TableĀ 3.

Consensus Recommendations for Genetic Counseling and Testing for Cowden Syndrome26

Major CriteriaMinor Criteria
Breast cancer Endometrial cancer Follicular thyroid cancer Multiple gastrointestinal hamartomas or ganglioneuromas Macrocephaly Mucocutaneous lesions Multiple trichilemmomas Multiple acral/palmoplantar keratosis, pits, papules Mucocutaneous neuromas Multifocal oral mucosal papillomatosis Multiple cutaneous facial papules (verrucous)Autism spectrum disorder Colon cancer Esophageal glycogenic acanthoses Lipomas Intellectual disability Thyroid adenomas, goiter, nodules Papillary or follicular thyroid caner Renal cell carcinoma Single gastrointestinal hamartoma or ganglioneuroma Vascular anomalies