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Related Experiment Videos

Adrenocortical neoplasms in children: radiologic-pathologic correlation.

G A Agrons1, G J Lonergan, G E Dickey

  • 1Department of Radiology, Pennsylvania Hospital, Philadelphia 19107, USA.

Radiographics : a Review Publication of the Radiological Society of North America, Inc
|August 28, 1999
PubMed
Summary

Pediatric adrenocortical neoplasms are rare tumors affecting young children, often presenting with endocrine abnormalities. Early diagnosis and surgical intervention, especially in younger children, improve outcomes for these adrenal cortex tumors.

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Area of Science:

  • Pediatric oncology
  • Endocrinology
  • Surgical pathology

Background:

  • Primary neoplasms of the adrenal cortex are uncommon in children, differing from adult presentations.
  • Adrenocortical neoplasms in children encompass both adenomas and carcinomas, often indistinguishable histopathologically.
  • These neoplasms typically affect girls under 5 years and are linked to genetic syndromes like Beckwith-Wiedemann.

Purpose of the Study:

  • To summarize the epidemiology, clinical presentation, diagnostic imaging, and management of pediatric adrenocortical neoplasms.
  • To highlight the unique features of these tumors in children compared to adults.
  • To discuss prognostic factors influencing patient outcomes.

Main Methods:

  • Review of clinical data, imaging findings, and pathological characteristics of pediatric adrenocortical neoplasms.

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  • Analysis of epidemiological trends, associated syndromes, and clinical manifestations.
  • Evaluation of treatment strategies including surgery and chemotherapy.
  • Main Results:

    • Pediatric adrenocortical neoplasms often present with endocrine dysfunction (virilization, Cushing syndrome).
    • Imaging reveals large, heterogeneous suprarenal masses, sometimes with calcification, local invasion, or metastases.
    • Increased retroperitoneal fat on CT/MRI suggests adrenocortical neoplasm in children with adrenal masses.

    Conclusions:

    • Surgical resection is the primary treatment for pediatric adrenocortical neoplasms.
    • Chemotherapy is indicated for metastatic disease or persistent hormonal abnormalities post-surgery.
    • Younger children (<5 years) with aggressive adrenocortical neoplasms demonstrate better prognoses.