Qap (Anatomical Pathology) 

Case: AP244
Contributor's Comment: Comment: assess the family history of cancer and check for germline p53 mutation if indicated.
The distinction between adrenocortical adenoma and carcinoma is difficult and may even be more problematic in pediatric adrenocortical neoplasm. Based on conventional criteria, the index case is more consistent with adrenocortical carcinoma. More significantly, when one encounter an pediatric adrenocortical carcinoma, especially for those less than 4 years old, there is a high chance of germline p53 mutation. The prevalence of the germline p53 mutation is estimated ~ 50% and may or may not be related to the classical Li-Fraumeni Syndrome. Thus family history and investigate for the germline p53 mutation have important counseling and management implications. The current index case only has a weak family history of cancer and germline p53 gene mutation is confirmed.