Rhabdomyosarcoma of the extremities: A focus on tumors arising in the hand and foot

Background: Extremities are peculiar sites of origin for rhabdomyosarcomas (RMS) and are usually associated with an unfavorable outcome. Methods: The authors reviewed the clinical data on 60 patients 21 years old with limb RMS treated at the Pediatric Oncology Unit of the Istituto Nazionale Tumori of Milan, in Italy, over a 30-year period. Twelve patients had tumors arising in the hand and foot. Results: Complete tumor resection was achieved in 21 patients (but in only 1 case of hand/foot RMS); all patients received chemotherapy and 43 also had radiotherapy. The alveolar subtype was identified in 62% of cases. Half of the hand/foot cases had metastatic disease at onset. Overall survival at 5 years was 33% among the hand/foot cases and 56% for the others. Most of the relapsing cases had distant metastases. Conclusions: A particularly poor survival was observed for RMS of the hand/foot due its marked tendency to spread. Surgical resection is particularly difficult in such cases and a multimodality treatment approach seems crucial to improving their outcome.