Journal
PEDIATRIC AND DEVELOPMENTAL PATHOLOGY
Volume 12, Issue 4, Pages 284-291Publisher
ALLIANCE COMMUNICATIONS GROUP DIVISION ALLEN PRESS
DOI: 10.2350/08-06-0489.1
Keywords
adrenal gland; adrenocortical adenoma; adrenocortical carcinoma; Beckwith-Wiedemann syndrome; Li-Fraumeni syndrome; p53
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Adrenal cortical neoplasms in children are represented by a disproportionate number of cases that have been diagnosed pathologically as adrenocortical carcinomas (ACCs)-as many as 90% of all cortical tumors in some pediatric series. Like other solid malignancies of childhood, over half of ACCs present in the first 4 years of life in over 50% of cases. Most are sporadically occurring neoplasms, but ACCs are a manifestation of Beckwith-Wiedemann and Li-Fraumeni syndromes. Despite the fact that the microscopic features are often quite atypical and identical in many respects to ACCs in adults, the clinical outcome is favorable in 70% or more of cases. Tumor weight is seemingly a significant determinant in prognosis at a threshold of greater than 400 g. A fisk assessment system is proposed that incorporates tumor weight, localization of tumor to the gland without invasion into the surrounding tissues or organs, and absence of metastasis.
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