Orbital sarcoma with metastases at diagnosis: a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group

诊断时已发生转移的眼眶肉瘤:儿童肿瘤协作组软组织肉瘤委员会的报告

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Abstract

We reviewed clinicopathologic features and treatment outcomes in seven patients diagnosed with Stage 4/Group IV orbital sarcoma and treated on IRSG protocols I-III. Three patients had embryonal rhabdomyosarcoma (RMS), and two 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 six with normal complete blood count at diagnosis. Cerebrospinal fluid was normal in six patients. Three patients survived >5 years, including one with local recurrence. In conclusion, further study is needed to determine necessity of bone marrow and CSF examination in orbital sarcoma patients.

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