肾黏液小管状和梭形细胞癌的临床病理特点及预后分析

Clinicopathological characteristics and prognosis analysis of mucinous tubular and spindle cell carcinoma of the kidney

目的探讨肾黏液小管状和梭形细胞癌(MTSCC)的临床病理特征及预后。方法回顾性分析2013年7月至2022年7月郑州大学第一附属医院收治的16例MTSCC患者的临床资料。男6例,女10例;年龄(56.4±11.4)岁。无症状10例,血尿2例,腰疼3例,下肢疼痛1例。12例术前行增强CT检查,6例行超声检查,3例行MRI检查,1例行骨扫描检查。影像学表现:肿物为圆形或类圆形,有明显边界;其中2例合并出血,3例合并钙化;5例表现为外生性生长,10例部分外生性,1例完全内生性;肿瘤最大径为(65.7±27.4)mm。病变位于左肾11例,右肾5例。增强CT检查示肿瘤为轻度延迟强化;MRI检查示肿瘤呈长/短T1信号混杂长/短T2信号,DWI弥散受限高信号;超声检查肿瘤表现为低回声包块,无明显血流信号。术前诊断:12例为肾占位性病变,2例为乏血供肾肿瘤,1例为肾肿瘤破裂出血;1例骨扫描提示胸椎、骨盆转移,诊断为转移性肾肿瘤,行肾穿刺术明确诊断。11例行腹腔镜根治性肾切除术,其中2例术后分别予培唑帕尼、舒尼替尼辅助治疗;4例行肾部分切除术;1例转移未行手术,予甲磺酸阿帕替尼、唑来膦酸治疗。结果术后病理标本可见肿物呈灰白、灰黄或灰红色,质软或质中。未见肾周、输尿管及肾上腺侵犯。镜下可见肿瘤细胞为圆形、卵圆形;瘤细胞排列成管状、条索状,局部可见黏液池;所有肿瘤均未见肉瘤样成分。16例均诊断为肾MTSCC。pT1N0M0期9例,pT2N0M0期6例,pT1N0M1期1例。所有患者均获得随访,随访时间50.7(25.8,75.0)个月,15例无复发转移;1例穿刺前转移患者,因肿瘤进展多发肺、骨转移而死亡,生存期为16.9个月。结论肾MTSCC罕见,多为体检发现,女性居多,影像学检查表现为乏血供肿瘤;手术是最主要的治疗方法,可根据肿瘤分期、肾功能及患者基础病情选择行肾部分切除术或根治性肾切除术,术后患者预后良好。

Objective To explore the clinicopathological features and prognosis of renal mucinous tubular spindle cell carcinoma(MTSCC).Methods The clinical data of 16 patients with MTSCC admitted to the First Affiliated Hospital of Zhengzhou University from July 2013 to July 2022 were retrospectively analyzed.There were six male cases and ten female cases.The mean age was(56.4±11.4)years old.Among them,10 cases were asymptomatic,two complained of hematuria,three complained of lumbar pain,and one complained of lower limb pain.Twelve cases underwent preoperative enhanced CT examination,6 cases of ultrasound examination,3 cases of MRI examination,and 1 case of bone scan.Imaging manifestations showed that the masses were round or round-like with clear borders.Two cases combined with hemorrhage and three cases combined with calcification.Five cases showed exophytic growth,10 cases partially exophytic,and 1 case completely endophytic.The maximum diameter of the tumor was(65.7±27.4)mm.The tumors were located in the left kidney in 11 cases and in the right kidney in 5 cases.The tumors were mildly delayed-enhancing under enhanced CT,long/short T1 signal mixed with long/short T2 signal under MRI,and diffusion-limited high signal under DWI.The tumors were hypoechoic masses without obvious blood flow signals under ultrasound.Twelve cases were diagnosed as renal occupying neoplasms,2 cases were suggested as lack of blood supply renal tumor,and one was considered renal tumor rupture and bleeding.In one case,a bone scan suggested metastasis to the thoracic spine and pelvis.The metastatic renal tumor was diagnosed,and a renal puncture was performed to clarify the pathology.Eleven patients underwent laparoscopic radical nephrectomy,and 4 patients underwent partial nephrectomy.One case was metastasized without surgery and treated with apatinib mesylate and zoledronic acid.Results The postoperative pathological specimens showed grayish,grayish-yellow,or grayish-red masses with a soft or medium texture.No perinephric,ureteral,or adrenal invasion was seen in all tumors.Microscopically,the tumor cells were round and ovoid.The tumor cells were arranged in tubular and striated shapes,and mucus pools were locally visible.No sarcomatous component was seen in all tumors.There were 9 patients with pT1N0M0,6 patients with pT2N0M0,and 1 patient with pT1N0M1.After operation,2 patients with pT2N0M0,who underwent laparoscopic radical nephrectomy were treated with pazopanib and sunitinib,respectively.All patients were followed up for a median of 50.7(25.8,75.0)months,15 patients were free of recurrent metastases,and 1 patient with pre-puncture metastasis died due to tumor progression of multiple pulmonary and bone metastases,with a survival of 16.9 months.Conclusions Renal MTSCC is rare,mostly found on physical examination,with female patients predominantly,and imaging shows a lack of blood supply tumor.Surgery is the primary treatment method.Partial nephrectomy or radical nephrectomy could be chosen according to the tumor stage,kidney function,and patient's underlying condition,and patients have a good prognosis.