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Signet ring cell carcinoma of the eyelid – the monocle tumour: Case report

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Mortensen AL, Heegaard S, Clemmensen O, Prause JU. Signet ring cell carcinoma of the eyelid—the monocle tumour. APMIS 2008;116:326–32.

We report the clinical and histopathological characteristics of two cases of signet ring cell carcinoma of the eye lids, and discuss the histogenesis of this neoplasm. Two 72-year-old Caucasian males both presented with slowly growing tumours of the eyelids. The tumours were excised and specimens were examined using light- and transmission electron microscopic techniques. Clinically, the tumours infiltrated both eyelids on one side of the face with swelling and periocular inflammation, creating a monocle-like appearance. Extensive clinical work-up excluded periocular metastases. Histopathologically, the tumours were composed of rather bland cells with mainly histiocytoid morphology. A minor proportion had a signet ring cell appearance. The cytoplasmic inclusions giving the signet ring morphology were PAS- and colloidal iron positive. The tumour cells reacted with antibodies against cytokeratins, carcinoembryonic antigen, epithelial membrane antigen, gross cystic disease fluid protein-15 and lysozyme. Transmission electron microscopy demonstrated tumour cells containing intracytoplasmic vacuoles lined by microvilli. The tumour cells aggregated in duct-like clusters. A diagnosis of primary signet ring cell carcinoma was made in both cases. Histopathological, immunohistological and ultrastructural findings indicated that the tumours were of sweat gland origin.

Keywords: Eye lid; monocle tumour; primary signet ring cell carcinoma; sweat gland

Document Type: Case Report


Affiliations: 1: Institute for Neuroscience and Pharmacology, Section of Eye Pathology, University of Copenhagen 2: Department of Clinical Pathology, Odense University Hospital, Denmark

Publication date: April 1, 2008


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