Non-metastatic pediatric head and neck rhabdomyosarcoma: a 24-year retrospective experience from Jordan.
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BACKGROUND: Pediatric head and neck rhabdomyosarcoma (HNRMS) is well characterized in Western cooperative group trials, but real-world outcomes from resource-constrained MENA settings are poorly documented. Whether the locally advanced-stage presentation typical of Middle Eastern referral centers precludes outcomes equivalent to international benchmarks is unknown. METHODS: We conducted a retrospective analysis of pediatric patients younger than 18 years with non-metastatic HNRMS diagnosed between January 2001 and May 2025. We extracted demographic, pathological, treatment, and outcome data. Risk stratification and treatment planning followed COG protocols throughout, with local control decisions made via multidisciplinary team assessment. RESULTS: Among 98 patients with HNRMS, 77 (79%) had non-metastatic disease and were included in the analysis. The male-to-female ratio was 1:1, with a median age at diagnosis of 5.9 years (range, 0.2-18). The parameningeal region (n = 31, 40%) and the orbital region (n = 20, 26%) were the most common primary sites. Histology was embryonal in 51 patients (66%), and tumor size exceeded 5 cm in 55 patients (71%). Sixty patients (78%) were classified as intermediate risk and 93.5% were IRS Clinical Group III. All patients received chemotherapy, with 32% receiving maintenance therapy. Local excision was performed in 15 patients (19%), and 71 patients (92%) received radiotherapy. Twenty-three patients (30%) experienced relapse or progression, with 74% presenting with local recurrence or progression. The median follow-up time was 30.7 months (range, 2.6-294). The 5-year event-free survival (EFS) and overall survival (OS) were 66.1% (55.4%-79%) and 70.1% (59.1-83.1%), respectively. Subgroup analysis demonstrated variable 5-year OS rates according to primary site: orbital (91.7 ± 8%), parameningeal (51.2 ± 10.6%), and other HN primaries (76.1 ± 9.4%). CONCLUSION: Despite 93.5% IRS Clinical Group III and 71% tumors >5 cm-rates substantially exceeding Western cooperative group series-5-year OS of 70.1% was achieved. Parameningeal location was the only factor significantly associated with adverse survival outcomes in this cohort, driven by local failure. These findings demonstrate that outcomes equivalent to international benchmarks are attainable in a resource-constrained MENA setting, and identify local control intensification for parameningeal disease as the priority unmet need.