Department of Oral Surgery and Pathology, School of Dentistry, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil.
Department of Oral Diagnosis, Area of Pathology, Piracicaba Dental School, University of Campinas, Piracicaba, Brazil.
Int J Surg Pathol. 2024 Feb;32(1):119-132. doi: 10.1177/10668969231167509. Epub 2023 May 7.
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.
原发性渗出性淋巴瘤（PEL）是一种侵袭性肿瘤，常发生于免疫抑制患者，表现为腹腔、胸腔或心包积液。这种高级别淋巴瘤与人类疱疹病毒 8（HHV8）感染密切相关，大多数病变中的肿瘤细胞还存在 EBV 感染，其缺乏 CD20 表达，呈现浆母细胞形态和表型。PEL 的腔外型或实体型更为罕见，通常累及淋巴结，目前被认为是经典 PEL 的一种临床表现。在口腔中，腔外型 PEL 极为罕见，既往仅报道过少数病例，且缺乏详细的临床病理描述。鉴于口腔黏膜中的浆母细胞淋巴瘤具有许多与 PEL 相同的临床、显微镜下和表型特征，因此，认识口腔腔外型 PEL 更为重要，病理医生需要在免疫抑制患者中寻找 HHV8，并进行适当的临床评估。本报告旨在描述一例罕见的发生于 36 岁患者腭部的腔外型 PEL，并复习关于这种侵袭性淋巴瘤的腔外型表现的文献。本报告强调了在具有浆母细胞特征的口腔淋巴瘤中寻找 HHV8 感染的重要性。
