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儿童内生软骨瘤影像学表现及其病理学特点探讨

作者:刘 菲 史新乐 赵小龙 王丽霞

所属单位:河北北方学院附属第一医院影像科(河北 张家口 075000)

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摘要

目的研究儿童内生软骨瘤影像 学及病理学特点。方法 回顾分析12例内 生软骨瘤影像特点,评估疾病大小、与生 长板位置关系、骨基质骨化特点、骨膜反 应、MR信号特点和骨及软组织水肿。结果 疾病位于指骨为33% 、掌骨为25%、股骨 为33%、胫骨为8%/。CT和X显示观察到20% 肿瘤出现典型环形和弧形基质骨化。75% (9/12)发现骨内扇形征。内生性软骨瘤邻 近生长板生长为50%。MR显示典型软骨骨 化形式(环形和弧形),T1低信号,T2高 信号,增强后周围环形强化。结论 儿童 内生软骨瘤相对少见,常表现为骨内扇形 征,基质骨化罕见。

Objective To study imaging and pathological findings of enchondromas in children. Methods 12 enchondromas of imaging feature were retrospectively reviewed. Imaging evaluation included the assessment of lesion size, location to the growth plate, mineralization matrix, periosteal reaction, MR signal characteristics, and intraosseous and soft-tissue edema. Results Lesions located in the phalanx, metacarpal, femur, tibia take account for 33%, 25%, 33% and 8% respectively. Matrix mineralization in typical "ringsand-arcs" pattern was observed in 20% of the lesions with radiography/CT. Lesions were expansive with endosteal scalloping in 75% of cases and 50% of them were close to growth plate. All enchondromas in MR imaging demonstrated as typical cartilaginous matrix of lobules with high T2 signal, low T1 signal, and peripheral enhancement. Conclusion Enchondroma in children are relatively rare tumors, with frequent endosteal scalloping and rare matrix mineralization.

【关键词】内生软骨瘤;X线;磁共振成像;计算机体层摄影术

【中图分类号】R445.2;R445.3;R738.3

【文献标识码】A

【DOI】10.3969/j.issn.1672-5131.2015.09.004

前言

儿童骨肿瘤发病率低,以良性骨肿瘤较为多见[1],其中又以软骨 类肿瘤最为常见,包括骨软骨瘤、内生性软骨瘤、骨膜软骨瘤、软 骨母细胞瘤、软骨粘液样纤维瘤[2]。内生性软骨瘤是一种良性髓内肿 瘤,是儿童第二常见良性软骨性骨肿瘤,占儿童良性骨肿瘤24%[3]。成 人内生性软骨瘤50%发生于手指,尤其为掌骨中部和远端部分和指骨近 端[4],其他好发于股骨、肱骨和足部近段和末端。内生性软骨瘤通常 无痛,通常30~40岁被发现[5]。长骨内生性骨软骨瘤大部分病例是在 外伤后或关节疾病进行X线检查偶然被发现[6],大约25%内生软骨瘤在 儿童发现,大部分在20岁左右发病[7]。文献报道在胚胎发育期,源于 骨骺板的生长软骨细胞移位到干骺端,随后这些细胞发展为内生性软 骨瘤[8],最近研究发现内生性软骨瘤与IDH1和 IDH2基因突变有关[9]。 本研究目的是详细描述儿童内生性软骨瘤的影像学表现和病理特点。