Titre | A benign schwannoma mimicking an orbital varix |
But | To present an unusual cause of proptosis. |
Méthodes | Case report: A patient with a slowly progressive left proptosis was referred to our department. Clinical assessment, full ophthalmologic workout, imaging, colour-duplex, biopsy of the lesion and surgical excision were performed. |
Résultats | A marked non-axial, painless proptosis of the left eye was noted. The eyeball was displaced inferolaterally. Palpation revealed a hard mass protruding in the upper eyelid. The lesion was not attached to the overlying skin and did not pulsate. Best corrected visual acuity was 10/10 in the right eye and 4/10 in the left. Slitlamp and fundoscopy were normal. Ocular motility showed restrictions in all directions of gaze. On CT and MRI scan a well encapsulated, tortuous mass with mild contrast enhancement was found. Colour-duplex showed absence of flow within the lesion. Additional echography confirmed the diagnosis of a non-cystic soft tissue lesion. During surgery a varix-like lesion was found. Histological examination of the specimen revealed a benign schwannoma. An association with neurofibromatosis was ruled out. |
Conclusion | Schwannomas are well-defined, encapsulated, slowly progressive tumours that develop as eccentric growths from peripheral nerves. No single feature is pathognomonic but a multiplicity of clinical, imaging and surgical features points to the diagnosis of schwannoma. As 18 % of the schwannoma are associated with neurofibromatosis, a genetic workout should always follow the diagnosis. In the orbit schwannomas represent 1% to 2% of all tumours and malignant transformation is rare. |
Nom | KESTELYN |
Initiales | PA |
Institut | Dept. of Ophthalmology |
Ville | Ghent University Hospital |
Nom | De Potter |
Initiales | P |
Institut | Dept. of Ophthalmology |
Ville | Université Catholique de Louvain |
Nom | Decock |
Initiales | C |
Institut | Dept. of Ophthalmology |
Ville | Ghent University Hospital |