Adrenocortical carcinoma in a child with specific pedigree of family associated with cancer aggregation.

We report on a 5-year-old boy with functioning adrenocortical carcinoma as a proband of a specific pedigree with several young family members who had cancer. Most of the members who died of cancer had early onset of osteosarcoma, hepatoblastoma or malignant lymphoma. The finding of cancer aggregation in the family corresponded to the criteria for the cancer family syndrome.