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Merkel cell carcinoma: recent progress and current priorities on etiology, pathogenesis, and clinical management
Journal of Clinical Oncology, 07/17/09
Rockville Merkel Cell Carcinoma Group - In a study to expedite improved understanding, diagnosis, treatment, and prevention of Merkel cell carcinoma (MCC), it appears that development and validation of a range quantitative polymerase chain reaction and serologic assays for detection of MCPyV, as well as an infectious clone of the virus, would clarify the fundamental biology, natural history, and epidemiology of the virus, of MCC, and of other diseases.
Methods- This article summarizes a workshop that discussed state-of-the-art research and priorities for research on MCC and on a new human polyomavirus (ie, MCPyV) recently discovered in 80% of MCC tumors.
- Normal Merkel cells are widely distributed in the epidermis near the end of nerve axons and may function as mechanoreceptors or chemoreceptors.
- Malignant MCC cells typically stain for cytokeratin 20 as well as for other epithelial and neuroendocrine markers.
- MCC subtypes, which are based on histology, on cell line growth properties, and on gene expression profiles, have been reported but have not been linked to prognosis.
- Clinical management has been empiric.
- MCPyV is clonally integrated at various sites in the human genome of MCC tumors, with truncating mutations in the viral, large T antigen gene that interrupt viral replication.
- MCPyV seroprevalence may be high, as with previously known human polyomaviruses.
- MCC risk is increased 11-fold with AIDS and with other cell-mediated immune deficiencies, B-cell neoplasms, and ultraviolet radiation exposure.
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