Effective diagnosis and surgical management of primary signet ring cell carcinoma of the eyelid

Primary signet ring cell carcinoma of the eyelid is an aggressive and rare neoplasm. It generally develops from the eccrine sweat gland and diffuses to the dermis and subcutis of the eyelid or axillae. The lesion usually presents as a progressive, diffuse and painless swelling of the eyelid with or without erythema and is frequently misdiagnosed as a chalazion or a chronic blepharoconjunctivitis leading to delayed management. The histology typically shows a signet ring cell or histiocytoid morphology. The authors present a case of a 76-year-old woman with a diagnosis of a primary signet ring cell carcinoma of the eyelid with atypical presentations. With this report, we seek to increase clinician awareness toward this tumor and to highlight the need for systematic recommendations in order to improve the management of these patients.