Abstract The treatment of paratesticular rhabdomyosarcoma (PT‐RMS) has varied over time and by cooperative group. The International Soft Tissue Sarcoma Database Consortium (INSTRuCT) is a collaboration of the Children's Oncology Group (COG) Soft Tissue Sarcoma Committee, European pediatric Soft tissue sarcoma Study Group (EpSSG), and the Cooperative Weichteilsarkom Studiengruppe (CWS). The INSTRuCT surgical committee has been given charge of the development of internationally applicable consensus guidelines for the surgical treatment of rhabdomyosarcoma. This clinical consensus opinion document addresses accepted principles and areas of controversy, such as scrotal violation and retroperitoneal nodal evaluation, providing an evidence‐based guideline for the surgical treatment for PT‐RMS.
Abstract Aim No widely agreed consensus protocols exist for the management of benign ovarian tumors (BOT) in children. This presents a substantial risk for suboptimal management. We aimed to generate multispecialty consensus guidance to standardize surgical management and provide a clear follow‐up protocol for children with BOTs. Methods Prospective two‐round confidential e‐Delphi consensus survey distributed among multispecialty expert panel; concluded by two semistructured videoconferences. Main results Consensus was generated on these core outcome sets: preoperative/intraoperative management; follow‐up; adolescent gynecology referral. (1) Children with BOTs should receive the same management as other patients with potentially neoplastic lesions: Preoperative discussion at a pediatric oncology multidisciplinary meeting to risk stratify tumors, and management by health professionals with expertise in ovarian‐sparing surgery and laparoscopy. (2) Ovarian‐sparing surgery for BOTs should be performed wherever possible to maximize fertility preservation. (3) Ovarian masses detected during emergency laparoscopy/laparotomy should be left in situ wherever feasible and investigated appropriately (imaging/tumor markers) before resection. (4) Follow‐up should be undertaken for all patients after BOT resection. Patients should be offered referral to adolescent gynecology to discuss fertility implications. Conclusion This best practice Delphi consensus statement emphasizes the importance of managing children with BOTs through a well‐defined oncological MDT strategy, in order to optimize risk stratification and allow fertility preservation by ovarian‐sparing surgery wherever possible.
Abstract Background/objectives Rhabdomyosarcoma of the perianal/perineal region (PRMS) is rare, with poor survival and limited understanding of the functional consequences of treatment. Design/methods International Society of Pediatric Oncology (SIOP) malignant mesenchymal tumor (MMT) 95, Italian RMS 96, and European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 studies were interrogated to identify factors that impact survival; in RMS 2005, functional outcomes were analyzed. Results Fifty patients (nonmetastatic) were identified, median age 6.4 years (range: 0.1–19.6): 29 male, 21 female. Tumors were >5 cm in 33 patients. Histopathological subtype was alveolar in 35. Lymph nodes were involved in 23 patients. In RMS 2005, 16/21 (76%) tested alveolar tumors had positive FOXO1 fusion status. Diagnostic biopsy was performed in 37. Primary resection (13) was complete (R0) in one. Delayed primary excision (16) was complete in three. Radiotherapy (RT) in 34/50 patients included external beam (28), brachytherapy (3), and both (3). Nodal RT was given in 16/23 N1 patients (70%). Median follow‐up of alive patients (29) was 84.1 months (range: 3.6–221.1). Relapse or progression occurred in 24 patients (48%), 87% were fatal and most events (63%) were locoregional. Five‐year event‐free survival (EFS) was 47.8 (95% CI: 32.8–61.3), and 5‐year overall survival (OS) was 52.6 (95% CI: 36.7–66.2), with age ≥10 years and tumor size >5 cm impacting 5‐year EFS and OS ( p < .05). Functional outcome data showed bowel, genito‐urinary, and psychological issues; fecal incontinence in four of 21 survivors, and urinary symptoms in two of 21. Conclusions About 60% of patients with nonmetastatic PRMS survive; older patients and those with large tumors have the worst outcomes. Biopsy should be the initial procedure, and definitive local therapy individualized. Quality‐of‐life and functional studies are needed to better understand the consequences of treatment.
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