肾黏液样小管状和梭形细胞癌的临床病理特点及预后

doi:10.19723/j.issn.1671-167X.2023.02.011

摘要/Abstract

摘要：

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。

关键词: 肾细胞癌, 黏液腺癌, 临床病理学, 免疫组织化学, 预后

Abstract:

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.

Key words: Renal cell carcinoma, Mucinous adenocarcinoma, Clinical pathology, Immunohistoche-mistry, Prognosis

中图分类号:

R737.11

沈棋,刘亿骁,何群. 肾黏液样小管状和梭形细胞癌的临床病理特点及预后[J]. 北京大学学报（医学版）, 2023, 55(2): 276-282.

Qi SHEN,Yi-xiao LIU,Qun HE. Mucinous tubular and spindle cell carcinoma of kidney: Clinicopathology and prognosis[J]. Journal of Peking University (Health Sciences), 2023, 55(2): 276-282.

图/表 5

表1

图1

表2

图2

图3

参考文献 32

1	Eble JN, Sauter G, Epstein JI, et al. World Health Organization classification of tumours: Pathology and genetics of tumours of the urinary system and male genital organs[R]. Lyon, France: International Agency for Research on Cancer, 2004.
2	Moch H, Humphrey P, Ulbright T, et al. World Health Organization classification of tumours of the urinary system and male genital organs[R]. Lyon, France: International Agency for Research on Cancer, 2016.
3	MacLennan GT , Farrow GM , Bostwick DG . Low-grade collecting duct carcinoma of the kidney: Report of 13 cases of low-grade mucinous tubulocystic renal carcinoma of possible collecting duct origin[J]. Urology, 1997, 50 (5): 679- 684. doi: 10.1016/S0090-4295(97)00335-X
4	Ged Y , Chen YB , Knezevic A , et al. Mucinous tubular and spindle-cell carcinoma of the kidney: Clinical features, genomic profiles, and treatment outcomes[J]. Clin Genitourin Cancer, 2019, 17 (4): 268- 274. doi: 10.1016/j.clgc.2019.04.006
5	Xu X , Zhong J , Zhou X , et al. Mucinous tubular and spindle cell carcinoma of the kidney: A study of clinical, imaging features and treatment outcomes[J]. Front Oncol, 2022, 12, 865263. doi: 10.3389/fonc.2022.865263
6	Ferlicot S , Allory Y , Comperat E , et al. Mucinous tubular and spindle cell carcinoma: A report of 15 cases and a review of the literature[J]. Virchows Arch, 2005, 447 (6): 978- 983. doi: 10.1007/s00428-005-0036-x
7	Rakozy C , Schmahl GE , Bogner S , et al. Low-grade tubular-mucinous renal neoplasms: Morphologic, immunohistochemical, and genetic features[J]. Mod Pathol, 2002, 15 (11): 1162- 1171. doi: 10.1097/01.MP.0000031709.40712.46
8	Nathany S , Monappa V . Mucinous tubular and spindle cell carcinoma: A review of histopathology and clinical and prognostic implications[J]. Arch Pathol Lab Med, 2020, 144 (1): 115- 118. doi: 10.5858/arpa.2017-0506-RS
9	Xiao L , Xiao W , Guo Y , et al. Huge mucinous tubular and spindle cell carcinoma of the kidney: A case report[J]. Urol Case Rep, 2021, 40, 101914.
10	Bajpai M , Pooja S , Tyagi M , et al. Mucinous spindle and tubular renal cell cancer: A rare variant of renal cell cancer[J]. J Cancer Res Ther, 2022, 18 (4): 1168- 1170. doi: 10.4103/jcrt.jcrt_99_21
11	Kuroda N , Hes O , Michal M , et al. Mucinous tubular and spindle cell carcinoma with Fuhrman nuclear grade 3: A histological, immunohistochemical, ultrastructural and FISH study[J]. Histol Histopathol, 2008, 23 (12): 1517- 1523.
12	Fuchizawa H , Kijima T , Takada-Owada A , et al. Metastatic mucinous tubular and spindle cell carcinoma of the kidney responding to nivolumab plus ipilimumab[J]. IJU Case Rep, 2021, 4 (5): 333- 337. doi: 10.1002/iju5.12342
13	Arafah M , Zaidi SN . Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid transformation[J]. Saudi J Kidney Dis Transplant, 2013, 24 (3): 557- 560. doi: 10.4103/1319-2442.111066
14	Bulimbasic S , Ljubanovic D , Sima R , et al. Aggressive high-grade mucinous tubular and spindle cell carcinoma[J]. Hum Pathol, 2009, 40 (6): 906- 907. doi: 10.1016/j.humpath.2009.03.004
15	Pillay N , Ramdial PK , Cooper K , et al. Mucinous tubular and spindle cell carcinoma with aggressive histomorphology: A sarco-matoid variant[J]. Hum Pathol, 2008, 39 (6): 966- 969. doi: 10.1016/j.humpath.2007.10.006
16	Simon RA , di Sant'agnese PA , Palapattu GS , et al. Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid differentiation[J]. Int J Clin Exp Pathol, 2008, 1 (2): 180- 184.
17	Dhillon J , Amin MB , Selbs E , et al. Mucinous tubular and spindle cell carcinoma of the kidney with sarcomatoid change[J]. Am J Surg Pathol, 2009, 33 (1): 44- 49. doi: 10.1097/PAS.0b013e3181829ed5
18	Fine SW , Argani P , DeMarzo AM , et al. Expanding the histologic spectrum of mucinous tubular and spindle cell carcinoma of the kidney[J]. Am J Surg Pathol, 2006, 30, 1554- 1560. doi: 10.1097/01.pas.0000213271.15221.e3
19	Reuter VE , Argani P , Zhou M , et al. Best practices recommendations in the application of immunohistochemistry in the kidney tumors: Report from the International Society of Urologic Pathology[J]. Am J Surg Pathol, 2014, 38 (8): e35- e49. doi: 10.1097/PAS.0000000000000258
20	Paner GP , Srigley JR , Radhakrishnan A , et al. Immunohistochemical analysis of mucinous tubular and spindle cell carcinoma and papillary renal cell carcinoma of the kidney: Significant immunophenotypic overlap warrants diagnostic caution[J]. Am J Surg Pathol, 2006, 30 (1): 13- 19. doi: 10.1097/01.pas.0000180443.94645.50
21	Uchida S , Suzuki K , Uno M , et al. Mucin-poor and aggressive mucinous tubular and spindle cell carcinoma of the kidney: Two case reports[J]. Mol Clin Oncol, 2017, 7 (5): 777- 782. doi: 10.3892/mco.2017.1400
22	Kuroda N , Nakamura S , Miyazaki E , et al. Low-grade tubular-mucinous renal neoplasm with neuroendocrine differentiation: A histological, immunohistochemical and ultrastructural study[J]. Pathol Int, 2004, 54 (3): 201- 207. doi: 10.1111/j.1440-1827.2004.01608.x
23	Peckova K , Martinek P , Sperga M , et al. Mucinous spindle and tubular renal cell carcinoma: Analysis of chromosomal aberration pattern of low-grade, high grade, and an overlapping morphologic variant with papillary renal[J]. Ann Diagn Pathol, 2015, 19 (4): 226- 231. doi: 10.1016/j.anndiagpath.2015.04.004
24	Kuroda N , Naroda T , Tamura M , et al. High-grade mucinous tubular and spindle cell carcinoma: Comparative genomic hybridization study[J]. Ann Diagn Pathol, 2011, 15 (6): 472- 475. doi: 10.1016/j.anndiagpath.2010.08.003
25	Sadimin ET , Chen YB , Wang L , et al. Chromosomal abnormalities of high-grade mucinous tubular and spindle cell carcinoma of the kidney[J]. Histopathology, 2017, 71 (5): 719- 724. doi: 10.1111/his.13298
26	Mehra R , Vats P , Cieslik M , et al. Bi-allelic alteration and dysregulation of the Hippo pathway in mucinous tubular and spindle cell carcinoma of the kidney[J]. Cancer Discov, 2016, 6 (11): 1258- 1266. doi: 10.1158/2159-8290.CD-16-0267
27	Ren Q , Wang L , Al-Ahmadie HA , et al. Distinct genomic copy number alterations distinguish mucinous tubular and spindle cell carcinoma of the kidney from papillary renal cell carcinoma with overlapping histologic features[J]. Am J Surg Pathol, 2018, 42 (6): 767- 777. doi: 10.1097/PAS.0000000000001038
28	邹子归, 王玉红, 周晋星, 等. 肾黏液样小管状和梭形细胞癌临床病理分析及全外显子组测序分析[J]. 中华病理学杂志, 2021, 50 (7): 762- 767. doi: 10.3760/cma.j.cn112151-20200922-00731
29	Xu H , Li W , Zhu C , et al. Proteomic profiling identifies novel diagnostic biomarkers and molecular subtypes for mucinous tubular and spindle cell carcinoma of the kidney[J]. J Pathol, 2022, 257 (1): 53- 67. doi: 10.1002/path.5869
30	Bharti JN , Choudhary GR , Madduri VKS , et al. Mucinous tubular and spindle cell carcinoma: A difficult diagnosis[J]. Urol Ann, 2021, 13 (2): 180- 182. doi: 10.4103/UA.UA_44_20
31	Thway K , du Parcq J , Larkin JM , et al. Metastatic renal muci-nous tubular and spindle cell carcinoma. Atypical behavior of a rare, morphologically bland tumor[J]. Ann Diagn Pathol, 2012, 16 (5): 407- 410. doi: 10.1016/j.anndiagpath.2011.04.001
32	Cossu-Rocca P , Eble JN , Delahunt B , et al. Renal mucinous tubular and spindle carcinoma lacks the gains of chromosomes 7 and 17 and losses of chromosome Y that are prevalent in papillary renal cell carcinoma[J]. Mod Pathol, 2006, 19 (4): 488- 493. doi: 10.1038/modpathol.3800565

Metrics

Viewed

Full text

Abstract

Cited

Shared

Discussed

Case no.	Gender	Age/years	Location	Nephrectomy	Tumor size/cm	WHO/ISUP nuclear grade	Necorsis	T stage	Follow-up/months	Status
1	F	51	Right	Total	7.6	2	No	2a	90	NED
2	F	77	Left	Total	2.0	2	Yes	1a	15	Dead (lung metastasis)
3	F	56	Left	Partial	2.5	2	No	1a	70	NED
4	M	69	Left	Total	5.5	3	No	3a	6	Dead (lung metastasis)
5	M	50	Left	Total	6.0	2	No	1b	54	NED
6	F	56	Right	Total	7.3	2	No	2a	50	NED
7	F	51	Left	Total	6.8	2	No	1b	38	NED
8	M	54	Left	Partial	3.5	2	No	1a	27	NED
9	F	78	Left	Total	2.5	3	No	1a	20	NED
10	F	50	Right	Total	12.0	2	No	2b	14	NED
11	M	67	Left	Total	3.6	2	No	1a	8	NED
12	F	39	Right	Partial	2.2	2	No	1a	8	NED
13	F	44	Left	Total	6.0	2	No	1b	6	NED

[1]	欧俊永,倪坤明,马潞林,王国良,颜野,杨斌,李庚午,宋昊东,陆敏,叶剑飞,张树栋. 肌层浸润性膀胱癌合并中高危前列腺癌患者的预后因素[J]. 北京大学学报（医学版）, 2024, 56(4): 582-588.
[2]	刘帅,刘磊,刘茁,张帆,马潞林,田晓军,侯小飞,王国良,赵磊,张树栋. 伴静脉癌栓的肾上腺皮质癌的临床治疗及预后[J]. 北京大学学报（医学版）, 2024, 56(4): 624-630.
[3]	虞乐,邓绍晖,张帆,颜野,叶剑飞,张树栋. 具有低度恶性潜能的多房囊性肾肿瘤的临床病理特征及预后[J]. 北京大学学报（医学版）, 2024, 56(4): 661-666.
[4]	周泽臻,邓绍晖,颜野,张帆,郝一昌,葛力源,张洪宪,王国良,张树栋. 非转移性T_3a肾细胞癌患者3年肿瘤特异性生存期预测[J]. 北京大学学报（医学版）, 2024, 56(4): 673-679.
[5]	方杨毅,李强,黄志高,陆敏,洪锴,张树栋. 睾丸鞘膜高分化乳头状间皮肿瘤1例[J]. 北京大学学报（医学版）, 2024, 56(4): 741-744.
[6]	曾媛媛,谢云,陈道南,王瑞兰. 脓毒症患者发生正常甲状腺性病态综合征的相关因素[J]. 北京大学学报（医学版）, 2024, 56(3): 526-532.
[7]	苏俊琪,王晓颖,孙志强. 舌鳞状细胞癌根治性切除术后患者预后预测列线图的构建与验证[J]. 北京大学学报（医学版）, 2024, 56(1): 120-130.
[8]	李建斌,吕梦娜,池强,彭一琳,刘鹏程,吴锐. 干燥综合征患者发生重症新型冠状病毒肺炎的早期预测[J]. 北京大学学报（医学版）, 2023, 55(6): 1007-1012.
[9]	刘欢锐,彭祥,李森林,苟欣. 基于HER-2相关基因构建风险模型用于膀胱癌生存预后评估[J]. 北京大学学报（医学版）, 2023, 55(5): 793-801.
[10]	薛子璇,唐世英,邱敏,刘承,田晓军,陆敏,董靖晗,马潞林,张树栋. 青年肾肿瘤伴瘤栓的临床病理特征及预后分析[J]. 北京大学学报（医学版）, 2023, 55(5): 802-811.
[11]	卢汉,张建运,杨榕,徐乐,李庆祥,郭玉兴,郭传瑸. 下颌牙龈鳞状细胞癌患者预后的影响因素[J]. 北京大学学报（医学版）, 2023, 55(4): 702-707.
[12]	时云飞,王豪杰,刘卫平,米岚,龙孟平,刘雁飞,赖玉梅,周立新,刁新婷,李向红. 血管免疫母细胞性T细胞淋巴瘤临床与分子病理学特征分析[J]. 北京大学学报（医学版）, 2023, 55(3): 521-529.
[13]	许云屹,苏征征,郑林茂,张孟尼,谭珺娅,杨亚蓝,张梦鑫,徐苗,陈铌,陈雪芹,周桥. 转录通读环状RNA rt-circ-HS促进低氧诱导因子1α表达和肾癌细胞增殖与侵袭[J]. 北京大学学报（医学版）, 2023, 55(2): 217-227.
[14]	朱晓娟,张虹,张爽,李东,李鑫,徐玲,李挺. 人表皮生长因子受体2低表达乳腺癌的临床病理学特征及预后[J]. 北京大学学报（医学版）, 2023, 55(2): 243-253.
[15]	赖玉梅,李忠武,李欢,吴艳,时云飞,周立新,楼雨彤,崔传亮. 68例肛管直肠黏膜黑色素瘤临床病理特征及预后[J]. 北京大学学报（医学版）, 2023, 55(2): 262-269.