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World J Gastrointest Oncol. Feb 15, 2013; 5(2): 34-37
Published online Feb 15, 2013. doi: 10.4251/wjgo.v5.i2.34
Published online Feb 15, 2013. doi: 10.4251/wjgo.v5.i2.34
Pathognomonic criteria |
Lhermitte-duclos disease-adult |
Mucocutaneous lesions |
Trichilemmomas, facial |
Acral keratoses |
Papillomatous lesions |
Major criteria |
Breast cancer |
Thyroid cancer (papillary or follicular) |
Macrocephaly (≥ 97% ile) |
Endometrial cancer |
Minor criteria |
Other structural thyroid lesions (e.g., adenoma, multinodular goiter) |
Mental retardation (i.e., intelligence quotient ≤ 75) |
Gastrointestinal hamartomas |
Fibrocystic disease of the breast |
Lipomas |
Fibromas |
Genitourinary tumours (e.g., uterine fibroids, renal cell carcinoma) or |
genitourinary structural 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 or more must be trichilemmoma; or |
Cutaneous facial papules and oral mucosal papillomatosis; or |
Oral mucosal papillomatosis and acral keratoses; or |
Palmoplantar keratoses, six or more |
Two or more major criteria, but one must include macrocephaly or lhermitte-duclos disease |
One major and three minor criteria; or |
Four minor criteria |
Operational diagnosis in a family where one individual is diagnostic for Cowden |
One pathognomonic criterion |
Any one major criterion with or without minor criteria |
Two minor criteria |
History of Bannayan-Riley-Ruvalcaba syndrome |
- Citation: Herranz Bachiller MT, Barrio Andrés J, Pons F, Alcaide Suárez N, Ruiz-Zorrilla R, Sancho del Val L, Lorenzo Pelayo S, De La Serna Higuera C, Atienza Sánchez R, Perez Miranda M. Diffuse intestinal ganglioneuromatosis an uncommon manifestation of Cowden syndrome. World J Gastrointest Oncol 2013; 5(2): 34-37
- URL: https://www.wjgnet.com/1948-5204/full/v5/i2/34.htm
- DOI: https://dx.doi.org/10.4251/wjgo.v5.i2.34