Childhood orbital rhabdomyosarcoma: Report from Children’s Cancer Hospital-57357-Egypt

Abstract

Background: Rhabdomyosarcoma (RMS) in the head and neck especially orbit represents a major anatomic site for this tumor in pediatrics. Orbital RMS is the most common primary orbital malignancy in children with approximately 35 new cases per year.

Objectives: The aim of this work is to study cases of orbital RMS and assess epidemiology, clinical and pathological characteristics as well as survival outcomes.

Methods: Patients diagnosed with orbital RMS between July 2007 and July 2012 follow-up till July 2014. They were treated according to IRS-IV and IRS V protocols. Case report forms were analyzed and treatment outcome, OS and FFS for patients were analyzed.

Results: Seventeen orbital RMS patients were diagnosed at the mentioned period. Complete remission was identified in 7 (41.2%) cases, Partial remission in 4 (23.5%) cases and progressive disease in 4 (23.5%) cases while 2 cases died before evaluation. Three patients had experienced different management-related ophthalmic sequelae. Only one patient died due to chemotherapy-associated toxicity. The 4-years OS and 4-years FFS were 94.1 ± 5.7% and 65.4 ± 1.5% respectively.

Conclusion: The current study demonstrated that RMS cases that present with orbit involvement are associated with better clinical outcome. Future treatment of patients with non-metastatic orbital RMS will focus on adjustments in therapy to reduce acute and late adverse effects while maintaining their excellent treatment outcome. New therapeutic approaches are required for the patients whose present outcome is less than optimal.