Monosotic fibrous dysplasia and solitary intramuscular myxoma of the head and neck: A unique presentation of Mazabraud’s syndrome and a literature review

Abstract

Mazabraud’s syndrome (MS) is a rare disease that is a combination of fibrous dysplasia and intramuscular myxomas. MS is a benign lesion and there is little data on the disease due to its low incidence. In the present study, the case of a 38-year-old patient who presented with a soft-tissue mass involving the masseter and swelling at the mandibular body and mandibular ramus is reported. Since the mandible is an important aesthetic and functional organ in the oral and maxillofacial region, surgery was primarily aimed at resecting the tumor, with good safety margins, and reconstructing the resultant defect. The lesions were pathologically diagnosed as MS. The unique features of this case included the painless and monostotic fibrous dysplasia, the solitary intramuscular myxomas involving the jaw and the male gender of the patient. MS usually occurs in the lower extremities, with an unusual predilection for the right limb; however, it rarely occurs in the head and neck region. A retrospective analysis of the clinical features and management of MS was also performed in the present study, together with a literature review. From the literature, it was concluded that the incidence of MS is ~2.3-fold greater in female patients than in male patients, and that the age of onset of MS ranges between 17 and 82 years, with an average age of 46.25 years.