Table 3.
Clinical features of 33 Carney Complex patients reported in Japan
| Clinical features | No. of patients | % |
|---|---|---|
| Age | mean, 30.3 years (range; 3-73 years) | |
| with PRKAR1A mutation | mean, 26.6±20.2 (n=7) | |
| without PRKAR1A mutation | mean, 64.3±11.7 (n=3) | |
| not available | mean, 27.0±15.5 (n=23) | |
| Gender | ||
| Male | 7 | 21 |
| Female | 26 | 79 |
| Family history | ||
| yes | 13 | 39 |
| no | 20 | 61 |
| Chief complaints | ||
| subcutaneous or beast tumor | 9 | 27 |
| pigment deposition of the skin | 8 | 24 |
| Cushing sign | 11 | 33 |
| others | 1 | 3 |
| no symptom | 4 | 12 |
| Clinical manifestations | ||
| spotty skin pigmentation | 16 | 49 |
| cardiac myxoma | 14 | 42 |
| cutaneous myxoma | 13 | 39 |
| primary pigmented nodular adrenocortical disease (PPNAD) | 17 | 52 |
| large-cell calcifying Sertoli cell tumor or steroid-type tumor or both | 1 | 3 |
| mammary myxomatosis or fibroadenoma | 7 | 21 |
| pituitary adenoma (acromegaly) | 13 | 39 |
| thyroid tumors | 5 | 15 |
| osteochondromyxoma | 0 | 0 |
| psammomatous melanotic schwannoma | 0 | 0 |
| blue nevus | 0 | 0 |
| Operation history | ||
| none | 6 | 18 |
| once | 15 | 46 |
| twice | 6 | 18 |
| more than three times | 6 | 18 |