颅骨硬纤维瘤一例报告并文献复习

目的探讨颅骨硬纤维瘤(desmoplastic fibroma,DF)的临床、病理、放射学特征及治疗方法。方法报告1例颅骨硬纤维瘤病例,结合文献探讨该疾病的临床特点、放射学特征、病理表现、治疗及预后。结果颅骨硬纤维瘤在临床上极为罕见。X线和CT表现为溶骨性破坏,形成均匀一致或泡沫状的软组织包块,MRI T1加权像表现为中等信号或低信号,有明显增强效应,T2加权表现为低信号,局部区域为高信号。病理学特点:胶原纤维构成病灶的主要成分,其中分布有长梭形或圆胖形的纤维细胞,无异形性,没有有丝分裂像,可与其他恶性肿瘤相鉴别。本病有复发和转移倾向,手术应彻底,可配合放射治疗和内分泌治疗。结论颅骨硬纤维瘤是一种局部侵袭性较强的病变,应结合CT、MRI和病理学特点诊断本病,最好的治疗措施为手术全切,根据激素受体水平考虑内分泌治疗,可减少复发。

Desmoplastic fibroma of the cranium: a case report and review of literatures

Objective To study on clinical,pathological,radiological feature and treatment of the desmoplastic fibroma (DF) in the calvaria.Methods A patient with desmoplastic fibroma was reported,at the same time,the disease's clinical feature,radiological findings,pathological character,treatment and prognosis were reviewed in the literatures.Results DF is a rare tumor.Typical CT and X-ray findings include lyric bone destructive,forming uniform or soap bubble soft tissue tumor.The mass was homogeneously hypointense or isointense with gray matter on T1 weighted MRI scans,and it enhanced fairly uniformly with gadolinium,the mass was predominantly of low signal on T2 weighted images,with discrete areas of high signal.Microscopically,DF is composed of slender or plump fibroblasts with ovoid nuclei sparsely dispersed within a collagenous,a lack of mototic figures and nuclear atypia may distinguish DF from more malignant bone neoplasms.The disease has the polarity of recurrence and metastasis,suggesting possible en bloc resection and management either primarily or with adjunctive radiotheraply and endocrine therapy.Condusions Desmoplastic fibroma in calvaria is a highly invasive local aggressive lesion whose diagnosis result from CT findings,MRI scans and pathological features,which is best treated by surgical intervention.Immunohistochemical analysis may demonstrate hormone receptors,in which case endocrine therapy may reduce recurrence.