Outcome of patients with localized orbital sarcoma who relapsed following treatment on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols-III and -IV, 1984-1997: A report from the Children's Oncology Group

Background: We wanted to ascertain patterns of recurrence, re-treatment, and outcome among 188 eligible patients treated for localized orbital sarcoma on IRSG Protocols III/IV, 1984-1997. Procedure: Retrospective chart review. Results: Twenty-four of 188 patients (12.8%) developed local (n=22) or distant relapse (n=2) at 0.057-7.05 years (median, 1.58) after enrollment. Ages at study entry were 0.14-17 years (median, 5 years). Initial tumor operations included biopsy (n=20) or gross resection with microscopic residual (n=4). Initial tumor diameters were 0.5-7cm (median, 3). Pathologic subtypes were embryonal rhabdomyosarcoma (ERMS, n=19), sarcoma not otherwise specified (n=2), and alveolar RMS, botryoid ERMS, or undifferentiated sarcoma (n=1 each). Initial treatment included vincristine/dactinomycin (n=24) including an alkylator (n=4) and radiotherapy (RT, n=21). Twenty patients responded, 14 completely, 6 partially. After recurrence, patients underwent orbital exenteration (n=10), enucleation (2), tumor excision (3), or biopsy (1); 7 had no operation, and 1 had no data. Post-relapse chemotherapy included combinations of etoposide (n=14 patients), doxorubicin (14), ifosfamide (12), cyclophosphamide (7), and dacarbazine (n=1). Six patients received RT, including four previously treated and two not irradiated initially. Two patients died; one at 1.79 years after contralateral brain metastasis followed by local recurrence, and another at 2.49 years after multiple local recurrences. Twenty-two patients (91.7%) survived sarcoma-free for 0.04-17 years (median, 6.9) after relapse, and 18 of 22 (82%) were alive ≥5 years after relapse. Conclusion: Survival following recurrent localized orbital sarcoma appears likely after vigorous re-treatment given with curative intent.