Multiple jaw cysts and ectopic calcification

Description

A 41-year-old woman presented with pus discharge from the gingival pocket and a 10-year history of intermittent buccal swelling on the left side. Physical examination revealed increased height, macrocephaly, hypertelorism, wide nasal bridge and sloping shoulders. Orthopantomography showed multiple jaw cysts (figure 1). Keratocystic odontogenic tumour (KCOT) was histologically suggested by the preoperative biopsy. CT revealed ectopic calcifications in the falx and tentorium (figure 2A). Subcutaneous radiopaque bodies that seemed like granules were diffusely scattered in the skull (figure 2B). Punch biopsy revealed foreign calcifications but no histological abnormalities in the hair follicle. Then all jaw cysts were extirpated under general anaesthesia. The histological analysis confirmed the diagnosis of KCOT. These clinical presentations were consistent with a diagnosis of nevoid basal cell carcinoma syndrome (NBCCS). On the other hand, palmar or plantar pits, basal cell carcinoma (BCC) and bifid ribs, which...

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