A consecutive case series of nevoid basal cell carcinoma syndrome affecting the Hong Kong Chinese

Abstract

To identify the clinical and radiologic features of nevoid basal cell carcinoma syndrome (NBCCS) in the Hong Kong Chinese, particularly those of keratocystic odontogenic tumors (KCOTs), at first presentation at a dental hospital. A consecutive case series of NBCCS was identified in the University of Hong Kong Dental Hospital. All 5 Hong Kong NBCCS cases presented with symptoms arising from their KCOTs; 3 with swelling, 3 with pain, and 2 with nasal discharge. The cases exhibited 4 major features (KCOTs, calcified falx cerebri, palmar/plantar pits, and basal cell carcinoma) and 4 minor features (sella bridges, bossing, hypertelorism, and mandibular prognathism). The KCOTs were all unilocular. The tumors displaced teeth in 4 cases. Only 1 had root resorption. There were 2 nonsyndromic cases with multiple KCOTs. The unilocular presentation of the syndromic KCOTs was significantly greater than that of the solitary cases, arising within the same community over the same period. The other presenting features of the syndromic KCOTs did not differ from the solitary KCOTs. The recurrence rate of syndromic KCOTs was significantly greater than of the solitary KCOTs. Nonsyndromic cases with multiple KCOTs could be more common in East Asians.