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Functioning adrenocortical neoplasms in children.

K K Patil1, P G Ransley, M McCullagh

  • 1Great Ormond Street Hospital, University Hospital Lewisham, UK. kalpanakp@aol.com

BJU International
|April 12, 2002
PubMed
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Functioning adrenocortical tumours in children are rare and often benign, but malignant forms have a poor prognosis. Tumour size is not a reliable indicator of malignancy in pediatric adrenal neoplasms.

Area of Science:

  • Pediatric Endocrinology
  • Pediatric Oncology
  • Surgical Oncology

Background:

  • Functioning adrenocortical tumours (FATs) in children are rare endocrine neoplasms.
  • These tumors can present with various clinical manifestations, including virilization, Cushingoid features, precocious puberty, and hypertension.
  • Distinguishing between benign and malignant adrenocortical lesions in pediatric patients is crucial for appropriate management.

Purpose of the Study:

  • To characterize the clinical presentation, treatment strategies, and outcomes of children with functioning adrenocortical tumours.
  • To evaluate the utility of tumour size as a predictive marker for malignant potential in pediatric adrenocortical neoplasms.

Main Methods:

  • A retrospective review of 21 children diagnosed with functioning adrenal masses between 1972 and 2000.

Related Experiment Videos

  • Data collection included clinicopathological features, diagnostic imaging modalities (intravenous pyelography, X-ray, ultrasonography, CT, MRI), treatment interventions, and patient outcomes.
  • Analysis focused on presenting symptoms, tumour characteristics, and treatment response.

    • Main Results:

    • The study identified 21 pediatric patients with functioning adrenal masses, predominantly unilateral and more common in girls, with a peak incidence in the first decade.
    • Common presenting symptoms included virilization (18 children), Cushingoid features (3), precocious puberty (3), and hypertension (4).
    • Of the three malignant neoplasms, one presented with metastasis and had a poor outcome despite aggressive treatment; the other two had varied prognoses. Eighteen benign neoplasms showed favorable outcomes with surgical extirpation.

    Conclusions:

    • Functioning adrenocortical neoplasms in children are rare, with most being benign and curable by surgery.
    • Malignant adrenocortical tumours in children carry a poor prognosis, particularly with incomplete surgical resection.
    • While an association exists between tumour size and malignancy, it is not a definitive individual predictor.