Primary Signet-Ring/Histiocytoid Carcinoma of the Eyelid
Amanda Layne, Michael Richard, Julie Woodward, Alan D Proia, Thomas J Cummings. Duke University Health System, Durham, NC
Background: We present two cases of primary signet-ring/histiocytoid carcinomas of the eyelid. These are rare, aggressive tumors, with approximately thirty reported cases.
Design: Both cases were men, aged 60 and 69-years, who presented with non-painful thickening of the eyelid. Biopsies were submitted for histopathologic review with histochemial and immunohistochemial stains to further classify the neoplasm.
Results: Biopsies showed eyelid skin with a dermal proliferation of histiocytoid cells with eccentrically placed nuclei and eosinophilic cytoplasm. A moderate percentage of these cells had vacuoles in the cytoplasm typical of a signet- ring cell appearance. Tumor cells percolated through the dermis in a patchy pattern without forming a discrete mass. Mucicarmine histochemical stains highlighted the signet-ring cells, and by immunohistochemistry the signet-ring/histiocytoid cells were positive for cytokeratin 7, carcinoembryonic antigen (CEA), epithelial membrane antigen (EMA) gross cystic disease fluid protein-15 (GCDFP-15 / BRST-2), and, e-cadherin. The histological and immunohistochemical differential diagnosis includes metastatic signet-ring/histiocytoid carcinoma, especially histiocytoid lobular carcinoma from the breast, axillary skin, or other internal organs. Extensive systemic work-ups in both cases were negative for a primary site. Positron emission tomography and computerized tomography scans showed extensive orbital involvement. Orbital exenteration was performed in one case and showed diffuse involvement of upper and lower eyelids, conjunctiva, lacrimal gland, and orbit. Tumor cells focally involved the outer one-third of the sclera, but did not enter the eye.
Conclusions: We emphasize the patchy distribution, and in some small biopsies only a few tumor cells may be seen, giving the tissue the impression of being hypercellular. In these cases, immunostains for cytokeratin are useful.
Wednesday, March 21, 2012 1:00 PM
Poster Session VI # 316, Wednesday Afternoon