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.