Pre-Gardner's syndrome, thyroglossal cysts and undifferentiated tumor of neural crest origin.

A child with a malignant undifferentiated adrenal medullary tumor of neural crest origin, removed at six months of age, was treated with chemotherapy and is alive without evidence of cancer after seven years. He developed osteomas of the skull, a thyroglossal cyst and has had one episode of gastrointestinal bleeding. A three-generation history of multiple colonic polyposis and colon cancer, osteomas, recurrent pancreatitis, and Wilms' tumor was elicited. One of three females in this family, with direct lineage from polyposis afflicted fathers, exhibited Gardner's Syndrome and Wilms' tumor. The constellation of tumors afflicting this family may represent an underlying defect of excessive cell proliferation affecting mesenchymal and neuroblastic tissue. These may be extracolonic conditions associated with Gardner's Syndrome.