Complications of the naevoid basal cell carcinoma syndrome: results of a population based study.

TLDR

The naevoid basal cell carcinoma syndrome is associated with numerous potential complications. A population‑based study of 84 patients found that over 90 % developed major complications by age 40, with additional risks of ovarian calcification/fibroma (24 %), medulloblastoma (5 %), cardiac fibroma (3 %), cleft palate (5 %), and ophthalmic abnormalities (26 %), offering clearer counseling and screening guidelines.

Abstract

There are many potential complications which have been reported in association with the naevoid basal cell carcinoma syndrome. We have been able to show the relative frequencies of these problems in a population based study of 84 cases in the north west of England. The major complications of basal cell carcinomas and jaw cysts occur in over 90% of patients by 40 years of age, but may both occur before 10 years of age. Less well described complications are ovarian calcification or fibroma (24%), medulloblastoma (5%), cardiac fibroma (3%), cleft palate (5%), and ophthalmic abnormalities such as squint or cataract (26%). This study more clearly defines the possible complications of the syndrome and gives clearer guidelines for counselling and screening affected and at risk persons.