Sigel J E, Smith T A, Reith J D, Goldblum J R
Department of Anatomic Pathology, The Cleveland Clinic Foundation, Cleveland, OH 44195, USA.
Ann Diagn Pathol. 2001 Feb;5(1):10-4. doi: 10.1053/adpa.2001.21474.
Calcifying fibrous pseudotumor (CFT) is a rare benign soft tissue lesion composed of dense hyalinized fibrous tissue containing bland spindle-shaped cells admixed with a lymphoplasmacytic infiltrate and foci of dystrophic and often psammomatous calcifications. It has been suggested that CFT represents a late sclerosing stage of inflammatory myofibroblastic tumor (IMT). Recently, clonal cytogenetic abnormalities involving the anaplastic lymphoma kinase (ALK) gene on chromosome 2p have been identified in IMT, particularly those arising in deep soft tissue sites. We evaluated seven cases of deep soft tissue CFT diagnosed at the Cleveland Clinic Foundation and the University of Florida with available paraffin-embedded blocks using a monoclonal antibody to ALK (Dako, Carpenteria, CA) and a modified avidin-biotin complex method. The cohort included six women and one man with a median age at diagnosis of 43 years (range, 26 to 67 years). Sites of CFT included mesentery (3), peritoneum (1), omentum (1), serosa of small bowel (1), and anterior mediastinum (1). Immunohistochemically, only one case showed focal staining for ALK. The remaining six cases were negative, with appropriate positive and negative control staining. In conclusion, unlike IMT, CFT in deep soft tissue locations rarely expresses ALK by immunohistochemistry, suggesting that CFT is a different clinicopathologic entity than IMT, as opposed to representing a "burned out" IMT. Ann Diagn Pathol 5:10-14, 2001.
钙化性纤维性假瘤(CFT)是一种罕见的良性软组织病变,由致密的玻璃样变纤维组织构成,其中含有温和的梭形细胞,并伴有淋巴浆细胞浸润以及营养不良性且常为砂粒体样的钙化灶。有人提出CFT代表炎症性肌纤维母细胞瘤(IMT)的晚期硬化阶段。最近,在IMT中已鉴定出涉及2号染色体上间变性淋巴瘤激酶(ALK)基因的克隆性细胞遗传学异常,尤其是那些发生于深部软组织部位的IMT。我们使用抗ALK单克隆抗体(Dako,加利福尼亚州卡平特里亚)和改良的抗生物素蛋白-生物素复合物方法,对克利夫兰诊所基金会和佛罗里达大学诊断的7例深部软组织CFT且有可用石蜡包埋块的病例进行了评估。该队列包括6名女性和1名男性,诊断时的中位年龄为43岁(范围26至67岁)。CFT的部位包括肠系膜(3例)、腹膜(1例)、大网膜(1例)、小肠浆膜(1例)和前纵隔(1例)。免疫组织化学检查显示,仅1例呈ALK局灶性染色。其余6例为阴性,同时有合适的阳性和阴性对照染色。总之,与IMT不同,深部软组织部位的CFT通过免疫组织化学很少表达ALK,这表明CFT是一种与IMT不同的临床病理实体,而不是代表“消退型”IMT。《诊断病理学杂志》5:10 - 14,2001年。