Multiple Exostosis Disease: Study of Three Senegalese Families

Abstract

Exostosis is a benign tumour corresponding to a well-differentiated bone excrescence, produced by a germinal cartilage cap during growth [1,2]. It is most often sporadic, but it can also be part of an autosomal dominant, multiple exostosis disease known as Bessel-Hagen disease [1]. Exostosis, especially in its solitary form, represents the most frequent benign tumour (20 to 50% of benign bone tumours and 10 to 15% of all bone tumours) [1]. The multiple exostosis disease is a rare disease first described by Boyer in 1814 [3-5]. It is mainly reported in Western literature where its prevalence is estimated at 1/50,000 [3,5] and, to a lesser extent, in North Africa [6,7]. This prevalence has not been determined in the African population. However, work has been carried out on the disease in North Africa and sub-Saharan Africa [3-12]. Studies evaluating family forms of the disease were mainly carried out in the Western literature [12-18]. In sub-Saharan Africa, reported cases are apparently sporadic. The objective of this work was to study the epidemiological, diagnostic and therapeutic aspects of familial forms of multiple exostosis disease.