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

Pediatr Hematol Oncol. Jul-Aug 2009;26(5):321-31. doi: 10.1080/08880010902964367.

Abstract

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.

MeSH terms

  • Adolescent
  • Antineoplastic Combined Chemotherapy Protocols
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Female
  • Follow-Up Studies
  • Foot / pathology*
  • Hand / pathology*
  • Humans
  • Infant
  • Italy
  • Male
  • Neoplasm Metastasis
  • Radiotherapy Dosage
  • Radiotherapy, Adjuvant
  • Rhabdomyosarcoma / drug therapy
  • Rhabdomyosarcoma / mortality*
  • Rhabdomyosarcoma / radiotherapy
  • Rhabdomyosarcoma / therapy*
  • Young Adult