CAJ | 학술논문

目的探讨骨原发性中间型血管内皮肿瘤病理特征、鉴别诊断和骨血管肿瘤的分类.方法收集5例发生于骨的中间型血管内皮肿瘤,对其临床、病理、影像学和免疫组织化学特征进行分析,免疫标记包括CD31、CD34、平滑肌肌动蛋白(SMA)、波形蛋白、CK、上皮细胞膜抗原(EMA)6种抗体.结果 5例中间型血管内皮肿瘤中3例为卡波西型血管内皮瘤(患者均为儿童),2例网状血管内皮瘤(患者均为中老年人).4例位于四肢长骨,1例在锁骨.影像学(包括X线平片、CT、MRI)表现为交界性或低度恶性骨质破坏,骨皮质均有不同程度缺损,4例伴病灶内或周边成骨,其中l例有放射状骨针.病理组织学形态均与软组织相应的中问型血管内皮肿瘤相似,但大多伴病灶内或周围反应性骨形成.免疫组织化学5例CD31、CD34、波形蛋白均不同程度阳性,3例SMA阳性,CK、EMA均阴性.结论骨原发性中间型血管内皮肿瘤是客观存在的,冈此骨血管肿瘤的分类应该逐步与软组织血管肿瘤分类相一致,在良性血管瘤和恶性血管肉瘤之间,增设一类中间型血管内皮肿瘤.骨中间型血管内皮肿瘤的生物学行为是否与软组织相应肿瘤一致,应通过长期随访资料的积累才能定论.
목적 탐토골원발성중간형혈관내피종류병리특정、감별진단화골혈관종류적분류.방법 수집5례발생우골적중간형혈관내피종류,대기림상、병리、영상학화면역조직화학특정진행분석,면역표기포괄CD31、CD34、평활기기동단백(SMA)、파형단백、CK、상피세포막항원(EMA)6충항체.결과 5례중간형혈관내피종류중3례위잡파서형혈관내피류(환자균위인동),2례망상혈관내피류(환자균위중노년인).4례위우사지장골,1례재쇄골.영상학(포괄X선평편、CT、MRI)표현위교계성혹저도악성골질파배,골피질균유불동정도결손,4례반병조내혹주변성골,기중l례유방사상골침.병리조직학형태균여연조직상응적중문형혈관내피종류상사,단대다반병조내혹주위반응성골형성.면역조직화학5례CD31、CD34、파형단백균불동정도양성,3례SMA양성,CK、EMA균음성.결론 골원발성중간형혈관내피종류시객관존재적,강차골혈관종류적분류응해축보여연조직혈관종류분류상일치,재량성혈관류화악성혈관육류지간,증설일류중간형혈관내피종류.골중간형혈관내피종류적생물학행위시부여연조직상응종류일치,응통과장기수방자료적적루재능정론.
Objective To study the radiologic and pathologic features of primary intermediate hemangioendothelioma of the bone.Methods Five cases of primary intermediate hemangioendothelioma of bone encountered in the past three years were enrolled into the study.The clinical,radiologic,pathologic and immunohistochemical features of the tumors were reviewed.Results The patients included 3 children with Kaposiform hemangioendothelioma and 2 elderly with retiform hemangioendothelioma.Four of the cases affected long bones and the remaining case affected the clavicle.One case showed multifocal involvement of the humerus. Radiologically,the tumors showed borderline to low-grade bony destruction,with various degrees of cortical defect.Intralesional or perilesional bone formation was demonstrated in 4 cases and radial spicules were seen in 1 case.The histopathologic features of primary intermediate hemangioendothelioma of bone were similar to those of soft tissue, except for the presence of reactive bone formation.Immunohistochemically,the tumor cells were positive for CD31 (5/5),CD34 (5/5),vimentin (5/5) and smooth muscle actin (3/5) but negative for cytokeratin and epithelial membrane antigen. Conclusions Primary intermediate hemangioendothelioma of bone is a distinct entity and similar histologic classification applies as in its soft tissue counterparts.Comparison of the biologic behavior requires long-term follow-up studies.