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Primary ocular, adnexal, and orbital rhabdomyosarcoma: A review

Ayushi Agarwal1, Vijitha S Vempuluru1, Swathi Kaliki1

  • 1The Operation Eyesight Universal Institute for Eye Cancer (AA, VSV, SK), LV Prasad Eye Institute, Hyderabad, Telangana 500034, India.

Survey of Ophthalmology
|March 29, 2025
PubMed

Insights

Orbital rhabdomyosarcoma (RMS) presents challenges, especially in recurrent or metastatic cases. Further research is needed for standardized treatments and improved outcomes in this rare cancer.

Area of Science:

  • Ophthalmology
  • Pediatric Oncology
  • Pathology

Background:

  • Rhabdomyosarcoma (RMS) is a rare soft tissue sarcoma that can occur in the orbit, eye, and adnexa.
  • Orbital RMS, despite favorable location, has a poor prognosis in relapsing, refractory, or metastatic stages.
  • Significant heterogeneity exists in reported survival rates and treatment protocols globally.

Purpose of the Study:

  • To comprehensively review orbital, ocular, and adnexal rhabdomyosarcoma (RMS).
  • To describe clinicoradiological, histopathological, and molecular features.
  • To discuss management, outcomes, and challenges, highlighting the need for standardized approaches.

Main Methods:

  • Systematic literature review of orbital, ocular, and adnexal RMS.
  • Analysis of clinicoradiological and histopathological findings.
  • Evaluation of molecular updates, management protocols, and survival data.

Main Results:

  • Orbital RMS prognosis is dismal for relapsed, refractory, and metastatic cases.
  • Wide variations in survival rates and treatment regimens are observed across different regions and age groups.
  • Molecular cytogenetics is critical for RMS diagnosis and management.

Conclusions:

  • Novel targeted therapies show promise in reducing treatment-related morbidity and mortality.
  • Standardized treatment regimens are essential for uniformity, particularly in advanced and metastatic RMS.
  • Large-scale studies are imperative to establish evidence-based, uniform management protocols.
Keywords:
EyeMalignancyOrbitPediatricRhabdomyosarcomaTumor

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