Abstract Purpose: Neural tumors of the orbit and eyelid are defined as proliferations of neurofibrous cells and/or Schwann cells. Merkel cell carcinomas (MCC) are rare neuroendocrine tumors that have characteristic histopathological features to differentiate them, particularly to metastatic disease. This series aims to identify clinical, epidemiological, and histopathological presentations to assess the implications for diagnosis, management, and prognosis. Materials and Methods: A retrospective analysis of 36 patients was obtained from the MUHC – McGill University Ocular Pathology and Translational Research Laboratory between 2009 and 2022. Demographic information regarding age, sex, and anatomical location was collected along with clinical and histopathological diagnosis. Results: Of the 36 tumors, 67% ( n = 24) of them affected females, 17% ( n = 7) had a right upper eyelid laterality, and the average age was 57 years. Seventeen percent ( n = 6) were correctly diagnosed clinically. Of the 36 tumors, 16 were neurofibromas. The average age was 53 years old, 63% were female ( n = 10), with the majority being on the eyelid ( n = 13). Schwannomas were diagnosed in 11 biopsies. The average age was 54 years, and the majority were females (82%, n = 9). Moreover, 6 MCC were identified histopathologically with panels of various immune markers. The average age was 77, where 67% ( n = 4) were female, and 50% ( n = 3) were found on the left lower eyelid. Conclusion: Neural tumors of the orbit and eyelid comprise a heterogeneous group of periocular lesions with variable clinical behavior which makes accurate clinicopathological and histopathological correlation essential for timely diagnosis and appropriate management.
Sebag et al. (Wed,) studied this question.