Abstract:
Nodular fasciitis (NF) is a benign myofibroblastic proliferation characterized by rapid growth, a self-limiting course, and USP6 gene rearrangement. Although it can arise in the head and neck region, very few cases of NF involving the sinonasal tract have been reported to date. Herein we report a case of NF involving the nasal cavity presenting as a polypoid well-defined mass causing obstructive symptoms in a male with a history of multiple local surgeries. Although the mass had an unusual clinical presentation, the histopathologic and immunohistochemical findings were consistent with NF. Fluorescent in situ hybridization performed with break-apart probes flanking the USP6 locus on chromosome 17p13 revealed the presence of USP6 gene rearrangement. The patient remained free of disease 124 months after surgical treatment. Considering its remarkably benign behavior despite its alarming clinical and histologic features, the distinction of NF from sinonasal malignant tumors is essential.
摘要:
结节性筋膜炎(NF)是一种良性的肌纤维母细胞增殖,其特征是快速生长,自我限制的课程,和USP6基因重排。虽然它可以出现在头部和颈部区域,迄今为止,很少有涉及鼻窦的NF病例报道。在此,我们报告了一例涉及鼻腔的NF的病例,该病例表现为息肉样明确的肿块,引起患有多次局部手术史的男性的阻塞性症状。尽管肿块有不寻常的临床表现,组织病理学和免疫组织化学结果与NF一致.使用染色体17p13上USP6基因座侧翼的分裂探针进行的荧光原位杂交显示存在USP6基因重排。手术治疗后124个月,患者仍无疾病。尽管具有惊人的临床和组织学特征,但考虑到其显着的良性行为,NF与鼻窦恶性肿瘤的区别至关重要。