Primary effusion lymphoma (PEL) is an aggressive neoplasm often diagnosed in immunosuppressed patients demonstrating peritoneal, pleural, or pericardial effusions. This high-grade lymphoma is strongly associated with human herpesvirus 8 (HHV8) infection and most of the lesions also show the presence of Epstein-Barr virus in tumor cells, which lacks CD20 expression and reveals a plasmablastic morphology and phenotype. The extracavitary or solid variant of PEL is even rarer and usually affects the lymph nodes and is currently considered a clinical manifestation of the classic PEL. In the oral cavity, extracavitary PEL is extremely rare and only a few patients have been previously reported, with no detailed clinicopathological description. The recognition of oral extracavitary PEL is even more important given the occurrence of plasmablastic lymphoma in the oral mucosa, which shares many clinical, microscopic, and phenotypic features with PEL, therefore, demanding from pathologists the search for HHV8, especially in immunosuppressed patients, and an appropriate clinical evaluation. In this report, we aim to describe a very rare extracavitary PEL affecting the palate of a 36-year-old patient and to review the literature regarding the extracavitary presentation of this aggressive lymphoma. This report demonstrates the importance of searching for HHV8 infection in oral lymphomas with plasmablastic features.
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http://dx.doi.org/10.1177/10668969231167509 | DOI Listing |
Am J Dermatopathol
January 2025
Department of Dermatology, Columbia University Medical Center, New York, NY; and.
Primary effusion lymphoma (PEL) is a rare and aggressive B-cell lymphoma typically associated with human herpesvirus 8 (HHV-8) and Epstein-Barr virus infections. It classically presents as a malignant effusion in body cavities, but rarely presents with an extracavitary variant characterized by solid tumors in lymph nodes or extranodal sites such as the gastrointestinal tract, skin, lungs, and nervous system. This case report describes an unusual presentation of primary cutaneous extracavitary PEL in an HIV-positive patient that has only been reported in 8 cases previously.
View Article and Find Full Text PDFCureus
December 2024
Infectious Diseases, Hospital Garcia de Orta, Lisbon, PRT.
Extra-cavitary primary effusion lymphoma (PEL), often associated with human herpes virus 8 (HHV8) infection, represents a rare and aggressive form of non-Hodgkin lymphoma, which is predominantly found in individuals with severe immunosuppression. As an acquired immunodeficiency syndrome (AIDS)-associated lymphoma, PEL typically manifests in the context of advanced human immunodeficiency virus (HIV) infection, requiring tailored therapeutic approaches to manage both the lymphoma and underlying immunodeficiency. A 53-year-old male patient from Cape Verde presented with a three-day history of fever, night sweats, right iliac fossa pain, hematochezia, and an unintentional weight loss of five kilograms over the previous two months.
View Article and Find Full Text PDFActas Dermosifiliogr
November 2024
Universidad de Zaragoza, Zaragoza, España; Servicio de Anatomía Patológica, Hospital Clínico Universitario Lozano Blesa, Zaragoza, España.
Epstein Barr virus (EBV) positive B lymphoproliferative disorders (LPD) with cutaneous involvement include a series of rare entities that go from indolent processes to aggressive lymphomas. B-cell EBV+ LPD mainly affect immunocompromised patients while T-cell EBV+ LPD are more prevalent in specific geographic regions such as Asia, Central America, and South America. Since the latest WHO-EORTC classification of cutaneous lymphomas in 2018, significant changes have been included in the new classifications of hematological malignancies.
View Article and Find Full Text PDFHematol Transfus Cell Ther
December 2024
Albert Einstein Israeli Faculty of Health Sciences, São Paulo, SP, Brazil.
Primary effusion lymphoma (PEL) is an aggressive and rare type of diffuse large B-cell lymphoma (DLBL) that commonly presents itself as pleural, pericardial or peritoneal effusion without lymph node or extranodal involvement in immunosuppressed patients, such as HIV-positive or transplanted receptors. On rare occasions, it may be found in solid sites without effusion, in an immunophenotypically and morphologically similar neoplasm well-known as extracavitary PEL (EC-PEL). Both PEL and EC-PEL are associated with extremely poor prognosis.
View Article and Find Full Text PDFCNS Oncol
June 2024
Department of Pathology & Laboratory Medicine, Emory University, Atlanta, GA 30322, USA.
Primary effusion lymphoma (PEL) is an uncommon B-cell lymphoma associated with human herpesvirus 8 and comprises 3-4% of all HIV-related lymphomas. It traditionally presents as a pleural, pericardial, and/or peritoneal effusion, though it can occasionally manifest as an extracavitary or solid mass in the absence of an effusion. The extracavitary or solid variant of primary effusion lymphoma has been reported in the skin, gastrointestinal tract, lung, and lymph nodes.
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