Retinoblastoma with Fulminant Neoplasic Meningitis: A Case Report

Backround: Retinoblastoma (RB) is a worldwide intraocular malignancy, with an incidence of approximately 1 in 15,000 - 20, 000 live births worldwide. It affects children under 4 years old and a late diagnosis in the mayority of cases has a fatal result.¹ The first clinical signs are leukocoria (white reflectios in pupil) and strabismus. Meningeal metastasis, though rare can be seen in advanced stages of retinoblastoma. ^2,3,4

Objective: To report a case of fulminant meningitis after enucleation and chemotherapy.

Methods: A 22 months old male child came to our outpatient clinic for “loss” of brightness of left eye. Clinical exam revealed leukocoria, fixed mydriasis and a big intraocular mass. To rule out neoplasic extension, bone gammagraphy, bone marrow aspirate and biopsy, CSF analysis , orbital and cerebral magnetic resonance imaging were performed every three months. Patient underwent enucleation where a retrolaminar involvement of the optic nerve was observed and he was started on chemotherapy (vincristine, etopside and carboplatin) for 6 cycles. 3 months after finishing the last chemotherapy cycle our patient developed sudden deafness and 36 hours later cerebral death due to a neoplasic meningitis, confirmed by CSF cytology.

Conclusion: Few cases of meningeal metastasis due to RB are reported. It is a rare condition with a very high mortality rate. Early diagnosis and treatment of RB are determinant in the prognosis of these patients.

Results: Few cases of meningeal metastasis due to RB are reported. It is a rare condition with a very high mortality rate. Early diagnosis and treatment of RB are determinant in the prognosis of these patients.