Retroperitoneal PNET/EWS：one case report and review of the literature

doi:10.3877/cma.j.issn.1674-0793.2009.02.013

Abstract

Abstract:

Objective

To investigate the clinical and histopathologic characteristics of retroperitoneal primitive neuroectodermal tumor with Ewing's sarcoma (PNET/EWS) and study its treatment and prognosis.

Methods

The clinical features,histopathological characteristics,diagnosis,treatment and prognosis of one retrop eritoneal PNET/EWS patient were observed, and relative literatures were reviewed.

Results

The patient was treated with kidney and tumor resection, postoperative chemotherapy was performed in the local hospital. Four months latter,the tumor recurred and was resected,while recurred and was resected again after one year.

Conclusions

Retroperitoneal PNET/EWS is small round-cell tumors.When making diagnisis,it is important to distinguish from other soft tissue sarcoma.Surgical resection is the main therapy measure,but chemotherapy effect is still not affirmed.The tumor is aggressive,local recurrence rate is high,and distant metastasis can always be found.Patients'prognosis is pool.

Key words: Carcinoma,small cell, Primitive neuroectodermal tumor, Ewing's sarcoma, Treatment, Prognosis

Zhan-qiang ZHANG, Chang-hua ZHANG, Jin-ping MA, Han-ping SHI, Wen-hua ZHAN. Retroperitoneal PNET/EWS：one case report and review of the literature[J]. Chinese Archives of General Surgery(Electronic Edition), 2009, 03(02): 39-41.

/ / Recommend

Add to citation manager EndNote|Ris|BibTeX

URL: https://zhptwkxwx.cma-cmc.com.cn/EN/10.3877/cma.j.issn.1674-0793.2009.02.013

https://zhptwkxwx.cma-cmc.com.cn/EN/Y2009/V03/I02/39

References 17

1	Parham DM, Roloson GJ, Feely M, et al. Primary malignant neuroepithelial tumors of the kidney: a clinicopathologic analysis of 146 adult and pediatric cases from the National Wilms' Tumor Study Group Pathology Center.Am J Surg Pathol,2001,25(2):133-146.
2	Lopez-Terrada D. Molecular genetics of small round cell tumors.Semin Diagn Pathol,1996,13(3):242-249.
3	Delattre O, Zucman J, Melot T, et al. The Ewing family of tumors a subgroup of small-round-cell tumors defined by specific chimeric transcripts.N Engl J Med,1994,331(5):294-299.
4	Grier HE. The Ewing family of tumors:Ewing's sarcoma and primitive neuroectodermal tumors. Pediatr Clin North Am, 1997, 44(4): 991-1004.
5	Nikitakis NG, Salama AR, O'Malley BW Jr, et al. Malignant peripheral primitive neuroectodermal tumor-peripheral neuroepithe-lioma of the head and neck: a clinicopathologic study of five cases and review of the literature.Head Neck,2003,25(6):488-498.
6	张卫东,谢传淼,莫运仙,等.外周型原始神经外胚层肿瘤的CT和MR 影像特征.癌症,2007,26(6):643-646.
7	肖勤,顾雅佳,杨天锡.CT、MRI 检查在外周原始神经外胚层肿瘤诊治中的作用.中国癌症杂志,2004,14(4):333-338.
8	Mendenhall WM, Zlotecki RA, Hochwald SN, et al. Retroperitoneal soft tissue sarcoma.Cancer,2005,104(4):669-675.
9	张智弘,范钦和,徐天蓉,等.外周原始神经外胚叶瘤/尤因肉瘤临床病理学.临床与实验病理学杂志,2001,17(4):306-309.
10	何乐健,李佩娟,刘淑荣,等.外周原始神经外胚层瘤和尤文肉瘤的临床病理与免疫组化研究. 诊断病理学杂志, 2000, 7(4):249-251.
11	余永伟,詹镕洲,马大烈,等.原始神经外胚层肿瘤(PNET)1 例光镜、免疫组化和电镜观察及文献复习.临床与实验病理学杂志,1998,14(2):162-164.
12	张智弘.外周原始神经外胚叶瘤和尤因肉瘤的分子病理学新进展.国外医学:生理·病理科学与临床分册,2002,22(2):191-193.
13	Rud NP, Reiman HM, Pritchard DJ, et al. Extraosseous Ewing's sarcoma.A study of 42 cases.Cancer,1989,64(7):1548-1553.
14	Covelli HD, Beekman JF, Kingry RL. Extraskeletal Ewing's sarcoma:prolonged survival with recurrence after operation. South Med J,1980,73(9):1294-1295.
15	Kinsella TJ,Triche TJ,Dickman PS,et al.Extraskeletal Ewing's sarcoma:results of combined modality treatment.J Clin Oncol,1983,1(8):489-495.
16	Martin RC,Brennan MF.Adult soft tissue Ewing sarcoma or primitive neuroectodermal tumors: predictors of survival? Arch Surg, 2003,138(3):281-285.
17	Venkitaraman R,George MK,Ramanan SG,et al.A single institution experience of combined modality management of extra skeletal Ewings sarcoma.World J Surg Oncol,2007,5(3):1-4.

[1]	Huayue Yan, Zixiang Liu, Shaobo Zhou. Research progress of phosphoenolpyruvate carboxykinase-1 in malignant tumors [J]. Chinese Archives of General Surgery(Electronic Edition), 2024, 18(06): 452-456.
[2]	Jie Xu, Yajun Li, Junwei Han. Comparison of the efficacy of radical laparoscopic total gastrectomy in the treatment of overweight gastric cancer with two approaches [J]. Chinese Journal of Operative Procedures of General Surgery(Electronic Edition), 2025, 19(01): 19-22.
[3]	Huazhi Li, Guang Cao, Diangang Liu, Yajing Zhang. Clinical comparison of descending hepatectomy with different approaches for primary hepatocellular carcinoma [J]. Chinese Journal of Operative Procedures of General Surgery(Electronic Edition), 2025, 19(01): 52-55.
[4]	Jiehong Gao, Pingping Li, Jing Qi, Yinhai Dai. Study on the relationship between the expression of ETFA gene and clinicopathological parameters and prognosis in breast cancer [J]. Chinese Journal of Operative Procedures of General Surgery(Electronic Edition), 2025, 19(01): 64-67.
[5]	Daiqin Li, Peijie Liu. Value of dynamic enhanced magnetic resonance imaging in evaluating the efficacy and prognosis of middle and advanced low rectal cancer after concurrent chemoradiotherapy [J]. Chinese Journal of Operative Procedures of General Surgery(Electronic Edition), 2025, 19(01): 100-103.
[6]	Hao Chen, Meng Wang. Advances in clinicopathological features and treatment options of sig-ring cell carcinoma of the stomach [J]. Chinese Journal of Operative Procedures of General Surgery(Electronic Edition), 2025, 19(01): 108-111.
[7]	Zun Chen, Ping Wang, Hua Jin, Meiling Zhou, Qingqing Li, Yonggang Huang. Application of sarcopenia in predicting incisional hernia after colorectal cancer surgery [J]. Chinese Journal of Hernia and Abdominal Wall Surgery(Electronic Edition), 2024, 18(06): 639-644.
[8]	of Organ Transplantation of Chinese Medical Association Branch. Chinese practice guidelines for diagnosis and treatment of ischemic-type biliary lesion after liver transplantation [J]. Chinese Journal of Hepatic Surgery(Electronic Edition), 2024, 13(06): 739-748.
[9]	Anti-Cancer Association China, Committee of Colorectal Cancer CACA the. CACA guidelines for holistic integrative management of anal cancer （2024 Edition） [J]. Chinese Journal of Colorectal Diseases(Electronic Edition), 2024, 13(06): 441-449.
[10]	Jiagang Han, Zhenjun Wang. The treatment strategies for obstructive left-sided colon cancer [J]. Chinese Journal of Colorectal Diseases(Electronic Edition), 2024, 13(06): 450-458.
[11]	Xuefeng Zhou, Zheyi Dong, Zhe Feng, Guangyan Cai, Xiangmei Chen. Formulation protocol of guidelines for diagnosis and treatment of diabetic kidney disease with integrated traditional Chinese and Western medicine [J]. Chinese Journal of Kidney Disease Investigation(Electronic Edition), 2024, 13(06): 301-305.
[12]	Tianyu Wu, Zixuan Liu, Puxin Yang, Siming Jia, Kai Ding, Xiaodong Cheng, Yonglong Li, Wei Chen, Hongzhi Lyu, Qi Zhang. Progress in Conservative Treatment of Lumbar Disc Herniation [J]. Chinese Journal of Geriatric Orthopaedics and Rehabilitation(Electronic Edition), 2024, 10(06): 379-384.
[13]	Yu Liu, Shaobin Duan, Zhixiang Ding, Zhitao Shi. Expression of miR-34a-5p in colon cancer patients and its correlation with clinical characteristics and prognosis [J]. Chinese Journal of Digestion and Medical Imageology(Electronic Edition), 2024, 14(06): 485-490.
[14]	Qianqian Chen, Chen Yuan, Ji Liu, Tingting Yin. Effects of multi-slice spiral CT parameters，carcinoembryonic antigen，mismatch repair gene，and pathological indicators on prognosis of colorectal cancer [J]. Chinese Journal of Digestion and Medical Imageology(Electronic Edition), 2024, 14(06): 507-511.
[15]	Mingfen Zeng, Yan Wang. Study on the correlation between CT and color Doppler ultrasound diagnostic parameters and the condition and prognosis of patients with acute pancreatitis complicated with fatty liver [J]. Chinese Journal of Digestion and Medical Imageology(Electronic Edition), 2024, 14(06): 531-535.

Please choose a citation manager

Content to export