颅骨促结缔组织增生性纤维瘤临床病理特征  

Clinicopathological features of desmoplastic fibroma of the skull

作　　者：张秀梅[1,2] 马捷[1] 王海[1] 周航波[1] 金行藻[1] 周晓军[1] 

机构地区：[1]南京军区南京总医院病理科,南京210002 [2]江苏省兴化市人民医院病理科

出　　处：《诊断病理学杂志》2008年第5期365-368,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨颅骨促结缔组织增生性纤维瘤(DF)的临床特征。方法对2例颅骨DF进行病理形态和免疫组化观察,结合临床资料进行分析并复习相关文献。结果2例颅骨DF患者均为男性,年龄分别为25岁和44岁,病程均较长,临床以局部无痛性肿块为主要表现。其中例1伴头晕、视物模糊,幼年时曾有外伤史,影像学示顶骨囊状膨胀性骨质破坏,病变周围骨皮质变薄;例2首次顶骨病灶切除半年后复发,并显示额、顶骨及左颞骨多处受累。光镜下肿瘤由梭形瘤细胞和大量胶原构成,胶原纤维密集而粗大,呈波浪状或束状,瘤细胞核形态温和,未见核分裂象。免疫组化:瘤细胞β-catenin(2/2)、AR(2/2)、ER(1/2)和PR(2/2)(+),SMA和S-100局灶(+),但desmin、CD117和CD34(-)。结论颅骨促结缔组织增生性纤维瘤是一种罕见的具有较强局部侵袭性的肿瘤,病灶切除不彻底易复发,手术广泛全切是本病的最佳治疗措施。Objective To investigate the clinicopathulogical featurs of desmoplastic fibroma （DF） of the skull. Methods Two cases of DF of the skull were observed by HE and immunohistochemical staining, and analyzed with their clinical features. Results Two cases of DF of the skull were both male and presented with a focal painless mass of long duration. Two men aged 25 and 44 years respectively. One case complained of dizziness and blurred vision with juvenile traumatic history. Radiologically, both showed cystic expansive destruction of parietal bone with peripheral cortical attenuation. One case relapsed half a year later and showed multiple involvement in frontal, parietal and left temporal bones. Microscopically, the tumor consisted of elongated spindle cells and thick wavy collagen bundles. The spindle cells had bland nuclei and mitosis was not found. Immanohistochemically, the spindle cells were positive for β-catenin （2/2）, AR （2/2）, ER （1/2）, PR （2/2）, focally positive for SMA and S-100, and negative for desmin, CD117, and CD34. Conclusion DF of the skull is a rare tumor with strongly local aggressiveness and high potential for recurrence after incomplete resetion. Complete resection is the best treatment.

关 键 词：纤维瘤 促结缔组织增生 颅骨 临床病理 免疫组化 

分 类 号：R730.262[医药卫生—肿瘤]