Optic nerve glioma and colonic polyposis: report of a new association

Aim: The authors describe the clinical, radiological and histopathological features of an optic nerve glioma associated with colonic polyposis. The association between colonic polyposis and neuroepithelial tumours of the central nervous system has been previously described, but no association with optic nerve gliomas has ever been reported. Methods: We report a 14‐y‐old girl with colonic polyposis and unilateral proptosis for whom an excisional biopsy of the orbital lesion was performed. Results: Histopathological evidence of juvenile pilocytic astrocytoma grade 1 was detected. Correlation with criteria for Turcot's syndrome was established. Conclusion: Optic nerve tumours should be included in the spectrum of central nervous system lesions associated with colonic polyposis.