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.