肾炎性肌纤维母细胞瘤临床病理观察

Renal inflammatory myofibroblastic tumor:a clinicopathological observation

目的探讨肾炎性肌纤维母细胞瘤的临床病理学特征。方法报道1例罕见的肾炎性肌纤维母细胞瘤,结合文献对其临床表现、组织形态、免疫组化特点及治疗和预后等进行分析。结果患者女性,41岁。左侧腰部间歇性胀痛1月余,CT示"左肾占位",行左侧肾切除术。大体见中上极肾实质内有一3 cm×3 cm×2 cm大小灰白色质韧肿瘤,无包膜,与周围界限欠清。镜下见肿瘤主要由梭形细胞组成,梭形细胞呈束状交错排列,伴有大量慢性炎细胞浸润,以淋巴细胞和浆细胞为主,局部见较多泡沫细胞。免疫表型SMA和Vim(+)。随访半年未见复发。结论肾炎性肌纤维母细胞瘤是一种非常罕见的肿瘤,临床表现以腰痛和/或血尿为主要症状,确诊需依靠病理检查及免疫组化。鉴别诊断包括肾的肉瘤样癌、Wilms瘤以及腹膜后炎性纤维肉瘤、恶性纤维组织细胞瘤、纤维瘤病等。手术切除预后良好。

Objective To investigate the clinicopathological characteristics of renal inflammatory myofibroblastic tumor （IMT）. Methods One case of renal IMT was reported with review of the literature. The clinical presentation, morphological appearance, immunohistochemical features, therapy and prognosis were analyzed in this tumor. Results A 41-year-old female was admitted with the complaint of left flank pain. Computerized tomography revealed a mass in the upper pole of the left kidney. Left radical nephrectomy was performed under the diagnosis of renal tumor. Histological examination demonstrated a tumor composed of spindle-shaped fibroblastic cells infiltrated by variable numbers of plasma cells, small lymphocytes and histiocytes, and immunostaining showed positive expression of vimentin and smooth muscle acfin in tumor cells. The pathologic diagnosis was renal IMT. Follow-up was available with no evidence of recurrence in six months after surgery. Conclusions Renal IMT is a very rare proliferative lesion of myofibroblasts. Flank pain and/or hematuria are main symptoms. The diagnosis should he made based on pathological examination. It must he differentiated from other renal lesions, including sarcomatoid carcinoma, Wilms tumor, inflammatory fibrosarcoma, malignant fibrohistocytoma and so on. Surgery may he the best treatment resulting in good prognosis.