Martynov Illya, Sparber-Sauer Monika, Heinz Amadeus, Vokuhl M Christian, Ebinger Martin, Gesche Jens, Münter Marc, Koscielniak Ewa, Fuchs Jörg, Seitz Guido
Department of Pediatric Surgery and Urology, University Hospital Giessen-Marburg, Philipps-University, Campus Marburg, Marburg, Germany.
Department of Pediatric Surgery, University Hospital Giessen-Marburg, Campus Giessen, Giessen, Germany.
Ann Surg Oncol. 2024 Sep;31(9):6209-6219. doi: 10.1245/s10434-024-15568-3. Epub 2024 Jun 15.
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).
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).
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).
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.
本研究旨在评估初次手术及后续手术质量对睾丸旁横纹肌肉瘤（PTRMS）患者生存的影响。
局部（IRS I - III期）和转移性（IRS IV期）PTRMS患者纳入两项合作性软组织肉瘤研究组（CWS）试验（CWS - 96、CWS - 2002P）以及软组织肉瘤登记处（SoTiSaR）。
196例患者（中位年龄8.4岁）中，106例（54.1%）接受了初次完整切除。局部队列中21例（11.5%）患者及转移队列中12例（92.3%）患者检测到影像定义的淋巴结（LN）疾病。局部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患者分别为46.2%和42.2%。IRS I - III期患者中有70例（42%）在初次手术时观察到违反方案情况（PV - PS）。这导致R1/R2切除率更高（53例[76%]对20例[21%]；p < 0.001），与接受正确初次手术的患者相比，需要术前再次切除（PRE）的比例更高（50例[83%]对10例[17%]；p < 0.001）。13例（20%）患者在PRE期间出现违反方案情况。虽然PV - PS不影响局部PTRMS队列的5年EFS或OS，但未调整的对数秩检验显示，PRE后的R状态是5年OS的预后因素（R1对R0[81.8%对97.6%]；p = 0.02）。
PTRMS手术局部控制质量不令人满意。在进一步的临床试验中应重视评估PRE后的切除状态。
