Outcome of pediatric parameningeal rhabdomyosarcoma : the Children Cancer Hospital, Egypt, experience

الملخص EN

Background : PM RMS represents a diagnostic and therapeutic problem as it is less visible than other superficial head and neck sites, and has tendency to local and intracranial extension.

Objectives : the aim of this work is to study the treatment outcome, overall survival (OS) and event free survival (EFS) of pediatric PM RMS patients diagnosed and treated at the Children Cancer Hospital-Egypt [CCHE-57357] during a 4 year period.

Methods: Retrospective review of charts of newly diagnosed pediatric PM RMS patients diagnosed and treated in CCHE during the period between July 2007 and the end of June 2011.

Results : Forty-two pediatric patients with PM RMS with age ranging from 3 months to 17.7 years (median 6.9 years) were studied.

The follow up period ranged from 4 to 55 months with a median of 24.8 months.

Twenty-one patients [50 %] were stage III, while 11 patients [26.1 %] were stage IV.

The 3-year overall survival (OS) was 58.4 ± 8.9 %.

OS was 65.9 ± 10 % for non-metastatic tumors while it was 35.8 ± 16.2 % for the metastatic ones (p = 0.039) the 3-year event-free survival (EFS) was 48 ± 8.6 % for the whole group.

The non-metastatic and metastatic patients had 3-year EFS of 56.5 ± 9.7 % and 24.9± 14.9 % respectively.

This difference was not statistically significant (p = 0.127) Conclusion : PM RMS remains a diagnostic and therapeutic problem.

Late presentation and advanced local disease compromise treatment options and decrease OS and EFS.