Inflammatory myofibroblastic tumor (IMT) is a spindle cell neoplasm with low malignant potential, which may appear in different parts of the body. Uterine localization is rare, especially among children. Etiology is unclear, although some authors suggest underlying trauma or distress. A 3.5-year-old girl was treated at our institute for recurring vaginal bleeding without injury or known pathology. Physical examination and laboratory analysis revealed no specific findings, contrast-enhanced MRI found a 25 × 28 × 30 mm-sized inhomogeneous soft tissue mass in the uterus wall, which was excised in toto. Histological examination identified a spindle cell pattern, and the FISH test revealed ALK gene rearrangement, the lesion was defined as an IMT. Six cases were published to date, and their diagnostic methods are not equivocal, CT, and PET CT were preferred instead of MRI. Aggressive therapy seems to be exaggerated according to low recurrence and metastasis occurrence, and crizotinib is proved as good therapeutic agent in those cases. Biopsy and histology has important role in order to distinguish IMT from malignancies completed with FISH examination because ALK positivity strengthens the diagnosis. No lethal outcome was published among children, as our patient is also symptom-free after 3 years.

译文

炎症性肌纤维母细胞瘤 (IMT) 是一种具有低恶性潜能的梭形细胞肿瘤,可能出现在身体的不同部位。子宫定位很少见,尤其是在儿童中。病因尚不清楚,尽管一些作者建议潜在的创伤或困扰。一名3.5岁的女孩在我们研究所接受了复发性阴道出血的治疗,没有受伤或已知的病理。体格检查和实验室检查未发现具体发现,增强MRI在子宫壁中发现25 × 28 × 30毫米大小的不均匀软组织肿块,并在toto中切除。组织学检查确定了梭形细胞模式,FISH测试显示ALK基因重排,病变被定义为IMT。迄今为止已发表了6例病例,其诊断方法并不模棱两可,首选CT和PET CT而不是MRI。根据低复发和转移发生率,积极的治疗似乎被夸大了,在这些情况下,克唑替尼被证明是良好的治疗剂。活检和组织学对于区分IMT和FISH检查完成的恶性肿瘤具有重要作用,因为ALK阳性可增强诊断。在儿童中没有发表致命的结果,因为我们的患者在3年后也没有症状。

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