Abstract

To explore clinical experience and propose new ideas for treating children diagnosed with orbital rhabdomyosarcoma (RMS). We retrospectively analyzed the clinical data for30 patients (16 males and 14 females, with a median age of 6.2 years) with primary orbital RMS who were enrolled in the Department of Eye Oncology and Pediatrics of our hospital from November 2004 to December 2012. International Rhabdomyosarcoma Organization Staging Standards indicated that among the 30 patients, 4 cases were in phase II, 20 were in phase III, and 6 were in phase IV. All patients underwent a multidisciplinary collaborative model of comprehensive treatment (surgery, chemotherapy, external radiotherapy, 125I radioactive particle implantation, and autologous peripheral blood stem-cell transplantation). Follow-up was conducted until March 2013, with a median follow-up time of 47.2 months (5 to 95 months), and 7 deaths occurred. The 2-year estimated survival rate reached 86.1%, the ≥3-year estimated survival rate was 77%, and the 5-year estimated survival rate was 70.6%. The multidisciplinary collaborative model can be a safe and effective approach to the comprehensive treatment of children with orbital RMS. It has clinical significance in improving the tumor remission rate.

Highlights

  • Rhabdomyosarcoma (RMS) is a type of malignant tumor in the original skeletal muscle cells; in children, it is the most common malignant soft tissue sarcoma, which accounts for approximately 4% to 8% of pediatric malignant solid tumors (Oberlin et al, 2001)

  • Basing from clinical staging and condition assessment, this study focuses on selecting a reasonable chemotherapy, radiotherapy, and surgical treatment for pediatric orbital RMS

  • Side-effect evaluation criteria analysis of orbital RMS indicated that the primary

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Summary

Introduction

Rhabdomyosarcoma (RMS) is a type of malignant tumor in the original skeletal muscle cells; in children, it is the most common malignant soft tissue sarcoma, which accounts for approximately 4% to 8% of pediatric malignant solid tumors (Oberlin et al, 2001). The orbit is the common primary site of RMS in children, and this condition accounts for about 35% to 45% of all cases (Breneman et al, 2012). Complete tumor resection is the key to curing orbital RMS. In pediatric orbital RMS, the anatomical structure of the lesion is complex and the tumor growth is rapid, a condition that makes complete surgical resection difficult and leads to relatively high recurrence, metastasis, and mortality rates (Van Gaal et al, 2012). Achieving complete surgical resection to reduce recurrence, metastasis, and mortality rates has become a key research area

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