婴儿纤维性错构瘤12例临床病理分析

目的 探讨婴儿纤维性错构瘤临床病理学特点及预后。 方法 收集2009—2014年北京儿童医院确诊的婴儿纤维性错构瘤12例，观察其临床及组织病理特点。 结果 本组婴儿纤维性错构瘤12例中男7例，女5例，中位年龄8个月（5 ～ 20个月），中位发病年龄3.5个月（1 ～ 12个月），中位病程5个月（1 ～ 12个月）。皮损表现为肤色至淡红色皮下结节或斑块，其中发生于躯干部5例，四肢6例，阴囊1例；12例中10例皮损为单发，2例为多发；皮损最大径2 ～ 25 cm，其中10例肿物生长迅速，质地软至硬，常首诊或误诊为血管瘤、脂肪瘤或纤维瘤等。9例患儿手术切除无复发，3例患儿术后复发，再次切除后未再复发。组织形态及免疫组化染色，12例肿瘤均可见编织状排列的纤维组织、成熟脂肪组织及同心圆排列的间叶细胞，肿瘤以前两者为主，但比例差异较大；纤维组织表达平滑肌肌动蛋白（SMA），脂肪组织表达S100，间叶细胞表达CD99；间叶细胞可不同程度地向纤维组织和脂肪组织分化。 结论 婴儿纤维性错构瘤多于婴儿期前6个月内起病，为好发于躯干或四肢、生长迅速的孤立性良性软组织肿瘤，其皮损表现多样，具有特征性的组织病理学改变。手术切除效果良好。

Clinicopathological analysis of fibrous hamartoma of infancy: report of 12 cases

Xu Jiaosheng*, Xu Zigang, Zhou Chunju, Han Xiaofeng, Xiao Yuanyuan, Xu Zhe, Zhang Lixin, Ma Lin. *Department of Dermatology, Beijing Children′s Hospital, Capital Medical University, Beijing 100045, China Corresponding author: Ma Lin, Email: bch_maleen@aliyun.com 【Abstract】 Objective To investigate the clinicopathological features and prognosis of fibrous hamartoma of infancy （FHI）. Methods Totally, 12 confirmed cases of FHI were collected from 2009 to 2014 at the Department of Dermatology, Beijing Children′s Hospital. The clinicopathological features of FHI were analyzed. Results Of the 12 patients, 7 were male and 5 were female. The median age of patients was 8 months （range, 5 － 20 months）, with a median age at onset of 3.5 months （range, 1 － 12 months）, a median clinical course of 5 months （range, 1 － 12 months）. Skin lesions were skin-colored or pink subcutaneous nodules or plaques, and were located on the trunk in 5 patients, on the extremities in 6 patients, and on the scrotum in 1 patient. Ten patients had solitary lesions, and two had multiple lesions. The largest diameter of skin lesions varied from 2 to 25 cm. Lesions rapidly progressed in 10 patients, which were soft or hard on palpation, and were usually mistaken for hemangioma, lipoma and fibroma. All the patients were treated by surgical excision. After the operation, relapse occurred in 3 patients, but not in the other 9 patients. After secondary resection, the 3 patients experienced no relapse again. Histopathological examination revealed fibrous tissue and mature fat tissue arranged in an interwoven pattern, as well as mesenchymal cells arranged in concentric circles. The tumor mainly consisted of fibrous tissue and mature fat tissue at varying ratios. Immunohistochemically, the fibrous tissue expressed smooth muscle actin （SMA）, fat tissue expressed S100, and mesenchymal cells expressed CD99. The mesenchymal cells were differentiated into fibrous tissue and fat tissue to different extents. Conclusions FHI often manifests as solitary, rapidly growing benign tumor of soft tissue located on the trunk or extremities, and mostly occurs during the first 6 months of life. The skin manifestations of FHI are usually various, but the pathological changes are characteristic. Surgical resection is usually effective for its treatment.