Original Article

Feminizing Adrenocortical Tumors as a Rare Etiology of Iso/Contrasexual Pseudopuberty

10.4274/jcrpe.galenos.2021.2021.0170

  • Dogus Vuralli
  • Nazli Gonc
  • Alev Ozon
  • Saniye Ekinci
  • H. Serkan Dogan
  • Serdar Tekgul
  • Ayfer Alikasifoglu

Received Date: 10.06.2021 Accepted Date: 10.08.2021 J Clin Res Pediatr Endocrinol 0;0(0):0-0 [e-Pub] PMID: 34380293

Objectives:

Estrogen secreting adrenocortical tumors (ACTs) are quite rare, feminizing adrenocortical tumors (FATs) accounting for 0.37–2% of all ACTs. Present study aims to evaluate clinical and hormonal characteristics as well as treatment options and follow-up of FATs in pediatric age group.

Methods:

We reviewed medical records of 25 children with ACTs in our unit in last two decades. We reported two new pediatric cases of FAT, one benign and the other malignant, in two genders with different clinical presentations. Literature review revealed 34 pediatric patients (22boys) with FAT among 192 articles identified in pubmed database.

Results:

FATs are extremely rare tumors that are most commonly seen in men and boys presenting with gynecomastia. FATs are more common in children ≤8 years of age, with a median age at diagnosis of 6 years. While boys present with contrasexual pseudopuberty signs, girls present with isosexual pseudopuberty. High estrogen level strongly supports diagnosis, while elevations in other adrenal hormones may be seen. FATs are usually malignant in adults whereas in children approximately half are benign. Assessment of malignant potential depends on clinical behavior of tumor in children. Although complete surgical resection of benign FATs is thought to be curative, long-term follow-up is required because of unpredictability of these tumors. Prognosis is generally very poor in adult males. FATs in childhood may carry a better prognosis with most of FATs in children are followed without recurrence of tumor.

Conclusions:

FATs are more common in children ≤8 years of age, with a median age at diagnosis of 6 years. FATs in childhood period may carry a better prognosis than in adult males.

Keywords: Adrenal adenoma,adrenal tumor,adrenocortical carcinoma,adrenocortical tumors