TY - JOUR

T1 - Malignant transformation of a medulloepithelioma of the optic nerve

AU - Lindegaard, Jens

AU - Heegaard, Steffen

AU - Toft, Peter Bjerre

AU - Nysom, Karsten

AU - Prause, Jan Ulrik

PY - 2010/6

Y1 - 2010/6

N2 - Objective: To describe a case of malignant transformation of a medulloepithelioma of the optic nerve.Methods: A 3-year-old boy presented with right-sided proptosis, swollen eyelids, restricted ocular movements, pain and nausea. Visual acuity was hand motions. A benign non-teratoid medulloepithelioma of the optic nerve was surgically removed. Due to relapse in the orbit 10 months postoperatively enucleation and partial orbital exenteration were performed. Light microscopy and immunohistochemistry now revealed a malignant medulloepithelioma. The boy was further treated with chemotherapy and radiotherapy and is still alive 36 months after primary surgery.Results: Medulloepithelioma of the optic nerve is extremely rare. Only eight cases have been described in the literature. Unlike intraocular medulloepithelioma, all have been malignant tumours. This is the first case described of a benign medulloepithelioma of the optic nerve, later transforming into a malignant tumour. One third of patients with medulloepithelioma of the optic nerve have died from direct intracranial spread or metastasis to the central nervous system (CNS).Conclusions: Medulloepithelioma of the optic nerve should be considered malignant despite morphology with tendency to spread to the CNS. Furthermore; it should be considered as a differential diagnosis when observing a tumour of the optic nerve in a child.Read More: http://informahealthcare.com.ep.fjernadgang.kb.dk/doi/abs/10.3109/01676830903421200

AB - Objective: To describe a case of malignant transformation of a medulloepithelioma of the optic nerve.Methods: A 3-year-old boy presented with right-sided proptosis, swollen eyelids, restricted ocular movements, pain and nausea. Visual acuity was hand motions. A benign non-teratoid medulloepithelioma of the optic nerve was surgically removed. Due to relapse in the orbit 10 months postoperatively enucleation and partial orbital exenteration were performed. Light microscopy and immunohistochemistry now revealed a malignant medulloepithelioma. The boy was further treated with chemotherapy and radiotherapy and is still alive 36 months after primary surgery.Results: Medulloepithelioma of the optic nerve is extremely rare. Only eight cases have been described in the literature. Unlike intraocular medulloepithelioma, all have been malignant tumours. This is the first case described of a benign medulloepithelioma of the optic nerve, later transforming into a malignant tumour. One third of patients with medulloepithelioma of the optic nerve have died from direct intracranial spread or metastasis to the central nervous system (CNS).Conclusions: Medulloepithelioma of the optic nerve should be considered malignant despite morphology with tendency to spread to the CNS. Furthermore; it should be considered as a differential diagnosis when observing a tumour of the optic nerve in a child.Read More: http://informahealthcare.com.ep.fjernadgang.kb.dk/doi/abs/10.3109/01676830903421200

U2 - 10.3109/01676830903421200

DO - 10.3109/01676830903421200

M3 - Journal article

C2 - 20497085

VL - 29

SP - 161

EP - 164

JO - Orbit

JF - Orbit

SN - 0167-6830

IS - 3

ER -