Email Alert | RSS

北京大学学报（医学版） ›› 2024, Vol. 56 ›› Issue (4): 636-639. doi: 10.19723/j.issn.1671-167X.2024.04.015

• 论著 • 上一篇    下一篇

6例肾尤文肉瘤伴静脉瘤栓的诊治

王滨帅1,邱敏1,张前进2,田茂锋3,刘磊1,王国良1,陆敏4,田晓军1,张树栋1,*()   

  1. 1. 北京大学第三医院泌尿外科, 北京 100191
    2. 南京医科大学附属宿迁第一人民医院泌尿外科, 江苏宿迁 223800
    3. 博兴县人民医院泌尿外科, 山东滨州 256500
    4. 北京大学第三医院病理科, 北京 100191
  • 收稿日期:2024-03-13 出版日期:2024-08-18 发布日期:2024-07-23
  • 通讯作者: 张树栋 E-mail:zhangshudong@bjmu.edu.cn
  • 基金资助:
    北京大学第三医院临床重点项目(BYSY2022059);北京大学第三医院创新转化基金(BYSYCY2024046);北京健康促进会项目(A76475)

Experience in diagnosis and treatment of 6 cases of renal Ewing's sarcoma with venous thrombus

Binshuai WANG1,Min QIU1,Qianjin ZHANG2,Maofeng TIAN3,Lei LIU1,Guoliang WANG1,Min LU4,Xiaojun TIAN1,Shudong ZHANG1,*()   

  1. 1. Department of Urology, Peking University Third Hospital, Beijing 100191, China
    2. Department of Urology, The Affiliated Suqian First People's Hospital of Nanjing Medical University, Suqian 223800, Jiangsu, China
    3. Department of Urology, Boxing People's Hospital, Binzhou 256500, Shandong, China
    4. Department of Pathology, Peking University Third Hospital, Beijing 100191, China
  • Received:2024-03-13 Online:2024-08-18 Published:2024-07-23
  • Contact: Shudong ZHANG E-mail:zhangshudong@bjmu.edu.cn
  • Supported by:
    the Peking University Third Hospital Clinical Key Project(BYSY2022059);Peking University Third Hospital Innovation Transformation Fund(BYSYCY2024046);Beijing Health Promotion Association Project(A76475)

RICH HTML

   

PDF (PC)

摘要：

目的: 回顾和分析肾尤文肉瘤伴静脉瘤栓患者的临床诊治过程，随访患者的生存预后，为疾病的诊治提供帮助。方法: 收集2016年6月至2022年6月在北京大学第三医院诊断为肾尤文肉瘤伴静脉瘤栓患者的临床资料，包括一般资料、手术资料和术后病理资料，电话随访患者预后情况，分析诊治过程对疾病预后的影响。结果: 患者6例，其中男性1例、女性5例；左肾肿物4例，右肾肿物2例；确诊时中位年龄28岁(16~52岁)；影像学表现均为外生性肿瘤，内部存在坏死组织及出血；平均肿瘤最大直径为12.6 cm，平均瘤栓长度为7.8 cm；4例行开放手术，2例行腹腔镜手术；术后病理结果均为肾尤文肉瘤，免疫组织化学结果中3例CD99(+)，2例FLI-1(+)，1例CD99、FLI-1(-)；术后接受化疗(环磷酰胺、多柔比星、长春新碱/异环磷酰胺、依托泊苷方案)3例，化疗联合放疗1例，未行放化疗2例；术后6例平均总体生存率(overall survival，OS)为37个月，接受化疗的4例患者平均术后OS(47个月)显著高于未行化疗的2例患者(16个月，P=0.031)。结论: 肾尤文肉瘤伴静脉瘤栓临床罕见，年轻女性患者多发，手术难度大，预后不良，手术切除加辅助放化疗可提高患者OS。

关键词: 肾肿瘤, 尤文肉瘤, 静脉瘤栓

Abstract:

Objective: To review and analyze the clinical diagnosis and treatment of renal Ewing's sarcoma with venous tumor embolus, to follow up the survival and prognosis of the patients, and to provide help for the diagnosis and treatment of the disease. Methods: Clinical data (including general data, surgical data and postoperative pathological data) of patients diagnosed with renal Ewing's sarcoma with venous tumor embolus in Peking University Third Hospital from June 2016 to June 2022 were collected, and the prognosis of the patients was followed up to analyze the influence of diagnosis and treatment process on the prognosis of the disease. Results: There were 6 patients, including 1 male and 5 females. There were 4 cases of left renal tumor and 2 cases of right renal tumor. The median age at diagnosis was 28 years (16-52 years). The imaging findings were all exogenous tumors with internal necrotic tissue and hemorrhage. The mean maximum tumor diameter was 12.6 cm, and the mean tumor thrombus length was 7.8 cm. Four patients underwent open surgery and 2 patients underwent laparoscopic surgery. The postoperative pathological results were renal Ewing sarcoma. Immunohistochemical results showed 3 cases of CD99 (+), 2 cases of FLI-1 (+), and 1 case of CD99, FLI-1 (-). 3 patients received chemotherapy (cyclophosphamide, doxorubicin, vincristine/ifosfamide, etoposide), 1 case received chemotherapy combined with radiotherapy, and 2 cases received no adjuvant therapy. The mean overall survival (OS) of the 6 patients was 37 months, and the mean OS of the 4 patients (47 months) who received chemotherapy was significantly higher than that of the 2 patients (16 months) who did not receive chemotherapy (P=0.031). Conclusion: Renal Ewing's sarcoma with venous tumor embolus is rare in clinic, and it is common in young female patients. The operation is difficult and the prognosis is poor. Surgical resection, adjuvant radiotherapy and chemotherapy can improve the overall survival rate of the patients.

Key words: Renal tumor, Ewing's sarcoma, Venous tumor thrombus

中图分类号: 

  • R737.1

表1

6例肾尤文肉瘤伴静脉瘤栓患者的一般临床资料"

Items Patient 1 Patient 2 Patient 3 Patient 4 Patient 5 Patient 6
Age/years 18 16 39 28 29 52
Gender Female Female Female Female Female Male
Clinical symptom Gross hematuria Lumbago Lumbago Gross hematuria Lumbago Gross hematuria
Imageological examination Wilms’ tumor? Right renal tumor Left renal tumor Left renal cancer Left renal tumor Left renal tumor
Tumor metastasis Retroperitoneal lymph node metastasis PET-CT: none metastasis Adrenal invasion PET-CT: none metastasis PET-CT: adrenal invasion Retroperitoneal lymph node metastasis
Maximum tumor diameter/cm 8.1 14.2 12.4 9.6 17.8 13.5
Maximum tumor embolus diameter/cm 6.6 9.6 8.0 3.5 13.5 5.6
Clinical stage T3bN1M0 T3bN0M0 T4N0M0 T3bN0M0 T4N0M0 T3bN1M0
Pathological stage pT3bN1 pT3bN0 pT3bN0 pT3bN0 pT3bN0 pT3bN0
Immunohistochemistry CD99(-), FLI-1(-) CD99(-), FLI-1(+) CD99(+), FLI-1(-) CD99(-), FLI-1(+) CD99(+), FLI-1(-) CD99(+), FLI-1(-)

图1

肾尤文肉瘤伴静脉瘤栓患者的典型影像学表现"

图2

6例肾尤文肉瘤伴静脉瘤栓患者的生存曲线"

表2

6例肾尤文肉瘤伴静脉瘤栓患者术后及随访资料"

Items Patient 1 Patient 2 Patient 3 Patient 4 Patient 5 Patient 6
Operation Laparoscopic surgery Laparoscopic surgery Open surgery Open surgery Open surgery Open surgery
Adjuvant therapy None Chemotherapy Chemotherapy None Chemotherapy+radiotherapy Chemotherapy
Follow-up result Death after 13 months Osseous metastasis after 6 months No tumor recurrence Death after 19 months Lung metastasis after 26 months No tumor recurrence
1 邓超, 宁刚. 原发肾脏尤文肉瘤2例并文献复习[J]. 中国CT和MRI杂志, 2022, 20 (6): 180- 182.
2 Coffin CM , Dehner LP . Peripheral neurogenic tumors of the soft tissues in children and adolescents: A clinicopathologic study of 139 cases[J]. Pediatr Pathol, 1989, 9 (4): 387- 407.
doi: 10.3109/15513818909022361
3 Venkitaraman R , George MK , Ramanan SG , et al. A single institution experience of combined modality management of extra skeletal Ewings sarcoma[J]. World J Surg Oncol, 2007, 5, 3.
doi: 10.1186/1477-7819-5-3
4 Narayanan G , Rajan V , Preethi TR . Primitive neuroectodermal tumors of the kidney[J]. Proc (Bayl Univ Med Cent), 2017, 30 (2): 205- 208.
5 何建风, 丁国恒, 孙红军, 等. 肾脏尤文肉瘤或原始神经外胚层肿瘤1例报告并文献复习[J]. 实用放射学杂志, 2019, 35 (9): 1548- 1549.
6 Murphey MD , Senchak LT , Mambalam PK , et al. From the radiologic pathology archives: Ewing sarcoma family of tumors: Radiologic-pathologic correlation[J]. Radiographics, 2013, 33 (3): 803- 831.
doi: 10.1148/rg.333135005
7 Dogra PN , Goel A , Kumar R , et al. Extraosseous Ewing's sarcoma of the kidney[J]. Urol Int, 2002, 69 (2): 150- 152.
doi: 10.1159/000065566
8 Thyavihally YB , Tongaonkar HB , Gupta S , et al. Primitive neuroectodermal tumor of the kidney: A single institute series of 16 patients[J]. Urology, 2008, 71 (2): 292- 296.
doi: 10.1016/j.urology.2007.09.051
9 Celli R , Cai G . Ewing sarcoma/primitive neuroectodermal tumor of the kidney: A rare and lethal entity[J]. Arch Pathol Lab Med, 2016, 140 (3): 281- 285.
doi: 10.5858/arpa.2014-0367-RS
10 黄健, 张旭, 魏强, 等. 2022版中国泌尿外科和男科疾病诊断治疗指南[M]. 北京: 人民卫生出版社, 2022: 4- 5.
11 毕文浩, 俞能旺, 蒋立城. 肾脏原发性尤文肉瘤1例[J]. 山东大学学报(医学版), 2022, 60 (10): 117- 119.
12 Risi E , Iacovelli R , Altavilla A , et al. Clinical and pathological features of primary neuroectodermal tumor/Ewing sarcoma of the kidney[J]. Urology, 2013, 82 (2): 382- 386.
doi: 10.1016/j.urology.2013.04.015
13 魏晋艳, 刘静妮, 张学凌, 等. 肾脏尤文肉瘤/原始神经外胚层肿瘤与乳头状肾细胞癌的CT征象比较[J]. 中国医学影像学杂志, 2021, 29 (9): 935- 939.
14 郑琪, 侯宇川. 肾门尤文肉瘤1例报道并文献复习[J]. 重庆医学, 2020, 49 (3): 515- 516.
15 冯瑶杰, 瞿姣, 危春容, 等. 骨外尤文肉瘤/外周原始神经外胚层肿瘤的CT及MRI表现[J]. 放射学实践, 2020, 35 (7): 900- 904.
16 Babapour S , Mohseni I , Piri R , et al. Left renal Ewing's sarcoma: A case study and a review of imaging literature[J]. Radiol Case Rep, 2020, 15 (4): 391- 395.
doi: 10.1016/j.radcr.2020.01.010
17 Llombart-Bosch A , Machado I , Navarro S , et al. Histological heterogeneity of Ewing's sarcoma/PNET: An immunohistochemical analysis of 415 genetically confirmed cases with clinical support[J]. Virchows Arch, 2009, 455 (5): 397- 411.
doi: 10.1007/s00428-009-0842-7
18 Tarek N , Said R , Andersen CR , et al. Primary Ewing sarcoma/primitive neuroectodermal tumor of the kidney: The MD Anderson Cancer Center experience[J]. Cancers (Basel), 2020, 12 (10): 2927.
doi: 10.3390/cancers12102927
19 阳晶, 施鸿金, 李宁, 等. 肾尤文肉瘤/原始神经外胚层肿瘤的诊断及治疗(附1例分析)[J]. 山东医药, 2022, 62 (6): 49- 51.
20 毕海, 黄毅, 马潞林, 等. 3例肾尤文肉瘤合并下腔静脉癌栓的诊治[J]. 北京大学学报(医学版), 2020, 52 (5): 985- 989.
21 Ellinger J , Bastian PJ , Hauser S , et al. Primitive neuroectodermal tumor: Rare, highly aggressive differential diagnosis in urologic malignancies[J]. Urology, 2006, 68 (2): 257- 262.
doi: 10.1016/j.urology.2006.02.037
[1] 张树栋,谢睿扬. 机器人手术时代的肾癌合并腔静脉瘤栓治疗策略[J]. 北京大学学报（医学版）, 2024, 56(4): 562-564.
[2] 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.
[3] 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.
[4] 邱敏,宗有龙,王滨帅,杨斌,徐楚潇,孙争辉,陆敏,赵磊,卢剑,刘承,田晓军,马潞林. 腹腔镜肾部分切除术治疗中高复杂程度肾肿瘤的效果[J]. 北京大学学报（医学版）, 2023, 55(5): 833-837.
[5] 周利群,徐纯如. 机器人时代中央型肾肿瘤的手术治疗策略[J]. 北京大学学报（医学版）, 2022, 54(4): 587-591.
[6] 李新飞, 彭意吉, 余霄腾, 熊盛炜, 程嗣达, 丁光璞, 杨昆霖, 唐琦, 米悦, 吴静云, 张鹏, 谢家馨, 郝瀚, 王鹤, 邱建星, 杨建, 李学松, 周利群. 肾部分切除术前CT三维可视化评估标准的初步探究[J]. 北京大学学报（医学版）, 2021, 53(3): 613-622.
[7] 邱敏,费月阳,邓绍晖,刘承,卢剑,何为,陆敏,田晓军,张树栋,马潞林. 后肾腺瘤的诊治经验及文献回顾[J]. 北京大学学报（医学版）, 2021, 53(2): 417-419.
[8] 毕海,黄毅,马潞林,陆敏,张树栋,张洪宪. 3例肾尤文肉瘤合并下腔静脉癌栓的诊治[J]. 北京大学学报（医学版）, 2020, 52(5): 985-989.
[9] 纪翔,王义,周哲,赵子臣,果宏峰,王刚,张志宏,晋连超,孙国锋,赵文锋,汪磊,贺利军,李宁忱,那彦群. 后腹腔镜超声引导下微波消融治疗复杂肾肿瘤[J]. 北京大学学报（医学版）, 2020, 52(4): 785-789.
[10] 李丽伟,刘茁,王国良,张华,陈文,马静,张丽,何为,马潞林,王淑敏. 肾癌伴下腔静脉瘤栓合并血栓的多种影像学比较[J]. 北京大学学报（医学版）, 2019, 51(4): 678-683.
[11] 田晓军,邱敏,刘茁,肖若陶,黄毅,王国良,侯小飞,张树栋,庄申榕,马潞林. 微创手术治疗肾癌合并Mayo 0~2级静脉癌栓的单中心研究[J]. 北京大学学报（医学版）, 2018, 50(6): 1053-1056.
[12] 徐奔，张喆楠，罗程，宋海峰，张骞. 后腹腔镜下肿瘤吸除术与肾部分切除术治疗肾血管平滑肌脂肪瘤的安全性与有效性对比[J]. 北京大学学报(医学版), 2018, 50(4): 700-704.
[13] 黄子雄，张晓鹏，董森，刘士军，杨荣利，周宇石，马伟国. 肾黏液性小管和梭形细胞癌合并骨转移1例及文献回顾[J]. 北京大学学报(医学版), 2018, 50(4): 732-735.
[14] 马伟国，秦彩朋，于路平，张晓鹏，刘士军，王永顺， 白文俊，徐涛. 后腹腔镜下孤立肾采用肾动脉非阻断法行保留肾单位肾肿瘤剜除术的疗效评价[J]. 北京大学学报(医学版), 2018, 50(4): 759-761.
[15] 叶剑飞，马潞林，赵磊，王国良. 腔静脉节段切除术在处理侵犯腔静脉的肾肿瘤瘤栓中的应用[J]. 北京大学学报(医学版), 2018, 50(1): 183-187.
Viewed
Full text


Abstract

Cited
  Shared   
  Discussed   
No Suggested Reading articles found!
ISSN 1671-167X
CN 11-4691/R
主管单位：中华人民共和国教育部
主办单位：北京大学
地址： 北京市海淀区学院路38号 北京大学医学部 《北京大学学报（医学版）》
邮编：100191
电话：010-82801551
Email： xbbjb2@bjmu.edu.cn

《北京大学学报（医学版）》
微信公众号
版权所有 © 2018 《北京大学学报（医学版）》编辑部