CAJ | 학술논문

报告1例Maffucci综合征.患者女,42岁.因有足、左手结节渐增多6～7年,手指结节疼痛1年就诊.患者7岁时右小腿骨折后出现畸形致跛行.左手X线摄片显示多根掌骨膨胀性改变,并有多发性钙化影.左手结节组织病理检查示海绵状血管瘤及梭形细胞血管瘤,部分可见血栓形成及静脉石.免疫组化染色示海绵状血管瘤区管腔内内皮细胞、裂隙状血管腔内皮细胞及圆形空泡细胞CD31、CD34和FVIII均阳性,而实心区波形蛋白(vimentin)阳性,余为阴性.临床表现和皮损组织病理改变符合Maffucci综合征诊断.
보고1례Maffucci종합정.환자녀,42세.인유족、좌수결절점증다6～7년,수지결절동통1년취진.환자7세시우소퇴골절후출현기형치파행.좌수X선섭편현시다근장골팽창성개변,병유다발성개화영.좌수결절조직병리검사시해면상혈관류급사형세포혈관류,부분가견혈전형성급정맥석.면역조화염색시해면상혈관류구관강내내피세포、렬극상혈관강내피세포급원형공포세포CD31、CD34화FVIII균양성,이실심구파형단백(vimentin)양성,여위음성.림상표현화피손조직병리개변부합Maffucci종합정진단.
A 42-year-old woman with Maffucci syndrome is reported. The patient began to develope multiple hard or com- pressible nodular which sometimes tender on beth right foot and left hand after her right calf bone was fractured and de- formed at age of 7. Radiographs of the left hand showed multiple radiolucent lesions with osseous expansible remodeling and well-defined round calcifications in the soft-tissue masses (typical of phleboliths). Histopathologic examination on skin speci- men of left hand demonstrated the vascular abnormalities consisting of multiple cavernous hemangiomas, spindle cell heman- giomas and thrembi formed and calcified as phleboliths, Immunohistochemistry for above specimen showed that endotheliocyte of the cavernous hemangiomsa and slit-shaped vascular channel and round vacuolated cell of slit-shaped vascular channel were positive staining for CD31, CD34 and the factor VIII. Spindle cell of tissue but vascular channel was positive staining for vimentin and negative for others. These finding are consistent with Maffucci syndrome.