Aggressive fibrous dysplasia of bone communicative involving the skull base: a case report and review of the relevant literatures

Abstract

Objective To explore the diagnosis and surgical management of huge aggressive fibrous dysplasia of bone communicative involving the skull base. Methods The clinical data of a patient with aggressive fibrous dysplasia of bone communicative involving the skull base was retrospectively analyzed, and combined the follow-up information for further analysis. Meanwhile, the relevant articles and latest researches were reviewed. The patient was a 29-year-old male with complaint of nasal obstruction, hyposphresia, and blurred vision. CT and MRI examination revealed a large communicating tumor involved both anterior and middle skull base ( 9.7 cm×8.8 cm×7.9 cm ). He received surgical treatment via transmaxillary approach which achieved total lesion resection. Results After first surgery, patient′s complaint of nasal obstruction and blurred vision archived complete remission, but with complete loss of sense of smell. The pathological result accorded with fibrous dysplasia with aggressive behavior of growth. Three months after surgery during follow-up, we found the patient got recurrence without clinical symptoms confirmed by MRI exam. One year after surgery, a second operation for saving the vision loss took place. The pathological result after second operation accorded with fibrous dysplasia but without aggressive behavior of growth this time. No obvious progress is found in follow-up till now. Conclusions Aggressive fibrous dysplasia of bone communicative involving the skull base progressed rapidly, and show atypical imaging manifestation. Pathology reveals adverse biological behavior and easy relapse after surgery. Radical surgery is advocated, while radiotherapy and chemotherapy and medication are not recommended. Furthermore, we should pay attention to follow-up closely and be on guard against the malignant transformation. Key words: Bone neoplasms; Fibrous dysplasia of bone; Skull base; Aggressive