AIDS-Related Primary Central Nervous System Lymphoma
Section snippets
Epidemiology and the link with Epstein-Barr virus
Patients who have AIDS have a markedly greater risk of developing PCNSL compared with the general population. Patients who have AIDS PCNSL are more likely to be men, with a median age of presentation in the late fourth decade as opposed to the sixth or seventh decade in immunocompetent patients [4]. In the period before highly active antiretroviral therapy (HAART), the relative risk of PCNSL was estimated to be over 1000-fold in HIV-infected individuals and as great as 3600-fold [14]. Since the
Histopathology
Nearly all PCNSLs are of B-cell origin. In the AIDS setting, diffuse large B-cell lymphoma and its immunoblastic variant are the dominant morphologies; small noncleaved cell lymphoma (Burkitt's or Burkitt-like) and other morphologies are less frequent [4]. Polymorphous or atypical features are common, making subclassification at times difficult [4]. The malignant lymphocytes are characterized by a high mitotic index and a diffuse growth pattern, classically with an angiocentric distribution and
Radiographic features
The radiographic appearance of PCNSL can differ substantially in the AIDS versus non-AIDS setting. Differentiating PCNSL from toxoplasmosis is one of the most frequently encountered diagnostic challenges in the patient with AIDS who has an intracranial mass lesion, particularly if the process appears multifocal.
Baseline Evaluations
Because AIDS-related systemic lymphomas have a predilection for extranodal dissemination, including the central nervous system (CNS), evaluation for systemic lymphoma in the patient who has AIDS PCNSL was once routinely recommended [6]. In patients who have AIDS PCNSL, however, evaluation for occult systemic lymphoma—including CT scan of the chest, abdomen, and pelvis and bone marrow biopsy—has a low diagnostic yield [6]. This finding was confirmed in a multicenter study of AIDS PCNSL in which
Treatment options
The median survival of patients who have AIDS PCNSL is 1 to 2.5 months with supportive care alone [11], [70]. Palliative whole-brain radiation is the mainstay of managing most patients because surgical resection is not curative and there are barriers to chemotherapy.
Epstein-Barr virus–targeted therapies
The consistent association of EBV and PCNSL suggests the possibility that the virus might in some way be a target for therapeutic interventions. This idea has attracted considerable attention but clinical successes have been limited at best. In this section, the authors consider various approaches to targeting the virus and what limits their application to AIDS PCNSL. Three general approaches have been explored: immune targeting of viral antigens, disruption of viral functions, and the use of
Summary
Because survival of patients infected with HIV has improved with HAART, more patients diagnosed with PCNSL may become candidates for chemotherapy. The role of chemotherapy is far from established, however, and there are clear limitations to dose-intensive approaches in the setting of advanced HIV infection. Because of the rarity of this lymphoma, multicenter collaborations are needed to address these and other fundamental management questions. Further insights into the biology of AIDS PCNSL may
Acknowledgments
The authors thank Adam El-Dieb, MD, Ayse Aralasmak, MD, and William Lavely, MD, for assistance with the neuroimages, and Sharon Swierczynski, MD, PhD, and Peter Burger, MD, for assistance with the pathology slides.
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Cited by (48)
Neoplasms of the cranial nerves
2022, Handbook of Neuro-Oncology NeuroimagingLessons from Epstein-Barr virus DNA detection in cerebrospinal fluid as a diagnostic tool for EBV-induced central nervous system dysfunction among HIV-positive patients
2022, Biomedicine and PharmacotherapyCitation Excerpt :A diagnosis of CNS lymphoma among HIV-positive patients is strongly correlated with the existence of EBV-DNA in CSF [52]. A cut-off value ranging from 200 copies/mL to 2000 copies/mL could improve the specificity for PCNSL and systemic ARL [59]. Among cases without clinical and radiological features of CNS lymphoma, the significance of detectable CSF EBV-DNA for predicting subsequent tumor development is inconclusive [52,58].
Differentiation of Acquired Immune Deficiency Syndrome Related Primary Central Nervous System Lymphoma from Cerebral toxoplasmosis with Use of Susceptibility-Weighted Imaging and Contrast Enhanced 3D-T1WI
2021, International Journal of Infectious DiseasesCitation Excerpt :Cerebral toxoplasmosis is the most common cause of expansive brain lesions in people living with HIV/AIDS (Vidal, 2019), and the incidence may be as high as 40% (Lee SB and Lee TG, 2017). Acquired immune deficiency syndrome-related primary central nervous system lymphoma (AR-PCNSL) is second only to toxoplasmosis as the most common intracranial mass lesion in adult patients (Kasamon and Ambinder, 2005). The initial diagnosis of cerebral toxoplasmosis is usually made empirically if multiple ring-enhancing lesions are present on brain MRI, the blood serology is positive for Toxoplasma gondii, and clinical-radiological improvement after anti-Toxoplasma therapy is observed (Benson et al., 2018).
Primary CNS lymphoma in HIV infection
2018, Handbook of Clinical NeurologyNeoplasms of the Cranial Nerves
2016, Handbook of Neuro-Oncology Neuroimaging: Second Edition
Supported in part by grants P50 CA96888 and UO1 CA70062 to R.F. Ambinder.