Malignant transformation of metachronous bilateral Schneiderian inverted papilloma of the lacrimal sac: management considerations and the contentious issue of orbital exenteration

Abstract

The authors present an unusual case of malignant transformation of metachronous bilateral Schneiderian inverted papilloma (IP) of the lacrimal sac. Such pathology has been sparingly covered in prior published literature, in particular with little formal discussion surrounding its optimal management. We describe the clinical presentation, course and management in a 35-year-old male with histopathological diagnosis of Squamous cell carcinoma (SCC) arising within IP. He underwent radical surgery, including orbital exenteration and medial maxillectomy. Our patient re-presented with IP in the contralateral side and subsequent malignant transformation to SCC with disseminated disease. Unfortunately he succumbed to disease approximately 20 months following initial surgery. This case raises several important questions with regard to appropriate treatment. In particular, we discuss the various management options with special consideration given to the contentious topic of orbital exenteration in such patients. We emphasise two particular controversial issues, namely, oncological efficacy of orbital preservation and the functional compromise that may result in the preserved eye.