Syringocystadenocarcinoma Papilliferum of the Eyelid: A Rare Case Report and Review of the Literature

Syringocystadenocarcinoma papilliferum is an exceptionally rare malignant adnexal neoplasm of sweat gland origin, with fewer than 90 cases reported in the literature, most arising within the head and neck region. Periocular involvement is exceedingly uncommon, with only 3 previously documented cases, one of which was confined to the eyelid. The authors describe an 80-year-old man who presented with new onset erythema, irritation, and growth of a long-standing upper eyelid lesion. Incisional biopsy was interpreted as squamous cell carcinoma with papillary morphology. Subsequent excision and histopathologic examination of the entire lesion led to a diagnosis of syringocystadenocarcinoma papilliferum arising in a background of syringocystadenoma papilliferum. This case adds to the limited literature of syringocystadenocarcinoma papilliferum in the periocular region, highlighting the clinical presentation, pathologic features, and biologic behavior of this rare tumor in the ocular adnexa.