Primary central nervous system lymphoma
Franco Cavalli, Harald Stein, Emanuele Zucca in Extranodal Lymphomas, 2008
Primary central nervous system lymphoma (PCNSL), a rare form of extranodal lymphoma, occurs in the brain, leptomeninges, spinal cord, or eyes; typically, it remains confined to the CNS,1,2 and accounts for 3.1% of all primary brain tumors.3 Its incidence increased nearly 3-fold between 1973 and 1984,4 but, recent data suggest that it may be stabilizing or declining slightly.5 Congenital or acquired immunodeficiency is the only established risk factor; persons infected with the human immunodeficiency virus (HIV) have a 3600-fold increased risk of developing PCNSL compared with the general population.6 In HIV-infected patients, a CD4+ count <50 cells/μl and a high peripheral HIV viral load are risk factors for PCNSL development. More than half of patients will have had an acquired immunodeficiency syndrome (AIDS)-defining illness prior to the development of PCNSL, offering additional evidence that immune function must be markedly impaired before this lymphoma arises. AIDS–PCNSL is usually associated with Epstein–Barr virus (EBV);5 this infection and the c-myc translocation result in the development and proliferation of PCNSL. With the advent of highly active antiretroviral therapy (HAART), the incidence of AIDS-related PCNSL has declined.7
AIDS-Related Malignancy
Pat Price, Karol Sikora in Treatment of Cancer, 2020
Primary central nervous system lymphoma (PCL) is defined as non-Hodgkin lymphoma (NHL) that is confined to the cranio-spinal axis without systemic involvement. This diagnosis is rare in immunocompetent patients but occurs more frequently in patients with both congenital and acquired immunodeficiency. Since 1985 high-grade B-cell NHL including PCL has been an AIDS-defining diagnosis. AIDS-related PCL occurs equally frequently across all ages and transmission risk groups. A meta-analysis of cohort studies has shown a significant decline in the incidence of PCL following the introduction of highly active antiretroviral therapy (HAART) (relative risk 0.42)30 which is attributed to the protective effects of HAART.31
Neurosurgery: Supratentorial tumors
Hemanshu Prabhakar, Charu Mahajan, Indu Kapoor in Essentials of Geriatric Neuroanesthesia, 2019
Primary central nervous system lymphoma (PCNL) are a relatively uncommon form of extranodal non-Hodgkin lymphoma (Figure 5.3). They are highly aggressive, may be solitary or multiple, and are located superficially in the cortex or may have a deep periventricular location. Typically, they are composed of noncohesive neoplastic lymphocytes that diffusely infiltrate the neural parenchyma and the blood vessels. The prognosis is poor, with an average survival time of approximately a year, despite optimal therapy (1) Tumor resection offers no benefit, except in the rare circumstances of neurologic deterioration due to brain herniation (1). These patients usually undergo a diagnostic biopsy followed by chemoradiotherapy.
Long-Term Evaluation of Combination Treatment of Single Agent HD-MTX Chemotherapy up to Three Cycles and Moderate Dose Whole Brain Irradiation for Primary CNS Lymphoma
Published in Journal of Chemotherapy, 2019
Hiroyuki Kobayashi, Shigeru Yamaguchi, Hiroaki Motegi, Sadahiro Kaneko, Shogo Endou, Rikiya Onimaru, Shunsuke Terasaka, Kiyohiro Houkin
Primary central nervous system lymphoma (PCNSL) is a rare malignant tumor arising in the brain, spinal cord, eyes, or leptomeninges. The histopathological diagnosis of PCNSL is diffuse large B-cell lymphoma (DLBCL) in more than 90% of patients. For the therapeutic management of PCNSL, several previous trials have indicated the efficacy of high-dose methotrexate (HD-MTX) monotherapy in combination with whole brain radiotherapy (WBRT), resulting in median survival of 33–42 months.1–3 This regimen had been thus regarded as basic treatment for PCNSL. To date, several clinical trials have investigated modified MTX-based chemotherapy regimens, such as dose escalation and/or combination with other agents, to increase survival and reduce radiation-induced neurotoxicity.4–10 However, few studies have prospectively verified the protocol of single-agent MTX and WBRT.11 As treatment of PCNSL becomes increasingly aggressive, the risk of adverse events increases in addition to prolonged treatment period and increased treatment costs. It is therefore appropriate to reassess the efficacy of this basic regimen to determine whether increasingly aggressive treatment is justified. The aim of this retrospective study was therefore to evaluate the long-term effectiveness of combination therapy consisting of a minimized number of HD-MTX cycles and moderate dose of WBRT in patients with newly diagnosed PCNSL.
A case of primary central nervous system lymphoma presenting as a shunt complication
Published in British Journal of Neurosurgery, 2023
Roberto J. Perez-Roman, Zachary S. Hubbard, G. Damian Brusko, Robert M. Starke
Primary central nervous system lymphoma (PCNSL) accounts for 3–5% of primary brain tumors, with an annual incidence of five per one million person-years.1–3 It is a category of extranodal non-Hodgkin lymphoma located within the brain, eyes, leptomeninges, and spinal cord.1–3 In 95% of cases, PCNSL consists of diffuse, large B-cell infiltrates. The remaining cases are either low-grade B-cell or T-cell origin.1,3 PCNSL is usually associated with immunosuppression but has recently been found with increasing frequency among immunocompetent individuals.4 Patients typically present with generalized symptoms such as headache, nausea, vomiting, and confusion.5 Despite advances in radiologic techniques, the diagnosis of PCNSL remains a challenge. In this article, we describe a case of periventricular PCNSL in an 82-year-old female presenting as a shunt malfunction.
Optic nerve biopsy in leukemic infiltrative optic neuropathy: a case report and review of the literature
Published in Orbit, 2022
Paul D. Chamberlain, Christopher R. Dermarkarian, M. Brent Woodland, Richard C. Allen, Nagham Al-Zubidi
Table 1 summarizes cases in the current literature where an optic nerve biopsy was performed to aid in the diagnosis of leukemia or lymphoma.3,5–14 Including the current case, 13 optic nerve biopsy cases were reported. Of note, this does not include cases where only optic nerve sheath biopsy was performed. Seven had known leukemia/lymphoma of which six were in clinical remission at the time of presentation. Five were an initial presentation of primary central nervous system lymphoma. Of the 13 patients, 7 had NLP vision, 3 had LP, HM, or CF vision, and 2 had 20/80 vision at the time of biopsy (1 not reported). Of the five patients with LP vision or better prior to biopsy, at least three had CF vision or better following biopsy (2 not reported).
Related Knowledge Centers
- Brain Tumor
- Burkitt Lymphoma
- Cd4
- Central Nervous System
- Immunosuppression
- Immunodeficiency
- HIV/AIDS
- Lymphoma
- HIV
- Epstein–Barr Virus