WOFAPS 2025 8th World Congress of Pediatric Surgery

sheetal uttaray, Sajid Qureshi, saif kaderi

TATA MEMORIAL HOSPITAL, MUMBAI, INDIA

Background:
Extremity rhabdomyosarcoma (RMS) in children presents unique clinical and surgical challenges, particularly in the setting of delayed presentation, previous inadequate treatment, and deep-seated tumors. This study evaluates the outcomes of pediatric extremity RMS with a focus on fusion status, nodal disease, response to neoadjuvant chemotherapy (NACT), surgical margin clearance, and the impact of prior incomplete surgeries managed through pretreatment re-excision (PRE).

Methods:
A retrospective review of 61 pediatric patients treated for extremity RMS at a tertiary cancer center from 2003 to 2023 was conducted. Data were analyzed for fusion gene status, lymph node involvement, surgical margins, and timing/type of surgery. Patients were stratified into de novo, residual disease (treated outside and presenting within 6 months), and recurrent disease (>6 months post-treatment).

Results:
Delayed surgery after NACT was performed in 78.7% of patients.PRE was performed in 5 patients who had prior incomplete surgeries; 60% of these showed residual viable tumor. Fusion analysis was performed for 59% patients, of which PAX3/7-FOXO1 positivity was seen in 16.4%, however it didn’t show significant relation with recurrence or survival. Lymph node sampling or dissection was performed in 52.4%, with 6 patients demonstrating histological positivity. NACT led to partial response in 67.2%, significantly reducing tumor burden and aiding limb-sparing surgeries. Margin-negative resection (R0) was achieved in 90.2% cases, yet local, regional and distant recurrence occurred in 13,13 and 26% respectively, highlighting the aggressive biology and potential for micro-metastatic spread. Margin status significantly influenced survival (p = 0.009), and involvement of neurovascular structures necessitated planned R1 resections in select cases.

Conclusion:
Extremity RMS in children often presents as residual or previously treated disease, requiring delayed surgery. Outcomes are influenced by lymph node status, margins, and recurrence. NACT aids downstaging, while PRE ensures margin control in outside-operated cases. Multidisciplinary, oncologic surgery-focused care is key to better outcomes.