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Journal of Peking University (Health Sciences) ›› 2024, Vol. 56 ›› Issue (4): 636-639. doi: 10.19723/j.issn.1671-167X.2024.04.015

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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)

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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

CLC Number: 

  • R737.1

Table 1

Clinical data of 6 cases of renal Ewing's sarcoma with venous tumor embolus"

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(-)

Figure 1

Imaging findings of renal Ewing's sarcoma with venous thrombus A, CT urography findings; B, MRI findings."

Figure 2

Postoperative survival curve of 6 patients with renal Ewing's sarcoma with venous thrombus"

Table 2

Postoperative and follow-up data of 6 patients with renal Ewing's sarcoma with venous tumor embolus"

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
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