Treatment of children and adolescents with localized parameningeal sarcoma: Experience of the Intergroup Rhabdomyosarcoma Study Group protocols IRS‐II through ‐IV, 1978–1997*†

Abstract

Biopsy, XRT to the target volume, and systemic chemotherapy are successful treatments for the large majority of patients with localized parameningeal sarcoma. Carefully defining and irradiating the initial volume should reduce the risk of CNS failure. Aggressive initial surgical management of these patients is unnecessary.

Improved care of rhabdomyosarcoma in Jordan using less intensive therapy

“…Overall survival (OS) rates for children with RMS have improved significantly over the last 30 years [2], primarily due to advances in multimodal therapy. All treatment strategies incorporate systematic multiagent chemotherapy and local therapy [3,4]. Chemotherapy for RMS typically comprises alkylating agents (ifosfamide or cyclophosphamide) at varying dosages, depending on regimen.…”

“…Chemotherapy for RMS typically comprises alkylating agents (ifosfamide or cyclophosphamide) at varying dosages, depending on regimen. Although surgical resection, either as a primary procedure or a secondary strategy, is important for local control, complete resection is not feasible for many tumors (e.g., parameningeal RMS) [4]. Radiotherapy (RT) plays a major role in the management of many patients with RMS although its late sequelae can be significant [5,6].…”

Local control of parameningeal rhabdomyosarcoma: Outcomes in non‐complete responders to chemoradiation

“…Approximately two-thirds of PM-RMS children are free from any failure at 5-years from initial diagnosis. Limited information, however, is available regarding the role of surgery in non-complete responders or those who relapse at the primary site after chemoradiation [1,4]. This study is an attempt to provide information regarding the local control outcome of these patients as treated at our institution.…”

“…Survival prior to the formation of the Intergroup Rhabdomyosarcoma Study (IRS) Group was less than 25% whereas the current estimated 5-year overall survival rate on the IRS is 73% [1]. Key to the improvement in outcome is the radiotherapeutic approach to these tumors; proper radiotherapy (RT) volume, dose, and timing are necessary to maximize local control [2,3].…”

“…Because of the close proximity of these tumors to the meninges and the possibility that surgery may cause cosmetic deformities, most patients with PM-RMS in North America are treated with chemotherapy and RT. It is estimated that 94% of localized PM-RMS fall into the Group III classification while the remaining 6% have Group I and II disease [1]. Approximately two-thirds of PM-RMS children are free from any failure at 5-years from initial diagnosis.…”