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A 67-year-old woman with a history of thyroid and ovarian cancer presented with recurrent right upper eyelid ptosis after 3 blepharoplasties. Slit-lamp exam revealed a superior subtarsal and subconjunctival lesion (25×15 mm). MRI showed eyelid and conjunctival involvement extending into the extraconal orbital space, lacrimal gland, and superior rectus muscle. Histopathology and immunohistochemistry confirmed extranodal marginal zone MALT lymphoma. She received 4 cycles of systemic R-CHOP immunochemotherapy, achieving complete lesion remission, though ptosis persisted. She remains under surveillance without recurrence. Conjunctival MALT lymphoma represents the most common malignant neoplasm of the conjunctiva. It generally exhibits an indolent clinical course and few symptoms, frequently resulting in delayed recognition. Recurrent eyelid ptosis is an uncommon presenting feature, underscoring the need for a high index of clinical suspicion. Definitive diagnosis depends on tissue biopsy followed by histopathologic and immunohistochemical characterization. Current therapeutic approaches include radiotherapy, chemotherapy, and biological agents, all of which achieve excellent rates of local control.