We reviewed clinicopathologic features and treatment outcomes in 7 patients diagnosed with Stage 4/Group IV orbital sarcoma and treated on IRSG protocols I-III. Three patients had embryonal rhabdomyosarcoma (RMS), and 2 patients each had alveolar RMS or unclassified sarcoma. Median age at diagnosis was 1.8 years (range 0.2 – 6.9 years). All patients had bone marrow involvement, including 6 with normal CBC at diagnosis. Cerebrospinal fluid (CSF) was normal in 6 patients. Three patients survived > 5 years, including one with local recurrence.
Conclusion
Further study is needed to determine necessity of bone marrow and CSF examination in orbital sarcoma patients.