Outcomes of therapeutic strategies in Merkel cell carcinoma among patients aged 0-30 years.
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Merkel cell carcinoma (MCC) in patients aged < 30 years is exceptionally rare, representing < 1% of all cases. Limited data exist regarding optimal therapeutic approaches and outcomes in this age group. Our objective was to analyse clinicopathological patterns and therapeutic outcomes of MCC in patients aged 0-30 years through systematic review of the published literature. We extracted all cases of MCC in patients aged 0-30 years published through October 2024, identifying 44 patients from 14 articles. Data on tumour stage, location, treatment modalities, outcomes and recurrence rates were analysed. The cohort comprised 23 male and 21 female patients with a median age of 14.8 years. Head and neck sites were most common (41%), with oral mucosal involvement in 7% of cases - notably higher than in older adults. Advanced presentation was frequent, with 22% presenting with stage IV disease, compared with 10-16% in older cohorts. Treatment analysis of 24 patients revealed that surgery alone achieved only 33% complete resolution (2 of 6), with a 67% relapse rate. Conversely, surgery with adjuvant radiotherapy achieved 100% complete resolution with no documented recurrences. Chemotherapy alone showed no complete responses. Disease-specific mortality was 8%, significantly lower than the 40% 5-year mortality in older adults. In conclusion, young patients with MCC frequently present with advanced disease but achieve excellent outcomes with multimodal therapy. Surgery alone carries unacceptable relapse rates. Standard adult treatment protocols incorporating wide excision and adjuvant radiotherapy are both feasible and highly effective in patients aged < 30 years, with minimal toxicity and superior locoregional control.