Multiple hamartoma and neoplasia syndrome (Cowden's syndrome)

A case report involving a patient with Cowden's syndrome is presented. The patient's family is shown to be affected in a pattern consistent with an autosomal dominant form of transmission of this inherited disorder. The disorder can be diagnosed in its early stages by gingival and cutaneous manifestations; it is emphasized that the dentist is therefore perhaps the best-qualified health care professional to recognize the disease in its early stages and to alert the patient to be monitored for later predictable neoplasia which can perhaps be treated before metastasis develops.