肾黏液样小管状和梭形细胞癌的临床病理特点及预后

Mucinous tubular and spindle cell carcinoma of kidney:Clinicopathology and prognosis

目的:探讨肾黏液样小管状和梭形细胞癌的临床病理学特征、免疫表型、鉴别诊断及预后。方法:回顾性分析13例肾黏液样小管状和梭形细胞癌病例,总结其临床和病理学特点以及免疫组织化学表达情况,并进行荧光原位杂交检测。结果:13例患者年龄39~78岁,平均57.1岁,其中男性4例,女性9例,男女比例为1∶2.25,均无临床症状,为偶然发现。3例行肾部分切除术,10例行肾根治切除术;9例肿瘤位于左侧肾,4例位于右侧肾,最大径2~12 cm。大部分病例镜下形态为经典型形态改变,细胞核分级采用世界卫生组织(World Health Organization,WHO)/国际泌尿病理协会(International Society of Urological Pathology,ISUP)分级系统,11例为G2,2例为G3。病理分期PT1a共6例,PT1b共3例,PT2a共2例,PT2b及PT3a各1例。免疫组织化学染色阳性率:波形蛋白(vimentin)、AE1/AE3、α-甲基脂酰辅酶A消旋酶(α-methylacyl-CoA racemase,αMACR)、细胞角蛋白(cytokeratin,CK)8/18均为100%(13/13),CK7为92.3%(12/13),上皮细胞膜抗原(epithelial membrane antigen,EMA)为92.3%(12/13),CK20为46.2%(6/13),CD10为30.8%(4/13),突触素(synaptophysin,Syn)为7.7%(1/13),嗜铬素(chromogranin A,CgA)、CD57、WT1、Ki-67均为0(0/13)。荧光原位杂交结果显示所有病例均未见7、17号染色体多倍体改变。术后随访6个月至7年6个月,2例出现肺转移后死亡(1例核分级为G3,1例伴有坏死),其余11例无复发和转移。结论:肾黏液样小管状和梭形细胞癌是一种低度恶性的独特类型肾肿瘤,好发于女性,年龄分布广泛。目前治疗方法为手术切除,伴有坏死及高级别形态的病例易发生复发和转移,虽然大部分病例预后良好,但术后仍需密切随访。

Objective:To investigate and summarize the clinicopathological features,immunophenotype,differential diagnosis and prognosis analysis of mucinous tubular and spindle cell carcinoma(MTSCC).Methods:The data of thirteen cases of MTSCC were retrospectively analyzed,the clinical and pathological characteristics and immunohistochemical expression were summarized,and fluorescence in situ hybridization was detected.Results:Among the thirteen patients,four were males and nine females,with a male-to-female ratio of 1∶2.25.The average age was 57.1 years,ranging from 39 to 78 years.The maximum diameter of the tumor was 2-12 cm.All cases had no symptoms,and were accidentally discovered,3 cases underwent partial renal resection,10 cases underwent radical renal resection,9 cases were located in the left kidney,and 4 cases were located in the right kidney.Most of the cases showed the classical morphological changes,with 11 cases of nuclear grading[World Health Organization(WHO)/International Society of Urological Pathology(ISUP)grading system]being G2 and 2 cases being G3.There were 6 cases of stage PT1a,3 cases of PT1b,2 cases of PT2a,and 1 case of PT2b and 1 case of PT3a.The positive rates of immunohistochemical staining were:vimentin,AE1/AE3,α-methylacyl-CoA racemase(αMACR)and cytokeratin(CK)8/18,100%(13/13);CK7,92.3%(12/13);epithelial membrane antigen(EMA),92.3%(12/13);CK20,46.2%(6/13);CD10,30.8%(4/13);synaptophysin(Syn),7.7%(1/13);chromogranin A(CgA),CD57,WT1 and Ki-67,0(0/13),and fluorescence in situ hybridization showed that no trisomy of chromosomes 7 and 17 were observed in any of the cases.The follow-up period was 6 months to 7 years and 6 months,2 cases died after lung metastasis(one with ISUP/WHO grade G3,one with necrosis),and the remaining 11 cases had no recurrence and metastasis.Conclusion:MTSCC is a unique type of low-grade malignancy kidney tumor,occurs predominantly in females,widely distributed in age,the current treatment method is surgical resection,and cases with necrosis and high-grade morphology are prone to recurrence and metastasis,although most cases have a good prognosis,but they still need close follow-up after surgery.