Primary signet ring cell carcinoma of the eyelid - Report of a case demonstrating further analogy to lobular carcinoma of the breast with a literature review

Primary signet ring cell carcinoma of the eyelid is a rare neoplasm with le ss than ten cases described. This report details another case, which shows further parallels to lobular carcinoma of the breast, and reviews the liter ature on this subject. A 73-year-old white female presented with diffuse in duration of her left eyelids. Histopathology revealed a delicate infiltrate of epithelial cells scattered throughout the lid stroma in a nondestructiv e pattern. The cells were relatively monomorphous and showed only mild atyp ia and rare mitotic figures. Many had slightly granular amphophilic cytopla sms, others showed distinct signet ring cell morphology, and all were stron gly positive for epithelial mucin. Immunomicroscopy showed strong reactivit y for estrogen receptor (ER), progesterone receptor (PR) and gross cystic d isease fluid protein-15 (GCDFP-15), and was negative for Her-2/neu (erb-2) and cytokeratin 20. An extensive workup for other primary sites was negativ e. Orbital exenteration showed extensive involvement of both lids and soft tissue, including diffuse muscle and lacrimal gland infiltration. In the br east, signet ring cell carcinoma is considered a variant of lobular carcino ma. The delicate infiltrating pattern in our case and the ER+, PR+, GCDFP-1 5+, Her-2/neu- phenotype further strengthen this analogy. Together, these d ata also support apocrine differentiation of primary eyelid signet ring cel l carcinoma.