浅表性CD34阳性纤维母细胞肿瘤6例临床病理分析及文献复习

Clinicopathological analysis of 6 cases of superficial CD34 positive fibroblastic tumors and review of literatures

目的探讨浅表性CD34阳性纤维母细胞肿瘤(superficial CD34 positive fibroblastic tumors,SCPFT)的临床病理特点、免疫表型、诊断和鉴别诊断。方法回顾性分析6例SCPFT,行常规HE染色、免疫组化EliVision法染色。应用FISH法检测PRDM10基因断裂情况,并复习相关文献资料。结果6例SCPFT患者中,男性2例、女性4例。其中4例肿块位于下肢皮下,1例位于下腹部,另1例位于外阴。镜检:肿瘤细胞排列呈束状、实片状,细胞核明显多形性或畸形,核仁明显,可见核内假包涵体,胞质嗜酸性,部分区域细胞呈上皮样,未见核分裂象,间质散在少量炎细胞,免疫表型:肿瘤细胞弥漫强表达CD34和INI1,部分细胞表达CK(AE1/AE3),不表达SMA、CD68、desmin、S-100、CD31和ERG,Ki67增殖指数低于3%。FISH检测:4例SCPFT行FISH检测,其中3例PRDM10呈阳性。结论SCPFT是新近报道的一种软组织肿瘤,具有交界性或低度恶性生物学行为,诊断需与多种CD34阳性的软组织肿瘤相鉴别,避免过度诊断及治疗。

Purpose To investigate the clinicopathological features,immunophenotype,diagnosis and differential diagnosis of superficial CD34-positive fibroblast tumor.Methods Retrospective analysis was performed in 6 cases of superficial CD34 positive fibroblastic tumors.Routine HE staining was performed under light microscopy,immunohistochemical EliVision staining was performed,and FISH was used to detect the PRDM10 gene.Literature review was also conducted.Results Among the 6 SCPFT patients,there were 2 males and 4 females.Four masses were located subcutaneous in the lower limbs,one in the lower abdomen,and one in the vulva.Microscopically,the tumor cells were arranged in bundles or solid patches,with obvious pleomorphism or deformity of the nucleus and nucleoli.Pseudoinclusion bodies were visible in the nucleus,and the cytoplasm was eosinophilic.Some areas of the cells were epithelioid,with no mitotic figures observed.A small amount of inflammatory cells were scattered in the stroma.Immunophenotypically,diffuse and strong expression of CD34 and INI1 was observed in the tumor cells,some cells expressed CK(AE1/AE3),but did not express SMA,CD68,desmin,S-100,CD31,ERG.Ki67 had a value-added index below 3%.FISH detection of PRDM10 gene was performed on 4 cases of SCPFT,and 3 cases were positive.Conclusion Superficial CD34-positive fibroblast tumor is a newly reported soft tissue tumor with borderline or low-grade malignant biological behavior.Diagnosis needs to be differentiated from multiple CD34 positive soft tissue tumors to avoid over diagnosis and over treatment.