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Atypical Midface Tumor Complicating Nevus of Ota

René Patzwahla, Klara Landaua and Spyros S. Kolliasa

a Institute of Neuroradiology and the Department of Ophthalmology, University Hospital of Zürich, Zürich, Switzerland



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  		FIG 1. 15-year-old boy who presented with a more than 8-year progressive exophthalmus of the left eye and a mass in the left oral cavity with a similar time course.

A, Contrast-enhanced axial 4-mm section through the skull base and orbits shows an abnormal strongly enhancing mass infiltrating the left cavernous sinus, superior orbital fissure, and orbit, leading to an exophthalmus and expansion of the superior orbital fissure.

B, Contrast-enhanced coronal 4-mm section reveals the infiltration and expansion of the left superior orbital fissure and the orbit; the masticator space, masseter, and temporal muscles are also infiltrated. The left maxillary sinus and the ethmoidal cells show a hypoplastic pattern.

C, Unenhanced coronal T1-weighted MR image corresponding to B shows short T1 signal intensity characteristics of the lesion.

D, Coronal 1.5 mm high-resolution CT section obtained through the skull base shows infiltration of the opening of the enlarged left round foramen to the pterygopalatine fossa (arrowhead), compared with the contralateral normal round foramen. The major sphenoid wing shows a hyperostotic pattern.



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  		FIG 2. Eight-year follow-up MR imaging. The now 23-year-old patient returned with a strong progression of the exophthalmus and nearly complete functional loss of the left eye.

A, Unenhanced T1-weighted MR image of the skull base and orbit reveals a further progression of the tumor with enlargement of the intraorbital component, leading to a stronger compression and stretching of the optic nerve and progression of the exophthalmus.

B, Corresponding T2-weighted section shows the typical short T2 signal intensity characteristics of this infiltrating process, indicating its melanocytic origin.

C, Contrast-enhanced coronal T1-weighted MR image demonstrates involvement of the ophthalmic (VI) and maxillary (VII) divisions of the left trigeminal nerve. The right nervus canalis pterygoidei is easily distinguished. On the left side, the nervus canalis pterygoidei is compressed by the more lateral VII.





Copyright © 2009 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X