Jaffe-Campanucci综合征影像诊断与鉴别诊断

目的探讨Jaffe-Campanucci综合征的影像学表现及鉴别诊断。方法回顾分析5例经病理证实的Jaffe-Campanucci综合征患者的影像学资料。5例均行数字成像系统(DR)检查,4例行CT,1例行磁共振(MR)检查。根据病灶分布及数目分为单骨多灶型、多骨单灶型及混合型。结果 5例均有皮肤咖啡色样斑,1例身材矮小伴发智力发育迟缓。多骨单灶型3例,混合型2例。多见于股骨下干骺端(3骨、4灶)及胫骨上干骺端(4骨、6灶)。病灶位于皮质内偏心性、膨胀性生长,内缘见连续的致密骨性间隔,外缘可见骨壳不完整、骨壳完整或无骨壳3种类型表现。结论 Jaffe-Campanucci综合征以多发非骨化性纤维瘤和皮肤咖啡色素斑为主要特征,其骨病灶影像特点与非骨化性纤维瘤完全一致。

Jaffe-Campanucci syndrome imaging diagnosis and differential diagnosis

Objective To access imaging features of Jaffe-Campanucci syndrome and differential diagnosis.Methods The imaging data of 5 patients proved pathologically were retrospectively analyzed.5 patients underwent DR examination,CT scans were performed on 4 of them,MR scan on 1 case.According to the distribution and amount of lesion is devided into multifocal of single bone,single stove of multiple bone and mixed type.Results Coffee colour spots of the skin were found in 5 cases,short stature in 1 case accompanied with mental retardation.Three of 5 cases were single stove of multiple bone type,2 of 5 cases were mixed type.Most of the focus were distributed in the inferior metaphysis of femur（3 bones,4 foci） and the upper metaphysic of tibia（4 bones,6 foci）.Lesions were located in the cortex of the eccentric,grew tumefaciently.Medial edge was revealed the completely continuous dense bony interval.The features of exterior edge were classified three types,including of incomplete bony shell,complete shell and none of bony shell.Conclusiaon Jaffe-Campanucci syndrome mainly exhibits multiple non-ossifying fibroma and skin pigmentation,the imaging characteristics of the involved bone are as same as the non-ossifying fibroma.