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.