Intracranial Dermoid in Patients With Fibrous Dysplasia

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OBJECTIVES: Intracranial epidermoid cyst (EC) and craniofacial fibrous dysplasia (CFD) were histogenetically different rare congenital benign diseases. The coexistence of intracranial EC and CFD was extremely rare and had not been reported yet. MATERIALS AND METHODS: We retrospectively reviewed the clinical and radiologic information of 3 patients diagnosed with concomitant EC and CFD at Beijing Tiantan Hospital from January 2003 to January 2021 and summarized their clinicopathological features, treatment modalities, and outcomes. In addition, we performed a systematic review of cases of the coexisting intracranial EC and other intracranial abnormalities to explore the potential connections. RESULTS: There were 2 women and 1 man with the mean age of 31 years old. Satisfactory resection was fulfilled for all the 3 ECs. CFD, however, was managed with watchful waiting. During the mean follow-up time of 58 months, all the ECs showed no sign of recurrence, and all the CFD lesions remained stable. Two EC specimens underwent genetic study, showing no GNAS mutations and negative G s α protein expression. In the literature review of concomitant intracranial EC and other intracranial abnormalities, 23 studies were included. With 5 reported cases, the intracranial aneurysm was found to be the most common intracranial disease that coexisted with EC. CONCLUSIONS: The coexistence of intracranial EC and CFD was extremely rare. However, no convincing mechanism and evidence underlying such coexistence had been found. To provide more profound understanding about these 2 diseases and improve diagnosis and treatment strategy, further research and verification should be considered.

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