肾脏黏液性小管状和梭形细胞癌三例报道并文献复习

Mucinous tubular and spindle cell carcinoma:a report of 3 cases and literature review

目的探讨肾脏黏液性小管状和梭形细胞癌(mucinous tubular and spindle cell carcinoma,MTSCC)的临床病理学特点。方法对3例MTSCC进行光镜、特殊染色、免疫组织化学染色及荧光原位杂交(FISH)检测,并复习临床资料及相关文献。结果 3例MTSCC中,2例为男性,1例女性,年龄分别为50、71、75岁(中位71岁),例2临床表现为腰痛,3例均无肉眼血尿。肿瘤长径分别为2.0cm、3.5cm、6.0cm(中位3.5cm),肿瘤切面灰白色,与周围肾实质界限清晰,例2局部伴出血、坏死。组织学上肿瘤由温和一致的立方细胞紧密排列成狭长的小管结构及梭形细胞两种成分构成,例1、例3部分肿瘤细胞胞质透明,例1黏液性间质稀少;例2、例3间质内见泡沫细胞聚集。免疫表型:3例均表达AMCAR、CK7、CK19、EMA、NSE等,Ki-67低于5%。FISH结果:3例均无乳头状肾细胞癌的染色体异常:3、7、17染色体扩增及Y染色体丢失。结论MTSCC为低级别肾细胞癌,形态学谱系广泛,免疫表型表达远曲小管上皮标记(AMCAR、CK7、CK19、EMA、NSE等),主要需与乳头状肾细胞癌相鉴别,一般预后较好,术后仍须密切随访。

Objective To investigate the clinicopathological characteristics of mucinous tubular and spindle cell carcinoma （M TSCC） .Methods Three cases of M TSCC were studied by light microscopy ,spe-cial stains ,immunohistochemistry ,fluorescence in situ hybrisation （FISH） and literature review .Results The ages of the M TSCC patients range from 51 to 75 years （mean 71 years） .Case 2 had the clinical mani-festation of flank pain .No case had hematuria .Tumor sizes were 2 .0cm-6 .0cm （mean 3 .5cm） ,exhibiting a well-circumscribed ,regular ,grayish-white cut surface .Case 2 had hemorrhage and necrosis .Histologi-cal examination of the tumor showed that it consisted of cuboidal and oval or spindle cells arranged in tubu-lar and trabecular patterns .The foci of case 1 and 3 were seen in clear cells ,myxoid stroma in case 1 was not easy to see ,and macrophages were seen in the myxomatous stroma of cases 2 and 3 .M TSCC tumors exhibited positive reactions to AMCAR ,vimentin ,EMA ,CK7 ,CK19 ,NSE and other markers ,and the MIB-1 labeling index was generally very low .Fluorescence in situ hybridization did not show gains of 3 ,7 , 17 chromosomes and loss of Y chromosome of in all 3 cases .Conclusion M TSCC is low grade renal cell car-cinoma with a wide histological spectrum .Immunohistochemically ,the tumor cells are positive for proxi-mal convoluted tubules and distal convoluted tubules immunophone （AMACR ,EM A ,CK7 ,CK19 ,NSE et al） and need to be differentiated with papillary renal cell carcinoma of the Kidney .With generally good prognosis ,it still need follow-up .