Wang Sun, Ma Qichao, Ying Hao, Jiao Qin, Yang Dan, Zhang Bin, Zhao Lihua
Department of Orthopedics, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China.
Department of Pathology, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China.
Medicine (Baltimore). 2020 Mar;99(11):e19489. doi: 10.1097/MD.0000000000019489.
Fibrous hamartoma of infancy (FHI) is a rare benign soft-tissue tumor in children with a triphasic organoid histologic appearance.
We here report a case with the largest FHI published so far. A 13-month-old boy with a rapidly growing tumor 45 cm in length and 69.3 cm in circumference of the left lower extremity was identified immediately at birth.
The diagnosis was uncertain before the operation, although biopsy was conducted. The postoperation histological examination showed arranged spindle cells, adipose tissue, and nests of immature small cells embedded in a myxoid background, which is the characteristic triphasic histology of FHI.
Under general anesthesia, hip and lower extremity amputation was performed.
The patient was followed-up for 6 years and no signs of recurrence were found, suggesting a favorable prognosis, although a part of the residual tumor was present after the surgery.
FHI exhibits pathological and clinical characteristics. However, differential diagnosis of FHI from other soft-tissue tumors before operation remains a challenge. Thus, sometimes, aggressive therapy for the treatment of FHI might be a good choice to get a satisfactory.
婴儿纤维性错构瘤（FHI）是一种罕见的儿童良性软组织肿瘤，具有三相类器官组织学表现。
我们在此报告一例迄今已发表的最大的FHI病例。一名13个月大的男婴出生时即被发现左下肢有一个迅速生长的肿瘤，长45厘米，周长69.3厘米。
尽管进行了活检，但术前诊断仍不明确。术后组织学检查显示有梭形细胞排列、脂肪组织以及嵌入黏液样背景中的未成熟小细胞巢，这是FHI典型的三相组织学特征。
在全身麻醉下进行了髋关节和下肢截肢手术。
对该患者进行了6年随访，未发现复发迹象，尽管术后仍有部分残留肿瘤，但提示预后良好。
FHI具有病理和临床特征。然而，术前将FHI与其他软组织肿瘤进行鉴别诊断仍然是一项挑战。因此，有时积极治疗FHI可能是获得满意疗效的一个不错选择。
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