Table 1.
Extraintestinal Manifestations of Cowden Syndrome With Their Diagnostic Criteria
| Manifestation | Type of criteria |
|---|---|
| Facial tirchilemmomas | Pathognomonic |
| Acral keratoses | Pathognomonic |
| Papillomatous papules | Pathognomonic |
| Lhermitte-Duclos disease | Pathognomonic |
| Breast adenocarcinoma | Major |
| Fibrocystic breast disease | Minor |
| Thyroid multinodular goiter | Minor |
| Thyroid adenoma | Minor |
| Thyroid carcinoma | Major |
| Uterine leiomyomas | Minor |
| Endometrial carcinoma | Major |
| Bicornutae uterus | Minor |
| Macrocephaly | Major |
| Developmental delay | Minor |
| Lipomas | Minor |
| Fibromas | Minor |
| Melanoma | Minor |