婴儿纤维性错构瘤临床病理分析

Fibrous hamartoma of infant:a clinicopathological study of 13 cases

目的探讨婴儿纤维性错构瘤的临床病理特征及预后。方法对13例婴儿纤维性错构瘤的临床资料、病理切片和随访结果进行分析。结果13例中,男性患儿11例(84.62%),发现肿块时平均年龄(6±4.9)个月,手术切除时平均年龄(14.1±9.6)个月。病损为皮下肿块,平均直径(3.6±1.7)cm,胸背部、头颈部为好发部位。镜下见错构瘤由梭形纤维成分、成熟脂肪组织及未成熟间叶细胞混合组成器官样结构。手术切除后局部复发率低。结论婴儿纤维性错构瘤需与婴儿纤维瘤病、弥漫性肌纤维瘤病、钙化性腱膜纤维瘤、神经纤维瘤、脂肪母细胞瘤、纤维脂肪瘤及纤维肉瘤、胚胎性横纹肌肉瘤等鉴别。手术切除是唯一的治疗方法。

Purpose To investigate the clinical, pathological features and prognosis of fibrous hamartoma of infant （FHI）. Methods The clinical data, pathological sections and prognosis were analyzed in 13 cases of FHI. Results There was a male preponderance. The median of patients was （6±4.9） months when the masses were observed, while it was （14.1±9.6） months when the masses were excised. The lesions were subcutaneous mass, and the median size was （3.6±1.7）cm in diameter. The chest and back, head and neck were the most common sites. The microscopical feature was typical, spindle shaped fibrous components, mature adipose tissue and immature mesenchymal cells formed an organoid pattern. The local recurrence rate was low after excision. Conclusions The differential diagnoses of the tumor include infantile fibromatosis, diffuse myofibromatosis, calcifying aponeurotic fibroma, neurofibroma, lipoblastoma, fibrolipoma, infantile fibrosarcoma, and embryonal rhabdomyosarcoma. The lesion does not spontaneously regress. Surgical excision is only option of the treatment.