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Primary effusion lymphoma (PEL) is a rare B-cell neoplasm characterized by Kaposi sarcoma-associated herpesvirus (KSHV) infection of the tumor clone (Cesarman et al. 1995). The spectrum of KSHV-associated PEL is greater than originally appreciated and includes extracavitary KSHV-associated solid lymphoma (Chadburn et al. 2004). The classic form of PEL tends to remain localized to serous body cavities and diffusely spread along serous membranes. Radiologic and morphologic findings provide a basis for the recognition of PEL as a lymphoma of the serous membranes. However, extracavitary KSHV-associated solid lymphoma (solid PEL) involves mainly extranodal tissues.
Solid PEL is often associated with Kaposi sarcoma (KS) or multicentric Castleman disease (MCD). In rare cases, secondary distant (noncontiguous) lymphomatous effusions may develop, supporting the notion that extracavitary KSHV-associated solid lymphomas may represent a...
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References and Further Reading
Carbone, A., Cesarman, E., Gloghini, A., & Drexler, H. G. (2010). Understanding pathogenetic aspects and clinical presentation of primary effusion lymphoma through its derived cell lines. AIDS, 24, 479–490.
Carbone, A., Vaccher, E., Gloghini, A., Pantanowitz, L., Abayomi A, de Paoli, P., Franceschi S (2014) Diagnosis and management of lymphomas and other cancers in HIV-infected patients. Nature Reviews Clinical Oncology, 11, 223–238.
Cesarman, E., Chang, Y., Moore, P. S., Said, J. W., & Knowles, D. M. (1995). Kaposi’s sarcoma-associated herpesvirus-like DNA sequences in AIDS-related body-cavity-based lymphomas. New England Journal of Medicine, 332, 1186–1191.
Chadburn, A., Hyjek, E., Mathew, S., Cesarman, E., Said, J., & Knowles, D. M. (2004). KSHV-positive solid lymphomas represent an extra-cavitary variant of primary effusion lymphoma. American Journal of Surgical Pathology, 28, 1401–1416.
Fend, F., Cabecadas, J., Gaulard, P., Jaffe, E. S., Kluin, P., Kuzu, I., Peterson, L. A., Wotherspoon, A., & Sundström, C. (2012). Early lesion in lymphoid neoplasia. Journal of Hematopathology, 5, 169–199.
Gloghini, A., Volpi, C. C., Caccia, D., Gualeni, A. V., Cilia, A. M., Carbone, A., & Bongarzone, I. (2014). Primary effusion lymphoma: Secretome analysis reveals novel candidate biomarkers with potential pathogenetic significance. American Journal of Pathology, 184, 618–630.
Said, J. & Cesarman, E. (2017) Primary effusion lymphoma. In Swerdlow, S.H., Campo, E., Harris, N.L., Jaffe, E.S., Pileri, S.A., Stein, H., Thiele, J., Arber, T.A., Hasserjian, R.P., Le Beau, M.M., Orazi, A. & Siebert, R. eds. WHO classification of tumours of haematopoietic and lymphoid tissues (pp 323–324) Revised 4th ed. Lyon: IARC.
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Carbone, A., Gloghini, A. (2018). Primary Effusion Lymphoma (PEL). In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_3896-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_3896-1
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