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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
Summary
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.
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.