Rhabdomyosarcoma is a rare
tumor, with an annual incidence of 4.3 cases per million children. Even thought, it is the most common
soft tissue sarcoma in childhood, with a mean age of 6 to 8 years at diagnosis. A 4 year-old boy presented with a history of a fast growing (1-month) nodular lesion in the caruncle of his left eye.
Slit lamp examination showed a vascularized solid nodular lesion in the semilunar fold. The lesion was surgically removed obtaining infiltrated edges with tumoral cells. A second surgery was performed with free tumour edges. The diagnosis of
embryonal rhabdomyosarcoma, botryoid type, of intermediate differentiation was made. The treatment for botryoid
rhabdomyosarcoma is basically surgical with the combined use of adjuvant
polychemotherapy. In adolescent or adult patients (not in infants where growth bone disturbances can occur) external beam
radiotherapy can be combined with
chemotherapy.
Rhabdomyosarcomas of the conjunctiva without orbital extension are rarely reported. We presented a case of a child with a rare
tumor which we had a high suspicion of
malignancy and early diagnosis and treatment and child is free of systemic disease 6 and half years later. Our research group believes that the key in these
tumors is the high index of suspicion and early treatment.