颅底侵袭性骨纤维性结构不良1例报道并文献复习

目的 探讨颅底巨大侵袭性骨纤维性结构不良(FDB)的诊断及外科治疗。方法 回顾性分析2013年8月中国医学科学院肿瘤医院神经外科收治的1例颅底侵袭性FDB患者的临床诊断资料和随访结果,并复习相关文献。患者男,29岁,CT和MRI检查发现前中颅底巨大(9.7 cm×8.8 cm×7.9 cm)沟通性肿瘤,于2013年8月28日经上颌骨掀翻入路切除肿瘤;因肿瘤复发、症状加重,于2014年9月3日二次行经右侧中颅底硬膜外入路肿瘤切除术。结果 首次手术后鼻塞和视力减退明显缓解、嗅觉消失,病理诊断为颅底侵袭性FDB;术后3个月MRI检查示肿瘤复发。二次术后病理诊断:颅底FDB,随访至今14个月,未见病情明显进展及肿瘤复发。结论 颅底侵袭性FDB的病程进展迅速,影像学表现不典型,病理学提示生物学行为不良,术后很快复发,主张根治性的手术治疗,不建议行放疗、化疗,同时注意密切随访、警惕恶变。

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

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.