Primary ocular, adnexal, and orbital rhabdomyosarcoma: A review.

Abstract

We review the primary orbital, ocular, and adnexal rhabdomyosarcoma (RMS) spectrum of tumors and describe its clinicoradiological and histopathological features, molecular updates, management protocols, outcomes, and challenges. Despite being a favorable site, the prognosis remains dismal for cases of relapsing, refractory, and metastatic orbital RMS. A wide heterogeneity of data exists in terms of survival rates and treatment regimens spread across various geographic locations and age groups. Molecular cytogenetics is a crucial factor in both the diagnosis and effective management of RMS. Novel targeted therapy may play an integral role in avoiding treatment-related morbidity and mortality. Large-scale studies are warranted to formulate standardized regimens and uniformity, especially for advanced and metastatic cases.