of 3-4 months. Her SMR staging was breast stage 4, pubic hair stage 3. Her bone age was advanced (6 year 10 months), stimulated LH 0.7 IU/L, estradiol 206 pmol/L and DHEAS >27.1 micromol/L. CT scan revealed a right adrenal mass with features of atypical adrenal adenoma. Laparoscopic adrenalectomy was done and histopathology revealed oncocytoma. Lin-Weiss-Bisceglia criteria classified it as likely benign, borne out till a 2 year follow up.

Conclusions: Adrenal oncocytoma can be a cause of isosexual peripheral precocity in a young girl. Recognition and correct classification of this histological variant, which is more often benign, is important for prognostication and choice of therapy after surgery.

Keywords: adrenal cortical neoplasm; child; feminizing tumor; pediatric; peripheral precocity.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adenoma* / complications
  • Adenoma, Oxyphilic* / complications
  • Adenoma, Oxyphilic* / diagnosis
  • Adenoma, Oxyphilic* / surgery
  • Adrenal Cortex Neoplasms* / complications
  • Adrenal Cortex Neoplasms* / diagnosis
  • Adrenal Cortex Neoplasms* / surgery
  • Adrenal Gland Neoplasms* / complications
  • Adrenal Gland Neoplasms* / diagnosis
  • Adrenal Gland Neoplasms* / surgery
  • Adrenalectomy
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Puberty, Precocious* / etiology
  • Puberty, Precocious* / pathology