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Past
Paratesticular rhabdomyosarcoma (PTRMS) is the most prevalent non-germ-cell scrotal malignant tumor, accounting for approximately 5% of all pediatric scrotal masses and 7% of all pediatric rhabdomyosarcomas (RMS).1,2 The recommended surgical approach for PTRMS involves primary inguinal orchidectomy with high ligation of the spermatic cord before tumor mobilization. However, protocol violations during primary surgery are common and often lead to incomplete tumor resections, necessitating revisional surgeries known as pretreatment re-excision (PRE). PRE can downstage tumors from IRS III (macroscopically incomplete tumor resection, R2) or IRS II (microscopically incomplete tumor resection, R1) to IRS I (microscopically complete tumor resection, R0), thus achieving excellent oncological outcomes. Nevertheless, there is a paucity of data on the effects of unsuccessful PRE, which may result in local R1/R2 situation, on event-free survival (EFS) or overall survival (OS).
Present
We evaluated the impact of the quality of initial and subsequent surgeries on the survival of patients with non-metastatic and metastatic PTRMS enrolled in the Cooperative Weichteilsarkom Studiengruppe (CWS) trials (CWS-96, 2002P) and the Soft Tissue Sarcoma Registry (SoTiSaR). Our findings reveal that the quality of local surgical control is frequently unsatisfactory, characterized by a high incidence of protocol violations during primary surgery (42%) and PRE (20%).3 Further, our analysis, using an unadjusted log-rank test, highlighted that R status following PRE is a significant prognostic factor for 5-years OS. This underscores the critical need for strict adherence to surgical guidelines, particularly during revisional surgery.
Future
Our study indicates that improvements are necessary in the quality of surgical local control for PTRMS. The path forward should focus on enhancing surgical training and preoperative planning to reduce protocol violations during both primary surgery and PRE. Notably, an R1 resection following PRE was associated with decreased OS, suggesting its potential role as a prognostic factor for patients with localized PTRMS.
References
Shapiro E, Strother D. Pediatric genitourinary rhabdomyosarcoma. J Urol. 1992;148(6):1761–8. https://doi.org/10.1016/s0022-5347(17)37023-4.
Ahmed HU, Arya M, Muneer A, Mushtaq I, Sebire NJ. Testicular and paratesticular tumours in the prepubertal population. Lancet Oncol. 2010;11(5):476–83. https://doi.org/10.1016/s1470-2045(10)70012-7.
Martynov I, Sparber-Saue M, Heinz A, et al. Importance of adequate surgical local control in fusion negative paratesticular rhabdomyosarcoma: data from the “Cooperative Weichteilsarkom Studiengruppe” trials CWS-96 2002P and European Soft Tissue Sarcoma Registry (SoTiSaR). Ann Surg Oncol. 2024. https://doi.org/10.1245/s10434-024-15568-3.
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This article refers to: Martynov I, Sparber-Saue M, Heinz A, et al. Importance of adequate surgical local control in fusion negative paratesticular rhabdomyosarcoma: data from the “Cooperative Weichteilsarkom Studiengruppe” trials CWS-96 2002P and European Soft Tissue Sarcoma Registry (SoTiSaR). Ann Surg Oncol. (2024). https://doi.org/10.1245/s10434-024-15568-3.
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Martynov, I., Sparber-Sauer, M., Heinz, A. et al. ASO Author Reflections: Surgical Management of Paratesticular Rhabdomyosarcoma. Ann Surg Oncol (2024). https://doi.org/10.1245/s10434-024-15687-x
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DOI: https://doi.org/10.1245/s10434-024-15687-x