Case Report
Copyright ©2008 The WJG Press and Baishideng.
World J Gastroenterol. Oct 7, 2008; 14(37): 5755-5759
Published online Oct 7, 2008. doi: 10.3748/wjg.14.5755
Table 2 Criteria of international Cowden Consortium (Verion 2000) for Cowden's disease
Pathognomonic criteria
Mucocutaneous lesions
Trichilemmomas, facial
Acral keratoses
Papillomatous papules
Mucosal lesions
Major criteria
Breast carcinoma
Thyroid carcinoma (non-medullary), especially follicular thyroid carcinoma
Macrocephaly (megalencephaly) (say, 95th centile)
Lhermitte-Duclos disease (LDD)
Endometrial carcinoma
Minor criteria
Other thyroid lesions (eg, adenoma or multinodular goitre)
Mental retardation (say, IQ 75)
GI hamartomas
Fibrocystic disease of the breast
Lipomas
Fibromas
GU tumours (e.g. renal cell carcinoma, uterine fibroids) or malformation
Operational diagnosis in a person
Mucocutaneous lesions alone if:
There are 6 or more facial papules, of which 3 or more must be trichilemmoma, or
Cutaneous facial papules and oral mucosal papillomatosis, or
Oral mucosal papillomatosis and acral keratoses, or
Palmoplantar keratoses, 6 or more
2 major criteria but one must include macrocephaly or LDD
1 major and 3 minor criteria
4 minor criteria
Operational diagnosis in a family where one person is diagnostic for Cowden syndrome
The pathognomonic criterion/ia
Any one major criterion with or without minor criteria
Two minor criteria