摘要
目的探讨巨大婴儿纤维性错构瘤的临床病理特点、诊断和鉴别诊断。方法对1例巨大婴儿纤维性错构瘤的临床和病理特征进行观察并复习文献。结果患儿因发现左下肢巨大肿物5个月后入院。大体见肿物位于皮下,大部由灰黄脂肪组织构成,其内穿插不规则灰白质韧区;镜下肿瘤由富含胶原的纤维组织、原始间叶细胞岛及成熟脂肪组织组成;免疫组化染色成熟脂肪细胞S-100(+);肌纤维母细胞SMA(+);原始间叶细胞及部分肌纤维母细胞CD99(+);8%瘤细胞Ki-67(+),且阳性细胞主要是原始间叶细胞。结论巨大婴儿纤维性错构瘤是一种更为少见的发生于皮下的良性肿瘤,也具有特征性病理表现,但其常因手术不易切净而有复发风险,故应密切随访。
Objective To discuss the clinicopathologic features and diagnosis and differential diagnosis of fibrous hamartoma of infancy. Methods A case of giant fibrous hamartoma of infancy was analyzed with pathological morphology and immunohistochemical staining and review of the related litera- ture. Results The infant was admitted to the hospital because of giant tumor left lower limb for 5 months. On pathologic examination, the tumor located in subcutaneous, most tan by adipose tissue composition, which thrust deep into the gray area of irregular toughness. On light microcopy,the tumor appeared organoid pattern, consisted of cross beam shape arranged fibrous tissue, immature mesenchymal cells island and mature adipose tissue. The adipose cells strongly expressed S - 100, myofibroblast coexpressed SMA and CD99, the immature mesenchymal cells moderately expressed CD99. No more than 8%cells expressed Ki - 67, and most of which were original mesenchymal cells. Conclusion Giant fibrous hamartoma of infancy has characteristics in clinic and pathology. It is a more rare occurs in subcutaneous benign a soft tissue tumor. However, it is not easily cut completely by one surgery and then it is reoccurred. It＇s necessary to follow-up.
出处
《中国病案》
2012年第12期48-50,共3页
Chinese Medical Record
关键词
婴儿纤维性错构瘤
免疫组化
临床病理
Fibrous hamartoma of infancy
lmmunohistochemistry
Clinical pathology
