婴幼儿肌纤维瘤病的临床病理学观察

Clinicopathologic study of infantile myofibromatosis

目的 探讨婴幼儿肌纤维瘤病的临床病理特征、诊断及鉴别诊断。方法12例患儿均以无痛性肿块为主要表现，发病的中位数年龄为4．5个月，病变以头颈部、肩背部、腋下及腿等浅表部位多见；手术切除标本采用HE染色及免疫组织化学技术进行病理学观察，并结合临床资料进行分析。结果12例患儿都采用了肿瘤完整切除，术后无放疗、化疗，手术切除标本组织学表现：肿瘤大部分由短梭形的、胞质嗜伊红的细胞构成，排列呈螺旋状或柬状，肿瘤内血管丰富，呈血管外皮瘤样结构，并见部分血管腔内肿瘤生长，局部见坏死和核异型；免疫组化：肿瘤细胞Vim（＋），CD34（＋），SMA（＋），Des（-），S-100蛋白（-）；术后临床平均随访时间约4年，2例出现复发，进行了再次手术，无恶性变发生。结论婴幼儿肌纤维瘤病是少见的先天性疾病，临床上多表现为体表孤立或多发性无痛性结节，病理学形态复杂，鉴别诊断范围广，本病有一定自限性，局部切除可治愈，但有一定的复发率。

Objective To evaluate the clinicopathologic features,diagnosis and differential diagnosis of infantile myofibromatosis （IM）. Methods Painless mass was the major clinical manifestation in all the 12 patients. The median age of the patients was 4. 5 months and the sex ratio of male to female was 1.4： 1. The masses located mostly at the superficial site of patients bodies, such as head, neck,shoulder, back,armpit and leg. All resected tumors were made into paraffin sections and evaluated by hematoxylin and eosin staining and immunohistochemical technique. The histological findings were analyzed together with clinical data. Results En bloc resection of tumor was performed on 12 patients. No radiotherapy or chemotherapy was done postoperatively. The histological findings showed that the tumor tissue was consisted of proliferative plump spindle-shaped cells with eosinophilic en dochylema and arranged in the form of whorls and fascicles; blood vessels were abundant in a hemangiopericytoma-like pattern; growth of neoplastic cells into vascular spaces,nuclear atypia and coagularive necrosis could be observed in some regions. The immunohistochemical staining showed positive immunoreactivity for vimentin,CD34 and smooth muscle actin, while negative for desmin or 8-100. The median year of following up was 4 years and only two children had recurrence and underwent a second operation. There was no evidence of malignancy. Conclusions IM is an uncommon proliferative congenital disorder with clinical manifestation of single or multiple painless nodular at any superficial site of patients＇ body. The histologic features of IM are quite variable. Complete excision of IM is curative.