肾脏炎性肌纤维母细胞瘤临床及影像学分析并文献复习

Clinical and radiographic study on renal inflammtory myofibroblastic tumor and review of literature

目的探讨肾脏炎性肌纤维母细胞瘤(IMT)的病理学、影像学特征及治疗和预后情况,以提高对该病的诊断水平。方法报告1例确诊为肾脏IMT的诊治资料,结合文献对其临床及影像资料进行回顾性分析。结果该例肾脏IMT患者的影像学表现、病理检查及临床表现与转归有一定特征性:(1)CT平扫呈等密度,边界欠清晰,增强扫描皮质期及实质期均呈轻到中度强化,且强化不均,排泄期呈带状环形强化,肿块邻近脂肪囊密度增高,肾筋膜明显增厚,增厚的包膜较广泛;PET/CT检查示肿块FDG代谢增高,肾外其他部位未见明确肿瘤转移征象。(2)镜下见肿瘤内梭形细胞分布,并见大量淋巴细胞及浆细胞浸润;免疫组织化学检测显示,肿瘤细胞平滑肌特异性肌动蛋白弥漫(+)、结蛋白局灶(+)。(3)临床上无血尿表现,随访未见病灶复发或扩散。结论肾脏IMT少见,容易误诊,熟悉其临床及影像学特征表现对IMT正确诊断及治疗有重要价值;肾脏IMT临床预后较好。

Objective To explore clinical pathological features,radiographic findings,treatment and prognosis of renal inflammtory myofibroblastic tumor（IMT）,and to improve the diagnosis of the disease. Methods The clinical data of one case of renal IMT were reported.The radiographic features of the patient with histology-proven were retrospectively analyzed and the relevant literature was reviewed. Results There were some distinctive findings of renal IMT：（1） On non-enhanced CT,the imaging features included isodense attenuation,round or irregular contour,and its margin with the renal parenchyma was ill defined.On contrast-enhanced CT,the lesion showed heterogeneously slight-to-moderate contrast enhancement at artery and vein phase,and thick-rim contrast enhancement at delayed imaging.The density of adipose capsule adjacent to mass increased.Renal fascia showed marked thickening,and the thickness of the coating was more extensive.18F-FDG PET/CT scan demonstrated a hypermetabolic mass featuring markedly increased 18F-FDG uptake.No clear signs of metastasis were found in extrarenal other parts.（2）Histological examination demonstrated the tumor composed of spindle-shaped fibroblastic cells and infiltrating variable amount of lymphocytes and plasma cells.Immunohistochemically,the tumor cells were positive diffusely for SMA and partially positive for desmin.（3） The patient often appear without hematuria.Follow-up showed no recurrence and metastasis of the tumor. Conclusion Renal IMT is a rare pathologic entity easily misdiagnosed.Radiographic findings combined with clinical features have important value in making correct diagnosis and treatment of IMT preoperatively.The renal IMT has a favorable prognosis.