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Bleeding eyelid with a diagnostic dilemma

© BMJ Publishing Group Limited 2021. No commercial reuse. See rights and permissions. Published by BMJ. DESCRIPTION A 70yearold Asian woman presented with a rapidly growing swelling in her left eyelid for the last month. It was associated with multiple bouts of spontaneous bleeding which resolved on the application of pressure. Clinical examination revealed a rapidly growing, violaceous mass lesion associated with softtissue ulceration and destruction of the tarsal plate (figure 1A). The mass effect resulted in ptosis of the upper eyelid. However, the vision was preserved in her left eye. The mass was painful and extremely friable. The contrastenhanced computed tomography scan of the head and neck region did not show any lymphadenopathy. Fineneedle aspiration cytology of the swelling suggested either an appendageal tumour or a nonHodgkin’s lymphoma. She underwent a wide local excision (WLE) with circumferential 5 mm margins to ascertain the definitive diagnosis and to control bleeding. The surgical defect was reconstructed via Fricke flap—a temporally based monopedicle forehead transposition flap. The postoperative period was uneventful (figure 1B). Histological analysis (figure 2A) revealed tumour cells arranged in an organoid pattern, cords, islands and sheets with brisk mitosis (4–5/HPF). On immunohistochemistry, tumour cells showed CD56 and chromogranin positivity with focal positivity for synaptophysin (figure 2B). A Ki-67 labelling index of 80% was noted. The distance from the closest margin was 3 mm. She was diagnosed as Merkel cell carcinoma (MCC) of the left upper eyelid, stage IIA (pT2N0M0) as per AJCC eighth edition. She received adjuvant radiotherapy following a discussion in the institutional multidisciplinary tumour board. She is currently disease free after 10 months of followup and is satisfied with the aesthetic outcomes. MCC is a rare and aggressive neuroendocrine malignancy with male preponderance and predilection for Caucasians than other ethnicities. An association with Merkel cell polyomavirus has been reported in 60%–80% of specimens, especially of the old and immunosuppressed patients. About 46%–48% of all carcinomas present in the head and neck region of which 5%–20% epicentre in the eyelids. The eyelid MCC typically affects the upper eyelids especially near its margin and is associated with madarosis, skin ulceration, and distortion of the normal architecture. The tumours are usually misdiagnosed as cysts, chalazia or basal cell carcinomas. WLE with 5 mm margins is considered as an adequate margin to control the primary tumour when located in the periocular region contrary to 2.5–3 cm margins, as is used for cutaneous MCC elsewhere on the body. This is essential to preserve the integrity of as many facial aesthetic subunits as possible without any compromise on oncological outcomes. Moreover, large postresection defects also pose a reconstructive challenge.