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Adrenocortical carcinoma presenting with heterosexual pseudoprecocious puberty shortly after birth: case report and

F Ghazizadeh1, M Ebadi, S Alavi

  • 1Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti Medical University, Tehran, Iran.

Ecancermedicalscience
|February 8, 2013
PubMed

Insights

Pediatric adrenocortical carcinoma is rare but can present as pseudoprecocious puberty. Early diagnosis and treatment are crucial for favorable outcomes in children with this rare adrenal cancer.

Area of Science:

  • Pediatric Endocrinology
  • Pediatric Oncology
  • Adrenal Gland Neoplasms

Background:

  • Adrenocortical tumors are uncommon in pediatric populations.
  • Pseudoprecocious puberty, characterized by early virilization, can be a presenting sign.

Observation:

  • A case of a female infant diagnosed with adrenocortical carcinoma at age two.
  • The infant presented with signs of virilization, including pubic hair growth and hirsutism.
  • Biochemical tests revealed elevated levels of DHEA-S, 17-OH progesterone, and testosterone.

Findings:

  • Computed tomography (CT) identified a right adrenal gland mass with necrosis, displacing the kidney.
  • Surgical exploration confirmed a large, hemorrhagic, and necrotic adrenal mass with inferior vena cava thrombosis.
  • Pathological examination definitively diagnosed adrenocortical carcinoma.

Implications:

  • Adrenocortical carcinoma should be considered in the differential diagnosis of pediatric pseudoprecocious puberty.
  • Prompt diagnosis and multimodal treatment, including chemotherapy, are essential for managing pediatric adrenocortical carcinoma.
  • This case highlights the importance of thorough evaluation for rare pediatric malignancies presenting with hormonal disturbances.

Keywords:
adrenocortical carcinomapaediatricpseudoprecocious pubertyvirilisation

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