Department of Orthopaedics, Children's Hospital, Medical College, Zhejiang University, Hangzhou, China.
J Pediatr Hematol Oncol. 2020 Nov;42(8):495-498. doi: 10.1097/MPH.0000000000001603.
Infantile myofibromatiosis (IM) is a rare benign tumor in the infants, but it has a bad prognosis if IM erncroaches on the viscera. Multiple tissues can be invaded by IM, including the subcutaneous tissue, the muscle of the neck, back, and head, but seldom in the bones and the viscera. The histopathologic and immunohistochemical examinations are necessary in daigonosis of IM as it might be misdiagnosed as the malignant tumor.
Thirty-two consecutive patients with IM in our hospital (2003-2013) were enrolled and the clinical date were analyzed to understand IM better, such as the feature of clinical manifestations, pathology, imaging tests, and treatment.
All of them underwent excision operations, 4 of them with invasion in the bones, 2 with invasion in the craniums, and the rest in the ulna and the humerus. The immunohistiochemical analysis shown that the tumor cells were positive to vimentin and smooth muscle actin while negative to the S100 protein and desmin. Twenty-five patients were in follow-up, 2 cases recurred.
IM is a benign tumor, but IM with the viscera involvement has a bad prognosis. The strategy of waiting and observation for IM without visceral involvement could be selected.
婴儿肌纤维瘤病（IM）是一种罕见的婴儿良性肿瘤，但如果 IM 侵犯内脏，则预后不良。IM 可侵犯多种组织，包括皮下组织、颈部、背部和头部的肌肉，但很少侵犯骨骼和内脏。由于 IM 可能被误诊为恶性肿瘤，因此组织病理学和免疫组织化学检查对 IM 的诊断是必要的。
我们医院（2003-2013 年）连续收治了 32 例 IM 患者，分析了这些患者的临床资料，以更好地了解 IM，如临床表现、病理、影像学检查和治疗的特点。
所有患者均行切除术，4 例有骨侵犯，2 例有颅骨侵犯，其余 25 例发生在尺骨和肱骨。免疫组化分析显示肿瘤细胞阳性表达波形蛋白和平滑肌肌动蛋白，而 S100 蛋白和结蛋白阴性。25 例患者获得随访，2 例复发。
IM 是一种良性肿瘤，但有内脏侵犯的 IM 预后不良。对于无内脏侵犯的 IM，可以选择等待和观察的策略。
