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Abstract：
BACKGROUND: This study aimed to assess the impact that the quality of primary and subsequent surgeries has on the survival of patients with para-testicular rhabdomyosarcoma (PTRMS).
METHODS: Patients with localized (IRS I-III) and metastatic (IRS IV) PTRMS were enrolled in the two Cooperative Weichteilsarkom Studiengruppe (CWS) trials (CWS-96, CWS-2002P) and the Soft Tissue Sarcoma Registry (SoTiSaR).
RESULTS: Among 196 patients (median age, 8.4 years), 106 (54.1%) had primary complete resection. Image-defined lymph node (LN) disease was detected in 21 (11.5%) patients in the localized cohort and 12 (92.3%) patients in the metastatic cohort. The 5-year event-free survival (EFS) and overall survival (OS) were respectively 87.3% and 94.0% for the patients with localized PTRMS and 46.2% and 42.2% for the patients with metastatic PTRMS. Protocol violations during the primary surgery (PV-PS) were observed in 70 (42%) of the IRS I-III patients. This resulted in higher rates of R1/R2 resections (n = 53 [76%] vs n = 20 [21%]; p < 0.001) with a need for pretreatment re-excision (PRE) (n = 50 [83%] vs n = 10 [17%]; p < 0.001) compared with the patients undergoing correct primary surgery. Protocol violations during PRE occurred for 13 (20%) patients. Although PV-PS did not influence the 5-year EFS or OS in the localized PTRMS cohort, the unadjusted log-rank test showed that R status after PRE is a prognostic factor for 5-year OS (R1 vs R0 [81.8% vs 97.6%]; p = 0.02).
CONCLUSIONS: The quality of surgical local control in PTRMS is unsatisfactory. Emphasis should be placed on evaluating the resection status after PRE in further clinical trials.
METHODS: Patients with localized (IRS I-III) and metastatic (IRS IV) PTRMS were enrolled in the two Cooperative Weichteilsarkom Studiengruppe (CWS) trials (CWS-96, CWS-2002P) and the Soft Tissue Sarcoma Registry (SoTiSaR).
RESULTS: Among 196 patients (median age, 8.4 years), 106 (54.1%) had primary complete resection. Image-defined lymph node (LN) disease was detected in 21 (11.5%) patients in the localized cohort and 12 (92.3%) patients in the metastatic cohort. The 5-year event-free survival (EFS) and overall survival (OS) were respectively 87.3% and 94.0% for the patients with localized PTRMS and 46.2% and 42.2% for the patients with metastatic PTRMS. Protocol violations during the primary surgery (PV-PS) were observed in 70 (42%) of the IRS I-III patients. This resulted in higher rates of R1/R2 resections (n = 53 [76%] vs n = 20 [21%]; p < 0.001) with a need for pretreatment re-excision (PRE) (n = 50 [83%] vs n = 10 [17%]; p < 0.001) compared with the patients undergoing correct primary surgery. Protocol violations during PRE occurred for 13 (20%) patients. Although PV-PS did not influence the 5-year EFS or OS in the localized PTRMS cohort, the unadjusted log-rank test showed that R status after PRE is a prognostic factor for 5-year OS (R1 vs R0 [81.8% vs 97.6%]; p = 0.02).
CONCLUSIONS: The quality of surgical local control in PTRMS is unsatisfactory. Emphasis should be placed on evaluating the resection status after PRE in further clinical trials.
摘要：
背景：本研究旨在评估原发性和后续手术质量对睾丸旁横纹肌肉瘤（PTRMS）患者生存率的影响。
方法：将患有局部（IRSI-III）和转移性（IRSIV）PTRMS的患者纳入了两项WeichteilsarkomStudiengruppe（CWS）合作试验（CWS-96，CWS-2002P）和软组织肉瘤登记处（SoTiSaR）。
结果：在196名患者中（中位年龄，8.4年)，106例（54.1%）进行了原发性完全切除。在局部队列中的21例（11.5％）患者和转移队列中的12例（92.3％）患者中检测到图像定义的淋巴结（LN）疾病。局限性PTRMS患者的5年无事件生存率（EFS）和总生存率（OS）分别为87.3％和94.0％，转移性PTRMS患者的5年无事件生存率（EFS）和总生存率（OS）分别为46.2％和42.2％。在70（42％）IRSI-III患者中观察到初次手术（PV-PS）期间违反了协议。与接受正确的初次手术的患者相比，这导致了更高的R1/R2切除率（n=53[76％]vsn=20[21％];p<0.001），需要进行预处理再切除（PRE）（n=50[83％]vsn=10[17％];p<0.001）。在PRE期间，有13名（20％）患者发生了协议违规。尽管PV-PS不影响局部PTRMS队列中的5年EFS或OS，未经校正的对数秩检验显示,PRE后的R状态是5年OS的预后因素(R1vsR0[81.8%vs97.6%];p=0.02).
结论：PTRMS的手术局部控制质量并不令人满意。在进一步的临床试验中，应重点评估PRE后的切除状态。
方法：将患有局部（IRSI-III）和转移性（IRSIV）PTRMS的患者纳入了两项WeichteilsarkomStudiengruppe（CWS）合作试验（CWS-96，CWS-2002P）和软组织肉瘤登记处（SoTiSaR）。
结果：在196名患者中（中位年龄，8.4年)，106例（54.1%）进行了原发性完全切除。在局部队列中的21例（11.5％）患者和转移队列中的12例（92.3％）患者中检测到图像定义的淋巴结（LN）疾病。局限性PTRMS患者的5年无事件生存率（EFS）和总生存率（OS）分别为87.3％和94.0％，转移性PTRMS患者的5年无事件生存率（EFS）和总生存率（OS）分别为46.2％和42.2％。在70（42％）IRSI-III患者中观察到初次手术（PV-PS）期间违反了协议。与接受正确的初次手术的患者相比，这导致了更高的R1/R2切除率（n=53[76％]vsn=20[21％];p<0.001），需要进行预处理再切除（PRE）（n=50[83％]vsn=10[17％];p<0.001）。在PRE期间，有13名（20％）患者发生了协议违规。尽管PV-PS不影响局部PTRMS队列中的5年EFS或OS，未经校正的对数秩检验显示,PRE后的R状态是5年OS的预后因素(R1vsR0[81.8%vs97.6%];p=0.02).
结论：PTRMS的手术局部控制质量并不令人满意。在进一步的临床试验中，应重点评估PRE后的切除状态。
