Table 1.

Clinical operational diagnostic criteria for CS based on International Cowden Consortium, which is same as National Comprehensive Cancer Network 2006.

Pathognomonic criteria	Adult Lhermitte-Duclos disease (LDD) (cerebellar tumors) Mucocutaneous lesions Trichilemmomas, facial Acral keratoses Papillomatous papules Mucosal lesions
Major criteria	Breast cancer Thyroid cancer (nonmedullary) Macrocephaly (megalocephaly) (i.e. 97th percentile and above) Endometrial cancer
Minor criteria	Other thyroid lesions (e.g. adenoma, multinodular goiter) Mental retardation (i.e. IQ of 75 and below) Gastrointestinal hamartomas Fibrocystic disease of the breast Lipomas Fibromas Genitourinary tumors (especially renal cell carcinoma) Genitourinary malformations Uterine fibroids
Operational diagnosis in an individual (any of the following)	Mucocutaneous lesions alone if there are: Six or more facial papules of which three must be trichilemmomas, or Cutaneous facial papules and oral mucosal papillomatosis, or Oral mucosal papillomatosis and acral keratoses, or Six or more palmoplantar keratoses, or Two or more major criteria of which one must be macrocephaly or LDD, or One major and at least three minor criteria, or Four or more minor criteria
Operational diagnosis in a family where one individual is diagnostic for Cowden Syndrome	Any one pathognomonic criterion Any one major criteria with or without minor criteria Two minor criteria History of Bannayan-Riley-Ruvalcaba syndrome
Cowden-Like Syndrome	Individuals meeting above criteria minus one criterion