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颅骨炎性肌纤维母细胞瘤1例

2018-06-13 张相华 方娟 黄宽明 中国临床神经外科杂志

女性,62岁,因发现额部包块1个月余入院。

【一般资料】

女性,62岁,

【主诉】

因发现额部包块1个月余入院。

【既往史】

既往有甲状腺包块切除术病史(自诉病理检查为良性),有结核病史(自诉已治愈)。

【体格检查】

神志清楚;额部可及一大小约3cm×3cm质软包块,压痛阳性,不能移动;双侧瞳孔等大等圆,直径2.0mm,光反射存在;颈软,颈部见术后疤痕;四肢肌力、肌张力正常。

【辅助检查】

外院颅脑MRI示额部包块。入院后复查颅脑MRI平扫+增强示(图1A~C):额骨骨质破坏,肿瘤可能性大,感染不排除;额骨示骨质破坏,局部示软组织肿块,病变侵及额窦及前组筛窦,T1WI呈等信号,T2WI呈稍高信号;增强扫描后,额骨病变均匀强化,与局部脑膜分界不清,脑实质未见明显受累。



【治疗】

全麻下行额骨肿瘤切除术。术中见肿瘤似结缔组织样,侵及前颅底骨质,硬脑膜未受累。术后复查颌面部CT三维重建示额部术后改变(图1D)。术后行放疗。随访2年无复发(图1E~G)。术后病理检查考虑炎性肌纤维母细胞瘤(inflammatorymyofibroblastictumor,IMT;图1H)。

【讨论】

IMT性质介于良恶性之间,常伴有炎性细胞浸润;可以发生于多个部位,最常见于肺部,颅骨少见。文献报道中枢神经系统IMT起源于硬膜。本文病例从影像学分析似乎硬脑膜有侵犯,但术中见硬脑膜并未受累,而是以局部骨质破坏为主。IMT好发于儿童和青少年,发生于40岁以上罕见。本文病例年龄62岁。目前IMT病因尚不清楚,因病检有炎性细胞浸润,有学者认为与感染有关。本文病例病变侵及额窦及前组筛窦,局部鼻窦炎症可能是其发生的诱因。IMT临床表现及影像学均缺乏特异性,确诊需术后病理检查,但术前影像学检查对确定手术治疗方案有帮助。IMT临床极其少见,目前尚无确切有效的治疗方案,仍以彻底手术切除为主,术后需密切观察随访。

原始出处:

张相华, 方娟, 黄宽明,等. 颅骨炎性肌纤维母细胞瘤1例[J]. 中国临床神经外科杂志, 2017, 22(8):605-605.

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