Collin Michael, Charles Adrian, Barker Andrew, Khosa Japinder, Samnakay Naeem
Princess Margaret Hospital for Children, Roberts Road, Subiaco, Western Australia, 6008, Australia.
Princess Margaret Hospital for Children, Roberts Road, Subiaco, Western Australia, 6008, Australia; University of Western Australia, Hackett Drive, Nedlands, Western Australia 6009, Australia.
J Pediatr Urol. 2015 Oct;11(5):239-45. doi: 10.1016/j.jpurol.2015.03.009. Epub 2015 Apr 20.
Inflammatory myofibroblastic tumours of the bladder (IMTB) are rare, and feature a benign and reactive proliferation of myofibroblasts. 25% of the reported IMTB cases in the literature occur in children. The present study presents a review of IMTB in children.
The data from 42 reported cases of paediatric IMTB in the world literature are summarised, including two recent cases from the present centre. Paediatric IMTB equally affects males and females. It mainly presents with haematuria, dysuria or abdominal pain. Lesions can vary in size, but mean size is 5.5 cm. Mean age is 7.5 years. The aetiology of IMTB is poorly understood, but includes infective or traumatic aetiologies, or a possible clonal lesion. IMTB may specifically show clonal gene rearrangements involving the anaplastic lymphoma kinase (ALK-1) gene. To differentiate IMTB from rhabdomyosarcoma, tissue diagnosis and careful histological analysis are essential. Tumour biopsy can be achieved by a transurethral approach or a transcutaneous approach with ultrasound guidance. Between 35 and 89% of cases of IMTB express ALK-1 by immunohistochemistry. ALK-1 expression is much less common in other bladder soft tissue tumours. ALK-1 is thus useful in the diagnosis of IMTB. The treatment of choice for IMTB is complete surgical resection of the lesion. In children, no proven recurrent or metastatic IMTB episodes are reported after excision. However IMTB recurrences are reported in adults, likely due to incomplete excision. Follow-up after excision is therefore recommended.
Paediatric IMTB is uncommon. Tissue biopsy is essential for diagnosis. Careful histological assessment is required to differentiate IMTB from malignant paediatric bladder tumours such as rhabdomyosarcoma. ALK-1 expression is useful in confirming the diagnosis of IMTB. Treatment of choice is complete surgical resection of the lesion. Recurrence is reported in adult IMTB. Follow-up is therefore recommended.
膀胱炎性肌纤维母细胞瘤（IMTB）较为罕见，其特征为肌成纤维细胞的良性反应性增生。文献报道的IMTB病例中25%发生于儿童。本研究对儿童IMTB进行综述。
总结了世界文献中42例儿童IMTB的报道数据，包括本中心最近的2例病例。儿童IMTB对男性和女性的影响相同。主要表现为血尿、排尿困难或腹痛。病变大小不一，但平均大小为5.5厘米。平均年龄为7.5岁。IMTB的病因尚不清楚，但包括感染性或创伤性病因，或可能的克隆性病变。IMTB可能特别显示涉及间变性淋巴瘤激酶（ALK-1）基因的克隆性基因重排。为了将IMTB与横纹肌肉瘤区分开来，组织诊断和仔细的组织学分析至关重要。肿瘤活检可通过经尿道途径或在超声引导下经皮途径进行。35%至89%的IMTB病例通过免疫组织化学表达ALK-1。ALK-1表达在其他膀胱软组织肿瘤中不太常见。因此，ALK-1有助于IMTB的诊断。IMTB的首选治疗方法是完整切除病变。在儿童中，切除术后未报告有经证实的复发或转移的IMTB病例。然而，成人IMTB有复发报道，可能是由于切除不完全。因此建议切除术后进行随访。
儿童IMTB不常见。组织活检对诊断至关重要。需要仔细的组织学评估以将IMTB与儿童恶性膀胱肿瘤如横纹肌肉瘤区分开来。ALK-1表达有助于确诊IMTB。首选治疗方法是完整切除病变。成人IMTB有复发报道。因此建议进行随访。
