A systematic review of the literature of nevoid basal cell carcinoma syndrome affecting East Asians and North Europeans

Objective: To compare, by systematic review, North European and East Asian consecutive case series of nevoid basal cell carcinoma syndrome (NBCCS).

Study design: A systematic review of the literature was performed for all consecutive case series of NBCCS, which included keratocystic odontgenic tumors (KCOTs) arising in North European and East Asian communities. The clinical and radiologically apparent features were identified and synthesized, and a meta-analysis was performed.

Results: East Asian reports were significantly more "proband only" compared with North European reports. Significant differences between these 2 communities were observed for 5 of the 6 major features and 11 of the 27 minor features. With regard to the major NBCCS features, the North Europeans displayed significantly more frequent basal cell carcinomas, calcified falx cerebri, palmar and plantar pits, and a family history, whereas the East Asians displayed KCOTs significantly more frequently. With regard to minor features, East Asians displayed significantly more frequent cleft lips and palates and hypertelorism.

Conclusions: East Asians displayed multiple KCOTs and cleft lips and palates more frequently compared with North Europeans.