肾盂淋巴上皮瘤样癌伴肺转移一例报告并文献复习

Lymphoepithelioma-like carcinoma of renal pelvis with lung metastasis： one case report with literature review

目的 探讨肾盂淋巴上皮瘤样癌（LELC）的临床表现、病理特点、治疗及预后. 方法 回顾性分析l例肾盂LELC患者的资料.患者,男,61岁,因左侧腰部胀痛2个月于2012年5月入院.患者4个月前因“左肾铸型结石”于我科行经皮肾镜取石术,术中见尿路上皮黏膜颜色质地正常.专科查体：左肾区轻度压痛、叩击痛,余未见明显异常.泌尿系统增强CT检查提示左肾盂癌侵及左输尿管上段,左肾盂扩张积水,左侧肾门及腹膜后多发淋巴结转移;泌尿系超声检查提示左肾重度积水,左肾集合系统重度分离,宽35 mm,左肾盂输尿管移行处实性占位,大小53 mm×38 mm,内回声不均,可见点状血运;胸部CT检查提示双肺散在结节影,大者横径约1.1 cm,考虑肺部多发转移癌,纵隔淋巴结肿大,大者横径约0.7 cm. 结果 临床诊断为左肾盂癌,肺部多发转移癌.于硬膜外麻醉下行左肾根治性切除术.病理诊断为左肾盂LELC,肉眼见肾盂处肿物,大小为4.5 cm×4.0 cm×3.5 cm,灰白质地,局部见坏死,累及肾实质及肾被膜;光镜下见左肾盂正常尿路上皮黏膜消失,代之以浸润性生长,条索状、实性巢状分布的肿瘤细胞,肿瘤细胞异形性大,胞质比例高,泡状核,可见大嗜酸性核仁.患者因经济原因未行相关治疗,随访18个月因多器官功能衰竭死亡. 结论 肾盂LELC临床罕见,症状与肾盂癌相似,影像学检查无特征性改变,确诊须依靠病理学检查,根治性手术是主要治疗手段.

Objective To investigate the clinical manifestations,pathologic features,treatment and prognosis of lymphoepithelioma-like carcinoma （LELC） of renal pelvis.Methods The clinical data of one case with LELC of renal pelvis was reviewed.A 61-year male patient was admitted in our hospital due to the progressive left waist pain on May 2012.He had the history of staghorn stone in left kidney.And the percutaneous nephrolithotomy was performed 4 months before admission.During the procedure,no abnormal urinary mucosa was found.After admission,the physical examination showed mild tenderness and rap pain in the left renal region.Urinary system enhanced CT revealed left renal pelvis cancer,involved the upper segment of left ureter.Left hydronephrosis and multiple lymph nodes metastasis in left renal hilum and retroperitoneum were also demonstrated.The ultrasound revealed severe left hydronephrosis,severe separation of left renal collective system （35 mm width）.An uneven-echo and blood-rich mass,measuring 53 mm×38 mm,was observed at the ureteropelvic junction.Lung CT demonstrated that scattered shadow in whole lung （max diameter was about 1.1 cm）,and longitudinal diaphragmatic lymph node enlargement （max diameter was about 0.7 cm）,which suggested multiple metastases in lung.Results The patient was diagnosed as the carcinoma of renal pelvis with multiple metastasis in lung.Left radical nephrectomy was successfully performed under continuous epidural anesthesia.The pathological diagnosis was LELC of left renal pelvis.In gross view,a uniform pale cream tumor,measuring 4.5 cm×4.0 cm×3.5 cm,was identified in the renal pelvis.Regional necrosis and tumor infiltration into the adjacent renal parenchyma and capsule were seen.Microscopic findings demonstrated that the normal urinary epithelium in left renal pelvis disappeared,instead of infiltrated tumor cells that formed funicular pattern orsyncytial nests with large nuclei and prominent nucleoli.Because of the financial problem,the patient denied to accept the following therapy after the operation.He died due to the multiple organ failure 18 months later.Conclusions LELC of renal pelvis is very rare in clinic.The clinical manifestation is similar to renal pelvis cancer.The image examinations have no distinct characteristic.The diagnosis is based on pathological examination.The prognosis depends on different pathological classification and radical nephrectomy is effective treatment.