Primary Central Nervous System Lymphoma: Treatment and Nursing Management of Immunocompetent Patients

Abstract Background Corticosteroid therapy (CST) prior to biopsy may hinder histopathological diagnosis in primary central nervous system lymphoma (PCNSL). Therefore, preoperative CST in patients with suspected PCNSL should be avoided if clinically possible. The aim of this study was thus to analyze the difference in the rate of diagnostic surgeries in PCNSL patients with and without preoperative CST. Methods A multicenter retrospective study including all immunocompetent patients diagnosed with PCNSL between 1/2004 and 9/2018 at four neurosurgical centers in Austria was conducted and the results were compared to literature. Results A total of 143 patients were included in this study. All patients showed visible contrast enhancement on preoperative MRI. There was no statistically significant difference in the rate of diagnostic surgeries with and without preoperative CST with 97.1% (68/70) and 97.3% (71/73), respectively (p = 1.0). Tapering and pause of CST did not influence the diagnostic rate. Including our study, there are 788 PCNSL patients described in literature with an odds ratio for inconclusive surgeries after CST of 3.3 (CI 1.7–6.4). Conclusions Preoperative CST should be avoided as it seems to diminish the diagnostic rate of biopsy in PCNSL patients. Yet, if CST has been administered preoperatively and there is still a contrast enhancing lesion to target for biopsy, surgeons should try to keep the diagnostic delay to a minimum as the likelihood for acquiring diagnostic tissue seems sufficiently high.

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Primary leptomeningeal lymphoma masquerading as infectious tubercular meningitis

BMJ Case Reports ◽

10.1136/bcr-2021-243574 ◽

2021 ◽

Vol 14 (9) ◽

pp. e243574

Author(s):

Salini Sumangala ◽

Thidar Htwe ◽

Yousuf Ansari ◽

Lidia Martinez- Alvarez

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Correct Diagnosis ◽

Central Nervous System Lymphoma ◽

Final Diagnosis ◽

Csf Analysis ◽

Antitubercular Treatment ◽

Lymphoma Treatment ◽

Work Up ◽

Timely Treatment

Primary central nervous system lymphoma (PCNSL) is infrequent and often poses diagnostic conundrums due to its protean manifestations. We present the case of a South Asian young man presenting with raised intracranial pressure and a lymphocytic cerebrospinal fluid (CSF) with pronounced hypoglycorrhachia. Progression of the neuro-ophthalmic signs while on early stages of antitubercular treatment led to additional investigations that produced a final diagnosis of primary leptomeningeal lymphoma. Treatment with chemoimmunotherapy (methotrexate, cytarabine, thiotepa and rituximab (MATRix)) achieved full radiological remission followed by successful autologous transplant. This case highlights the difficulties and diagnostic dilemmas when PCNSL presents as a chronic meningeal infiltrative process. While contextually this CSF is most often indicative of central nervous system tuberculosis and justifies empirical treatment initiation alone, it is essential to include differential diagnoses in the investigation work-up, which also carry poor prognosis without timely treatment. High suspicion, multidisciplinary collaboration and appropriate CSF analysis were the key for a correct diagnosis.

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PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMAS

Romanian Journal of Neurology ◽

10.37897/rjn.2015.2.3 ◽

2015 ◽

Vol 14 (2) ◽

pp. 77-84

Author(s):

Adriana Octaviana Dulamea ◽

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Treatment Strategies ◽

Central Nervous System Lymphoma ◽

B Cell Lymphoma ◽

Diagnostic Methods ◽

Hematopoietic Stem ◽

Whole Brain Radiation ◽

Author Review ◽

Immunocompetent Patients

Primary central nervous system lymphoma (PCNSL) is an aggressive non-Hodgkin B-cell lymphoma with characteristic clinical behavior, biological features and poor prognosis despite complex treatment. PCNSL has a median survival of 17 to 45 months in immunocompetent patients, and only 20-30% of cases can be cured successfully. Clinical outcome has improved since the advances in combination chemotherapy protocols, addition of whole brain radiation therapy, encouraging responses of rituximab administration in refractory PCNSL and autologous hematopoietic stem-cell transplantation as consolidative therapy. The author review the recent data on pathogenesis, diagnostic methods and treatment strategies of PCNSL in immunocompetent patients.

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Serial case report: Primary central nervous system lymphoma in immunocompetent patients

Journal of the Neurological Sciences ◽

10.1016/j.jns.2019.10.564 ◽

2019 ◽

Vol 405 ◽

pp. 73

Author(s):

D. Rahmawati ◽

R. Rachmasari

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Case Report ◽

Central Nervous System Lymphoma ◽

Immunocompetent Patients

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Recurrent genetic alterations in primary central nervous system lymphoma of immunocompetent patients.

Journal of Clinical Oncology ◽

10.1200/jco.2012.30.15_suppl.2023 ◽

2012 ◽

Vol 30 (15_suppl) ◽

pp. 2023-2023

Author(s):

Alberto Gonzalez ◽

Ahmed Idbaih ◽

Blandine Boisselier ◽

Anne Jouvet ◽

Marc Polivka ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Somatic Mutations ◽

Hot Spot ◽

Quantitative Polymerase Chain Reaction ◽

Snp Array ◽

Central Nervous System Lymphoma ◽

Genetic Alterations ◽

High Dose ◽

Immunocompetent Patients

2023 Background: Little is known about the molecular pathogenesis of primary central nervous system lymphoma (PCNSL) in immunocompetent patients. Our objective was to identify the genetic changes involved in PCNSL oncogenesis and evaluate their clinical relevance. Methods: Twenty nine and four newly diagnosed, HIV-negative PCNSL patients were investigated using high-resolution single nucleotide polymorphism (SNPa) arrays (Infinium Illumina Human 610-Quad SNP array-Illumina; validated by real-time quantitative polymerase chain reaction) and whole-exome sequencing respectively. Molecular results were correlated with prognosis. Results: All PCNSLs were diffuse large B-cell lymphomas, and the patients received high-dose methotrexate-based polychemotherapy without radiotherapy as an initial treatment.SNPa analysis revealed recurrent large and focal chromosome imbalances that target candidate genes in PCNSL oncogenesis. The most frequent genomic changes were (i) 6p21.32 loss (79%), corresponding to the HLA locus; (ii) 6q loss (27-37%); (iii) CDKN2A homozygous deletions (45%); (iv) 12q12-q22 (27%); (v) chromosome 7q21 and 7q31 gains (20%). Sequencing of matched tumor and blood DNA samples identified novel somatic mutations in MYD88 (L265P hot spot mutation) and TBL1XR1 in 38% and 14% of the cases, respectively. The correlation of genetic abnormalities with clinical outcomes using multivariate analysis showed that 6q22 loss (p=0.006 and p=0.01), and CDKN2A homozygous deletion (p=0.02 and p=0.01) were significantly associated with shorter progression free survival and overall survival. Conclusions: Our study identified novel genetic alterations in PCNSL, such as MYD88 and TBL1XR1 somatic mutations, which would both contribute to the constitutive activation of the NFkB signaling pathway and represent potential promising targets for future therapeutic strategies.

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