“…sparse types are often collectively called classical FAP (CFAP) or typical FAP, while strict differentiation between severe and sparse types is of little clinical significance. 6 In addition to colorectal manifestations, some extracolonic lesions are associated with FAP (►Table 1): (1) desmoid tumors (DTs), second cause of death of patients with FAP, are fibroblastic lesions that do not metastasize but present aggressive growth and it has demonstrated that familyhistory, specific APC mutations, female, and surgery are high-risk factors in FAP-associated DT 7,8 ; (2) upper intestinal polyposis, including fundic gland polyposis, gastric adenoma, duodenal adenoma, and ampullary adenoma, is often detected in FAP patients; (3) congenital hypertrophy of retinal pigment epithelium (CHRPE); (4) neoplastic lesions, such as subcutaneous soft tissue tumors, osteomas, and dental abnormalities; and (5) other tumorous lesions, including thyroid cancer, adrenal tumor, hepatoblastoma, and brain tumors. 6,9,10 Therefore, FAP can be diagnosed by clinical characters (!…”