Orbital cholesterol granuloma and cholesteatoma: significance of differentiating the two

Cholesteatoma and cholesterol granuloma are relatively rare lesions of the orbit. Both may involve the superior orbit and present with globe ptosis, proptosis, and double vision developing over weeks to years. In addition to their etiology being unknown, the nomenclature regarding these tumors historically has been confusing, with a variety of names being used to describe them. Their histopathology shares numerous similarities but has one key distinguishing feature: The cholesterol granulomas lack an epithelial lining, whereas the cholesteatomas have an epithelial lining. Surgical removal is the treatment of choice for both tumors. It is important to differentiate the two as the prognosis with recurrence differs. Cholesteatoma may recur with the possibility of malignancy found at the time of re-exploration. We present a case of each tumor type, illustrating their similarities and highlighting the histopathologic findings and treatment recommendations.