Department of Urology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Department of Pathology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Urology. 2020 Nov;145:229-235. doi: 10.1016/j.urology.2020.07.050. Epub 2020 Aug 8.
To present the clinicopathological characteristics and outcome of children with bladder and ureteral inflammatory myofibroblastic tumors (IMTs) in our center.
We reviewed the medical records of patients with bladder and ureteral IMTs from 2010 to 2018. We recorded patients' demographic data, presentation, hemoglobin level, presence of hydronephrosis, tumor size, treatment, and outcomes.
Eight patients with bladder IMTs and 3 with ureteral IMTs were treated at our center during this period. The mean age was 7.1 years. Four patients presented with anemia at diagnosis with the mean hemoglobin level 84.5 g/L. Among patients with bladder IMTs, 5 were male and 3 were female. The most common symptom was lower urinary symptoms in 6 patients, followed by hematuria in 4 patients. 2 patients had complications of hydronephrosis and hydroureter. Among patients with ureteral IMTs, 2 were male and one was female. The most common symptom was abdominal pain, and 3 patients presented with upper urinary tract dilation. All patients underwent surgery. A total of 81.8% were positive for anaplastic lymphoma kinase. Cytokeratin (CK) expression was present in all patients with bladder IMTs, while it was negative in 2 patients with ureteral IMTs. During mean follow-up of 43.4 months, all patients survived event-free.
The presence of hydronephrosis and hydroureter is rare in patients with bladder IMTs. Anemia caused by hematuria should be raised the index of suspicion for IMTs. Children with bladder and ureteral IMTs had excellent prognosis. The expression pattern of CK varied between bladder and ureteral IMTs.
介绍本中心儿童膀胱和输尿管炎性肌纤维母细胞瘤（IMT）的临床病理特征和转归。
我们回顾了 2010 年至 2018 年期间在本中心接受治疗的膀胱和输尿管 IMT 患者的病历。记录了患者的人口统计学数据、表现、血红蛋白水平、是否存在肾积水、肿瘤大小、治疗和结果。
在此期间，本中心治疗了 8 例膀胱 IMT 患者和 3 例输尿管 IMT 患者。平均年龄为 7.1 岁。4 例患者在诊断时出现贫血，平均血红蛋白水平为 84.5g/L。在膀胱 IMT 患者中，男性 5 例，女性 3 例。最常见的症状是 6 例患者出现下尿路症状，其次是 4 例患者出现血尿。2 例患者并发肾积水和输尿管积水。在输尿管 IMT 患者中，男性 2 例，女性 1 例。最常见的症状是腹痛，3 例患者出现上尿路扩张。所有患者均接受手术治疗。ALK 阳性率为 81.8%。所有膀胱 IMT 患者均表达细胞角蛋白（CK），而 2 例输尿管 IMT 患者 CK 表达为阴性。在平均 43.4 个月的随访中，所有患者均无事件生存。
膀胱 IMT 患者罕见出现肾积水和输尿管积水。由血尿引起的贫血应提高对 IMT 的警惕。膀胱和输尿管 IMT 患儿的预后良好。CK 的表达模式在膀胱和输尿管 IMT 之间存在差异。
