钙化性腱膜纤维瘤8例临床病理分析

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目的探讨钙化性腱膜纤维瘤(CAF)临床病理特点、免疫组化、病理诊断与鉴别诊断要点。方法收集8例CAF,分析其临床病理特点、免疫组化、治疗与预后,并复习相关文献。结果 8例CAF中男性5例,女性3例,年龄3~63岁,平均24岁。病变主要位于手掌和足底。肿瘤直径1~5 cm,境界不清;切面呈灰白色,质硬。镜下见典型的散在结节状钙化,周边围绕一定方向线性排列的小圆形细胞及软骨细胞样小岛;融合的钙化结节之间为束状排列的梭形纤维母细胞性细胞,向周围软组织浸润性生长,细胞无明显多形性、异型,核分裂少见;结节内间质常玻璃样变及呈软骨样。免疫组化:梭形肿瘤细胞8例vimentin均(+),4例SMA(+),1例CD34(+),5例S-100(+),6例CD99(+)。8例患者均行肿瘤切除术,随访5~66个月,其中3例复发。结论 CAF是一种少见的良性软组织肿瘤,其诊断需要结合临床病理形态学特点及免疫组化,且还需与婴幼儿指趾纤维瘤病、婴幼儿纤维性错构瘤、软组织软骨瘤、单相纤维型滑膜肉瘤、低度恶性纤维肉瘤等相鉴别。 Objective To investigate the clinicopathological characteristics, immunohistochemistry, pathological diagnosis and differential diagnosis of calcific aponeurotic fibroma (CAF). Methods Eight cases of CAF were collected and analyzed for their clinicopathological characteristics, immunohistochemistry, treatment and prognosis, and review of relevant literature. Results There were 5 males and 3 females in 8 cases of CAF, aged from 3 to 63 years old, with an average of 24 years old. Lesions are mainly located in the palms and soles. Tumor diameter of 1 ~ 5 cm, the state is not clear; section was gray, hard. Microscopic see typical scattered nodular calcification around the periphery of a certain direction of the linear arrangement of small round cells and chondrocyte-like islands; fusion of calcified nodules between the bundles of spindle-shaped fibroblasts cells to Peripheral soft tissue infiltration of growth, cells without significant pleomorphism, atypia, mitotic rare; nodular interstitial often glassy change and cartilage-like. Immunohistochemistry: 8 cases of spindle tumor cells were vimentin (+), 4 cases of SMA (+), 1 case of CD34 (+), 5 cases of S-100 (+), 6 cases of CD99 (+). Tumor resection was performed in 8 patients. The patients were followed up for 5 to 66 months and 3 of them relapsed. Conclusions CAF is a rare benign soft tissue tumor. The diagnosis of CAF needs to be combined with clinicopathological features and immunohistochemistry. It also needs to be combined with pediatric fibrosis of the thumb, fibrous fibromatomas of infants and young children, soft tissue chondroma, Phase fiber synovial sarcoma, low grade fibrosarcoma and other phase identification.
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