Table 1: Incidence and observed/expected ratio of Xeroderma Pigmentosum-associated malignancies [14-16].
Malignancy | Incidence (all ages) | Observed/Expected (age < 20) | Observed/Expected (all ages) | |
Cutaneous | NMSC | 58-60% | 4900-9757 | 327 |
Melanoma | 22-32% | 1879-8000 | 193-700 | |
Non-Cutaneous | Ocular^ | 9-26% | 1000 | 1700 |
Oral* | 2% | 100,000 | 800 | |
Central Nervous System# | 2% | 50 | 33 |
^Reported ocular malignancies include SCC, epithelioma/BCC, melanoma.
*Reported oral malignancies include SCC of the tongue apex, gingiva, and palate.
#Reported central nervous system malignancies include brain sarcoma and spinal cord astrocytoma.