ADULTS WITH RHABDOMYOSARCOMA

Am J Clin Oncol. 2009 Sep 18. [Epub ahead of print]

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Treatment of Adult Rhabdomyosarcoma.

Ogilvie CM, Crawford EA, Slotcavage RL, King JJ, Lackman RD, Hartner L, Staddon AP.

From the *Department of Orthopaedic Surgery, University of Pennsylvania, Philadelphia, PA; daggerDepartment of Orthopaedic Surgery, Drexel University, Philadelphia, PA; and double daggerDepartment of Hematology and Oncology, Pennsylvania Hospital, Philadelphia, PA.

OBJECTIVES:: Rhabdomyosarcoma is an exceedingly rare tumor in adults, and standard chemotherapy used for children is much less effective in adults. This study examines short-term outcomes using doxorubicin, ifosfamide, and vincristine for adult rhabdomyosarcoma. METHODS:: Pathology records were searched for adults (age, >18) with rhabdomyosarcoma treated at our musculoskeletal tumor center. Treatment involved surgical resection, radiation therapy, and chemotherapy with doxorubicin, ifosfamide, and vincristine. Eleven met inclusion criteria. Mean age was 49 (range: 19-72). Tumors sites included upper extremity (4 patients), lower extremity (6), and cervix (1). Subtypes were pleomorphic (7), alveolar (1), embryonal (1), and mixed alveolar/embryonal (2). RESULTS:: Of the 7 patients with nonmetastatic disease, 6 had no evidence of disease posttreatment, but 1 died of myelodysplastic syndrome after 51 months. Three patients who received neoadjuvant chemotherapy had 100% tumor necrosis. One patient with positive margins scheduled for adjuvant chemotherapy had local recurrence and metastasis within 2 weeks and died 5 months later. Of the 4 patients with metastatic disease on presentation, 1 had complete response, 2 had partial response with later progression and death at 8 and 24 months, and 1 had immediate progression and died at 12 months. Mean overall survival was 24 months with 6 of 11 (55%) alive at last follow-up. Mean disease-free survival was 17 months for all patients and 23 months for the 7 patients who had remission of all disease. CONCLUSIONS:: When combined with surgery and radiation therapy, chemotherapy using doxorubicin, ifosfamide, and vincristine yielded 55% overall and 64% disease-free survival at 2 years.