Abstract
Most cases of primary cutaneous signet ring cell carcinoma develop in the eyelid.
Clinically, eyelid lesions had been often misinterpreted as inflammatory disorders, such as chronic blepharoconjunctivitis or chalazion.
Histopathologically, primary cutaneous signet ring cell carcinoma appears as a dermal neoplasm composed of single scattered cells, small strands, or solid aggregations of neoplastic epithelial cells, interstitially arranged between the collagen bundles of the dermis. Neoplastic cells may show a signet ring or histiocytoid appearance.
Before establishing a diagnosis of primary cutaneous signet ring carcinoma, the possibility of a metastasis should be ruled out.
Immunohistochemically, neoplastic cells of primary cutaneous signet ring cell carcinoma express CAM 5.2, CK7, high-molecular weight CK, AE1/AE3, and MNF116 cytokeratins, CEA, EMA, GCDFP-15, p63, mucin-1 (MUC-1), BerEP4, E-cadherin MUC-2, podoplanin, mammaglobin, and N-cadherin. Estrogen and progesterone receptors show positive results in some cases and negative results in others. p63 and podoplanin are more frequently positive in primary signet ring cell carcinoma of the eyelid than in metastatic signet ring carcinoma of the eyelid.
Primary cutaneous signet ring cell carcinoma is a neoplasm with a high potential to produce distant metastasis.
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Requena, L., Sangüeza, O. (2017). Primary Cutaneous Signet Ring Cell Carcinoma. In: Cutaneous Adnexal Neoplasms. Springer, Cham. https://doi.org/10.1007/978-3-319-45704-8_30
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DOI: https://doi.org/10.1007/978-3-319-45704-8_30
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