Orbital sarcoma with metastases at diagnosis: A report from the soft tissue sarcoma committee of the Children's Oncology Group

Abstract

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.

Detection of Bone Metastases in Initial Staging of Orbital Embryonal Rhabdomyosarcoma by Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography

“…Patients presenting metastatic disease at initial diagnosis are uncommon. However, bone marrow involvement counts among the most common metastases, especially in the presence of extensive local invasion (1). Embryonal RMS is the most common histology in patients with non-metastatic orbital sarcoma.…”