Rhabdomyosarcoma of the extremities in adults

Rhabdomyosarcomas are uncommon in adults and literature regarding their management is limited. Eight patients with an average age of 32.6 years (range: 21 to 75) who were treated for rhabdomyosarcomas on the extremities between 1991 and 2002 in a regional centre for the treatment of soft tissue sarcomas were studied retrospectively. Treatment consisted of en bloc resection of the primary tumour in all the patients, combined with radiotherapy or chemotherapy or both. The tumour size ranged from 5.5 cm to 15 cm (average 9.3 cm). Histologically there were four alveolar, two pleomorphic, one embryonal and one anaplastic subtype. Seven of the eight patients developed metastasis and died after an average period of 15.3 months (range 4-28 months). At the final follow-up, only one patient was disease free and alive 48 months after surgery. Extremity rhabdomyosarcoma is a highly malignant tumour and our results are poor compared to the reported results in children. Metastatic recurrence is high in adult rhabdomyosarcomas even with local control of the disease and therefore development of effective systemic therapy is an urgent priority.