Table 1.
Clinical characteristics of FAP with and without desmoids
| FAP with desmoids (n=18) | FAP without desmoids (n=57) | p-value | |
|---|---|---|---|
| Gender | 0.912 | ||
| Male | 11 (61.1) | 37 (59.6) | |
| Female | 7 (38.9) | 23 (40.4) | |
| Age at diagnosed FAP (yr) | 28 (19-45) | 30 (13-78) | 0.264 |
| Incidence of CRC | 5 (27.8) | 20 (35.1) | 0.566 |
| AJCC stage of CRC | 0.018 | ||
| 0/I/II/III | 3/0/0/2 | 1/5/5/9 | |
| Pregnancy history | 3/7 (42.8) | 12/23 (52.1) | 0.907 |
| Extracolonic manifestation | |||
| Osteoma | 2 (11.1) | 5 (8.8) | 0.766 |
| Dental anomaly | 1 (5.6) | 3 (5.3) | 0.962 |
| CHRPE | 1 (5.6) | 8 (14.0) | 0.334 |
| Epidermal cyst | 5 (27.8) | 1 (1.8) | < 0.001 |
| Fundic gland polyps | 10 (55.6) | 38 (66.7) | 0.392 |
| Papillary thyroid cancer | 3 (16.7) | 3 (5.3) | 0.120 |
| Duodenum / small bowel adenoma | 4 (22.2) | 27 (47.4) | 0.059 |
| Stomach cancer | 1 (5.6) | 1 (1.8) | 0.383 |
| Adrenal adenoma | 2(11.1) | 3 (5.3) | 0.386 |
| Family history (CRC or FAP) | 11 (61.1) | 21 (36.8) | 0.070 |
| Survival rate at final follow-up | 15 (83.3) | 50 (87.7) | 0.633 |
| Cause of death | 0.007 | ||
| CRC related | 0 | 4 | |
| Desmoid related | 3 | 0 | |
| Other causes | 0 | 3 | |
| Follow-up (mo) | 76 (19-203) | 59 (2-247) | - |
Values are presented as number (%) or median (range). FAP, familial adenomatous polyposis; CRC, colorectal cancer; AJCC, American Joint Committee on Cancer; CHRPE, congenital hypertrophy of the retinal pigment epithelium.