头颈部侵袭性纤维瘤病(3例报告及文献复习)

Aggressive fibromatosis of the head and neck (A report of three cases and literature review)

目的 探讨一种头颈部少见肿瘤——侵袭性纤维瘤病的临床表现、病理特点、治疗选择及转归。方法 报告3例头颈部侵袭性纤维瘤病的临床资料并复习相关文献。结果 ①侵袭性纤维瘤病是一种纤维增生性良性或低度恶性肿瘤,生物学上呈局部浸润性和破坏性生长;②病理学表现为细胞中心和胶原周围成纤维细胞单克隆增生,由分化良好的成纤维细胞和肌成纤维细胞组成,缺乏恶性的细胞学特征和有丝分裂相,电镜示丰富的胶原网包绕多形态细胞增生,免疫组化波形蛋白和肌动蛋白阳性;③手术是主要治疗措施,多数病例需多次手术,术后复发率可高达70％,常需配合放射治疗和化学治疗;④1例接受3次手术仍复发,另2例手术后随访3～6个月未复发。结论 侵袭性纤维瘤病是一种少见的纤维增生性肿瘤,头颈部少见;以手术治疗为主,有高度复发倾向,但预后较好;对于复发或难以完整切除的病例,常需配合放射治疗和化学治疗。

Objective To determine the clinical manifestation, pathologic behavior, therapy and prognosis of rare aggressive fibromatosis in the head and neck. Methods Three cases of aggressive fibromatosis were analyzed and relevant literatures were reviewed. Results (1) Aggressive fibromatosis was a benign or semimalignant, non-metastatic proliferations of fibrous tissue that infiltrated surrounding tissues and tended to recur after surgical resection. The clinical heterogeneity and misleading histopathological appearances could make the diagnosis difficult or lead to misdiagnosis.(2) Pathology showed fibroblastic monoclonal proliferation between the cellular center and the collagen periphery. Fibromatosis was composed of well-differentiated fibroblasts and myofibroblasts, lacking cytologic features of malignancy and scanty or absent mitotic activity. Electron microscope showed an abundant collagen network enclosing a polymorphous cellular proliferation. Immunohistochemistry defined vimentin and actin positive desmoid tumors. (3) Complete surgical excision was a chief treatment. Several operations were needed in most cases. Recurrence rates may be as high as 70% . Chemotherapy and radiotherapy may be used together with surgery in recurrence or unsatisfactory surgical margin. (4) One patient recurred after three operations, and the other two did not recur after 3-6 months follow-up. Conclusion Aggressive fibromatosis is a rare benign, non-metastatic proliferation tumour of fibrous tissue with a capacity for infiltration of surrounding structures and a tendency of local recurrent after surgical resection. The cornerstone of therapy is surgery. Radiation therapy and chemotherapy can be used as adjuvant therapy in patients with recurrent or unresectable or inoperable disease.