Kovatch Kevin J, Purkey Michael T, Martinez-Lage Maria, Gausas Roberta E, Loevner Laurie A, Grady M Sean, O'Malley Bert W, Rassekh Christopher H
Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
Department of Otorhinolaryngology.
Laryngoscope. 2015 Nov;125(11):2457-60. doi: 10.1002/lary.25232. Epub 2015 May 9.
Neurofibromatosis types 1 (NF-1) and 2 are significant entities to otolaryngologists because they frequently involve head and neck skin, brain, skull base, orbits, and surrounding neural and vascular structures. We present the case of a 52-year-old male with progressive, marked unilateral proptosis due to a multilobulated orbital mass, secondary to biopsy-proven plexiform neurofibroma (PN). Acute worsening of proptosis leading to corneal abrasion, diplopia, and pain required debulking surgery, for which an orbitozygomatic approach was utilized. Genetic testing for NF-1 revealed no mutation. This rare case of NF-negative orbital PN and multidisciplinary treatment considerations for expansile orbital tumors are discussed.
