上颌窦炎性肌纤维母细胞瘤1例并文献复习

Inflammatory myofibroblastic tumor in the maxillary sinus:a case report and review of literature

目的:探讨炎性肌纤维母细胞瘤的临床特征、诊断与治疗方法。方法:报告1例上颌窦炎性肌纤维母细胞瘤,并复习相关的国内外的文献。结果:炎性肌纤维母细胞中,CT扫描示不规则的软组织密度影,有侵蚀性的骨质破坏,边界不清。病理示瘤组织由梭形纤维细胞及大量炎性细胞组成。免疫组织化学检测中Vimentin、SMA呈阳性,Desmin等呈阴性。结论:上颌窦的炎性肌纤维母细胞瘤是非常罕见的,诊断主要依靠病理及免疫组织化学检测。炎性肌纤维母细胞瘤中存在染色体的易位,这种易位导致间变性淋巴瘤激酶的过度表达,而呈现一种肿瘤的发展过程,其生物学特性仍然没有统一的说法。

To investigate the clinical feature, diagnostic and therapeutic methods of inflammatory myofibroblastic tumor（IMT）. Method：One case of IMT was reported and the relevant literatures were reviewed. Result：The computed tomography scan showed irregular soft tissue density shade and aggressive bone destruction with unclear boundary. Pathological findings showed variable numbers of inflammatory cells and myofibroblastic spindle cells. Tumor cells were immunoreactive for vimentin and smooth muscle actin, but negative for desmin et al. Conclusion： IMT of the maxillary sinus is very rare. The diagnosis of IMT base on histopathology and immuno- histochemistry. The genesis and development of IMT result from chromosomal translocations that often cause an overexpression of anaplastie lymphoma kinase. IMT are clinical and pathological distinct entities and its biological behavior is still uncertain.