Long-term evaluation of orbital rhabdomyosarcoma in children

Abstract

Background: Orbital rhabdomyosarcoma (ORMS) is associated with an excellent survival rate greater than 85%, and is considered to be a favourable site for this tumour. Treatment is based on combination chemotherapy together with best local therapy, sometimes surgery but more often radiation therapy. Local therapy is associated with frequent and potentially severe late sequelae.

Design: Retrospective hospital single-centre analysis.

Participants: Eighty-two patients treated in Institut Curie, Paris.

Methods: To define long-term status of survivors after localized ORMS, patients treated between 1975 and 2010 were analysed.

Main outcome measures: Clinical structural and functional orbital, and general sequelae.

Results: Median age at diagnosis was 6 years (range: 8 months-19 years), and median follow up was 8.5 years (range: 7 months-24 years). The 5-year globe conservation rate was 90.4%. Ophthalmic dysfunction was present in 79% of patients. Impaired visual acuity (VA), was present in 62% of patients; 38% of them had severe visual disability with VA < 6/60. Late effects on orbitofacial structure were present in 39.8% of patients. Ocular or palpebral sequelae were present in 79% of survivors, mainly cataract (42%), ocular surface lesions such as keratoconjunctivitis (40%) and eyelid abnormalities (29%). General late effects were rare.

Conclusions: These data suggest that ocular and orbital late effects are frequent after treatment of ORMS, indicating the need for systematic long-term ophthalmologic follow up of these patients. Radiation therapy is an important part of the total burden of therapy.

Keywords: chemotherapy; late effect; orbital rhabdomyosarcoma; paediatric; radiotherapy.