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Nonfunctioning adrenocortical carcinoma in a child.

Turan Kanmaz1, Savas Demirbilek, Ilyas Ozardali

  • 1Department of Pediatric Surgery, Harran University, Faculty of Medicine, Sanliurfa, Turkey. kanmaz@surgery.wisc.edu

Pediatric Pathology & Molecular Medicine
|December 25, 2003
PubMed
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A rare pediatric nonfunctioning adrenocortical carcinoma presented as an abdominal mass in a 4-year-old girl. Despite surgical removal, the patient succumbed to chemotherapy complications, highlighting challenges in treating this rare pediatric cancer.

Area of Science:

  • Pediatric Oncology
  • Surgical Pathology

Background:

  • Adrenocortical carcinoma (ACC) is a rare malignancy in children.
  • Nonfunctioning ACC, lacking hormonal symptoms, presents diagnostic challenges.
  • Germline p53 mutations are associated with a higher incidence of ACC.

Observation:

  • A 4-year-old girl presented with an abdominal mass and pain, with no hormonal abnormalities.
  • Imaging revealed a polylobular abdominal mass compressing the kidney.
  • Histopathology confirmed adrenocortical carcinoma.

Findings:

  • The tumor was surgically resected along with the right kidney.
  • Genetic analysis revealed no mutation in the tumor suppressor gene p53.
  • The patient died two months post-surgery due to chemotherapy complications.

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Implications:

  • This case underscores the rarity and aggressive nature of pediatric nonfunctioning adrenocortical carcinoma.
  • It highlights the importance of prompt diagnosis and surgical intervention.
  • The lack of p53 mutation in this case warrants further research into the genetic landscape of pediatric ACC.