Soares Andresa Borges, Lins Lauro Henrique Sousa, Macedo Adriana Parizzoto, Pereira-Neto Joao Sarmento, Vargas Pablo Agustin
Department of Oral Diagnosis, Oral Pathology Section, Dental School of Piracicaba, State University of Campinas, Piracicaba, Brasil.
Med Oral Patol Oral Cir Bucal. 2006 May 1;11(3):E243-6.
Fibrosarcoma is a malignant mesenchymal neoplasm of fibroblasts that rarely affects the oral cavity and can cause local recurrences or metastasis. In this article we describe a case of primary fibrosarcoma in the mandible in a 16-year-old girl. Microscopically 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, the mitotic activity was increased and there was nuclear pleomorphism. Immunohistochemically the cells only showed immunoreactivity for vimentin and negativity for S-100 protein, CD 68, cytokeratin cocktail, HMB-45, CD34, pan actin HHF 35, desmin, smooth muscle actin and epithelial membrane antigen (EMA). Based on clinical, radiological, histological and immunohistochemical findings the final diagnosis was high-grade intra-osseous fibrosarcoma. The treatment choice was radical surgery with mandibular reconstruction. After one year and nine months of the treatment the patient displayed multiple metastases. Radiation therapy and chemotherapy were used as adjuvant treatment. Unfortunately, the girl died two years after initial diagnosis.
纤维肉瘤是一种由成纤维细胞构成的恶性间叶性肿瘤,很少累及口腔,可导致局部复发或转移。在本文中,我们描述了一名16岁女孩下颌骨原发性纤维肉瘤的病例。显微镜下,肿瘤显示梭形细胞强烈增殖,大小和形状变化不大,呈平行束状排列,部分相互交叉,有丝分裂活性增加且存在核异型性。免疫组织化学检查显示,细胞仅对波形蛋白呈免疫反应性,而对S-100蛋白、CD 68、细胞角蛋白混合物、HMB-45、CD34、泛肌动蛋白HHF 35、结蛋白、平滑肌肌动蛋白和上皮膜抗原(EMA)呈阴性。根据临床、放射学、组织学和免疫组织化学检查结果,最终诊断为高级别骨内纤维肉瘤。治疗选择为根治性手术并进行下颌骨重建。治疗一年零九个月后,患者出现多处转移。放疗和化疗用作辅助治疗。不幸的是,该女孩在初次诊断两年后死亡。