Local control of parameningeal rhabdomyosarcoma: An expert consensus
guideline from the International Soft Tissue Sarcoma Consortium
(INSTRuCT)
Abstract
The International Soft-Tissue Sarcoma Database Consortium (INSTRuCT)
consists of a collaboration between the Children’s Oncology Group (COG)
Soft Tissue Sarcoma Committee, the European pediatric Soft-Tissue
Sarcoma Study Group (EpSSG), and the Cooperative Weichteilsarkom
Studiengruppe (CWS). As part of the larger initiative of INSTRuCT to
provide consensus expert opinions for clinical treatment of pediatric
soft tissue sarcoma, we sought to provide updated, evidenced-based
consensus guidelines for local treatment of parameningeal
rhabdomyosarcoma using both existing literature as well as
recommendations from the relevant cooperative group clinical trials.
Overall, parameningeal rhabdomyosarcoma represents a distinctly
challenging disease to treat given its location near many critical
structures in the head and neck, frequently advanced local presentation,
and predilection for local failure. Definitive chemoradiation remains
the standard treatment approach for parameningeal rhabdomyosarcoma, with
surgery often limited to biopsy or salvage therapy for recurrent
disease. In this consensus paper, we specifically discuss consensus
guidelines and evidence for definitive local management with
radiotherapy, with a focus on imaging for radiotherapy planning, dose
and timing of radiation, approach for nodal irradiation, various
radiation techniques including proton therapy, and the limited role of
surgical resection.