Primary conjunctival rhabdomyosarcoma: 2.5 years’ follow-up after combined chemotherapy and brachytherapy

Abstract 

· Background: The 2.5-year outcome of a 3-year-old girl with a primary intraconjunctival rhabdomyosarcoma treated by biopsy-controlled combined chemo- and brachytherapy is reported. · Methods: The patient presented with a conjunctival dermoid-like lesion which showed modest growth on 1-month follow-up. The first biopsy appointment was missed because of a viral illness. When she showed up 3 months later a further increase in size was obvious and several incisional biopsies were taken immediately. · Results: Light microscopy revealed an undifferentiated subepithelial small cell tumour. On immunohistochemistry the tumour reacted with vimentin and desmin antibodies. Thus, the diagnosis of an embryonal rhabdomyosarcoma was made. The patient underwent 9 cycles of polychemotherapy and the tumour mass disappeared macroscopically. However, immunohistochemistry of incisional biopsies showed residual tumour cells. The latter were eradicated by an individually shaped strontium-90 applicator. · Conclusion: At 2.5 years after biopsy-controlled combined chemotherapy and brachytherapy the patient is free of tumour at a manageable level of complications. This new approach appears to be a good alternative in the treatment of primary conjunctival rhabdomyosarcomas since it takes advantage of the unique location of this rare tumour and avoids hemifacial retardation, known to be induced by external beam radiation.