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子宫炎性肌纤维母细胞瘤:儿童病例报告及文献综述

Inflammatory Myofibroblastic Tumors in the Uterus: Childhood-Case Report and Review of the Literature.

作者信息

Etlinger Péter, Kuthi Levente, Kovács Tamás

机构信息

University of Szeged, Szeged, Hungary.

出版信息

Front Pediatr. 2020 Feb 14;8:36. doi: 10.3389/fped.2020.00036. eCollection 2020.

Abstract

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.

摘要

炎性肌纤维母细胞瘤(IMT)是一种具有低恶性潜能的梭形细胞瘤,可出现在身体的不同部位。子宫部位的IMT较为罕见,尤其是在儿童中。病因尚不清楚,尽管一些作者认为可能存在潜在的创伤或病变。一名3.5岁女童因反复阴道出血在我院接受治疗,无外伤史或已知病理情况。体格检查和实验室分析未发现特异性结果,增强磁共振成像(MRI)发现子宫壁有一个大小为25×28×30mm的不均匀软组织肿块,遂将其完整切除。组织学检查显示为梭形细胞形态,荧光原位杂交(FISH)检测显示ALK基因重排,该病变被定义为IMT。迄今为止已发表了6例病例,其诊断方法并不明确,相比MRI,CT和PET-CT更受青睐。鉴于低复发率和转移发生率,积极的治疗似乎有些过度,克唑替尼在这些病例中被证明是一种有效的治疗药物。活检和组织学检查对于将IMT与恶性肿瘤区分开来具有重要作用,同时需要进行FISH检查,因为ALK阳性可强化诊断。儿童中尚未有致死病例报道,我们的患者在3年后也无症状。

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