Primary CNS plasmablastic lymphoma: Report of a case with CSF cytology, flow cytometry, radiology, histological correlation, and review of the literature

Abstract

Plasmablastic lymphoma (PBL) is a rare subtype of diffuse large B cell lymphoma and commonly presents as an oral mass in HIV patients. Extraoral PBL has been reported, including one case of primary central nervous system PBL (PCNSPBL). The cytological features of PBL have been described, including cerebrospinal fluid (CSF) cytology findings for secondary CNS involvement by PBL. The etiology of PCNSPBL is still unknown. We report here the CSF cytology of a PCNSPBL, which shows a hypercellular specimen composed of markedly atypical, singly dispersed plasmacytoid cells with frequent abnormal mitoses and binucleation. The neoplastic cells are positive for CD138. Flow cytometry of the CSF specimen demonstrates a monoclonal neoplastic cell population, which is CD138 positive, kappa light chain positive, lambda light chain negative, and CD19 negative. Molecular analysis and immunohistochemical stains on a tissue biopsy confirmed the diagnosis and reveal concurrent infections with Epstein-Barr virus and human polyomavirus JC virus. Clinical and radiological correlations are reported, and the literature is reviewed. To the best of our knowledge, this is the first case report for CSF cytology of a PCNSPBL, demonstrating the utility of the cytological examination in the triage and diagnosis of this disease. Because of its dismal prognosis, it is critical for cytopathologists to be aware of the entity and recognize the neoplastic cells in CSF specimen. This report also emphasizes the importance of clinical and radiological correlation in the diagnosis of this lethal disease.

Extraoral plasmablastic lymphomas in a high human immunodeficiency virus endemic area

“…Plasmablastic lymphoma (PBL) is an aggressive disease that is highly associated with human immunodeficiency virus (HIV) infection and other immunodeficiencies, but it is also described in immunocompetent individuals (46%) . PBL usually occurs in the oral cavity and jaw, and less commonly in extraoral sites, mainly the gastrointestinal tract, skin, and lymph nodes . It is characterised by plasmablastic features, an immunophenotypic profile close to that of plasma cells, Epstein–Barr virus (EBV) positivity, and MYC dysregulation …”

Rare Lymphomas

“…In one series of 138 patients, all those who relapsed died from their lymphoma (Todeschini et al 2004 ), although another series from the MD Anderson Cancer Center had 42 % longterm survivors (Popat et al 1998 Rare cases have been reported in the brain parenchyma or ventricles (Kelley et al 2005 ) (Fig. Rare cases may demonstrate plasmablastic features (Urrego et al 2011 ). Their pathologic features are similar to non-CNS sites; however, their detailed histopathologic features are not well characterized.…”

Primary central nervous system plasmablastic lymphoma presenting in human immunodeficiency virus-negative but Epstein-Barr virus-positive patient: A case report

“…So far there were two cases of primary central neurous system PBL reports in the world. One case occurred in the right basal ganglian [1], another occurred in the left anterior frontal lobe [2]and both patients were HIV-positive. Here we report a HIV-negative but EBV-positive patient with a primary CNS plasmablastic lymphoma, a rare variant of DLBCL.…”