Angiero Francesca, Rizzuti Tommaso, Crippa Rolando, Stefani Michele
University degli studi di Milano Istituto di Anatomia Patologica sez. Patologia Orale, 20122 Milan, Italy.
Anticancer Res. 2007 Jul-Aug;27(4C):2573-81.
Fibrosarcoma (FS) is a malignant mesenchymal neoplasm of the fibroblasts that rarely affects the oral cavity. Two cases of primary FS of the jaws with intraosseous growth (2 men, aged 53 and 71 years) are described. Microscopically, in one case the tumor showed an intense proliferation of spindle-shaped cells, varying little in size and shape and arranged in parallel bands, partly crossing each other, with significant mitotic activity and nuclear pleomorphism; the second case was characterized by low cellularity comprising spindle-shaped cells, deposited in a variably fibrous and myxoid stroma. On immunohistochemistry, cells in both cases were strongly immunoreactive for MIB-1 and vimentin, focally positive for CD68, and negative for S-100 protein, pancytokeratin, HMB45, CD34, desmin, smooth muscle actin (SMA) and epithelial membrane antigen (EMA). Based on clinical, histological and immunohistochemical findings, the final diagnosis was FS in the first case, myxofibrosarcoma in the second. Treatment was radical surgery with mandibular reconstruction. After two years, the first patient displayed multiple metastases and died during the third year after the initial diagnosis; the second patient was still alive and doing well five years after treatment. We discuss the differential diagnosis versus other forms of sarcoma, examining the morphological appearance that is frequently very similar, the immunohistochemical expression of MIB-1, vimentin, S-100, CD-34, CD68, EMA, as well as conventional clinicopathological features that may help to distinguish FS from other sarcomas.
纤维肉瘤(FS)是一种罕见累及口腔的成纤维细胞恶性间叶性肿瘤。本文描述了2例颌骨原发性FS伴骨内生长的病例(2名男性,年龄分别为53岁和71岁)。显微镜下,1例肿瘤表现为梭形细胞强烈增殖,细胞大小和形态变化不大,呈平行束状排列,部分相互交叉,有明显的有丝分裂活性和核异型性;第2例的特征是细胞密度低,由梭形细胞组成,沉积于不同程度的纤维性和黏液样基质中。免疫组化显示,两例病例的细胞对MIB-1和波形蛋白均呈强免疫反应性,对CD68呈局灶性阳性,对S-100蛋白、全细胞角蛋白、HMB45、CD34、结蛋白、平滑肌肌动蛋白(SMA)和上皮膜抗原(EMA)均为阴性。根据临床、组织学和免疫组化结果,第1例最终诊断为FS,第2例为黏液纤维肉瘤。治疗方法为根治性手术并进行下颌骨重建。两年后,第1例患者出现多处转移,并在初诊后第三年死亡;第2例患者在治疗五年后仍存活且情况良好。我们讨论了与其他形式肉瘤的鉴别诊断,研究了通常非常相似的形态学表现、MIB-1、波形蛋白、S-100、CD-34、CD68、EMA的免疫组化表达,以及有助于将FS与其他肉瘤区分开来的传统临床病理特征。