Late relapse of primary central nervous system lymphoma

Yamanaka, Ryuya; Morii, Ken; Shinbo, Yoshikatsu; Sano, Masakazu; Homma, Jumpei; Tsuchiya, Naoto; Yajima, Naoki; Tsukamoto, Yoshihiro; Ogura, Ryosuke; Natsumeda, Manabu; Aoki, Hiroshi; Akiyama, Katsuhiko; Saitoh, Tomoko; Tamura, Tetsuro; Hondoh, Hiroaki; Kawaguchi, Atsushı; Takahashi, Hitoshi; Fujii, Yukihiko

doi:10.1080/10428194.2016.1201570

Cited by 12 publications

(14 citation statements)

References 14 publications

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“…( 18 ) also supported the same clonal relationship between primary and relapsed tumors. Notably, extra-CNS relapses of PCNSL, especially “early systemic relapses”, may be derived from occult systemic lymphoma at the time of initial diagnosis, rather than being a true relapse, and studies supported the presence of occult systemic lesions at the time of initial diagnosis in up to 12.5% of patients with PCNSL ( 4 , 19 , 20 ). In addition, extra-CNS subclinical lesions of PCNSL that could not be eradicated by first-line treatment are easy to confuse with relapses ( 21 ), especially “very early relapses”.…”

Section: Mechanismmentioning

confidence: 99%

“…PCNSL is an invasive NHL, and although some patients can achieve complete response (CR) after initial first-line treatment, 36-66.6% of patients still relapse ( 4 , 17 , 34 , 35 ). Jahnke et al.…”

Section: Relapse Rate and Relapse-related Factorsmentioning

confidence: 99%

“…PCNSL accounts for 2-3% of NHL and 4% of CNS malignancies ( 1 – 3 ). Despite significant improvements in the management of PCNSL, up to 60% of patients eventually relapse, and there is currently no accepted standard salvage regimen, additionally, the rate of remission is low, while prognosis after relapse remains poor ( 4 – 6 ). This article summarizes the mechanisms, clinical features, follow-up, treatment and prognosis of relapsed PCNSL, aiming to provide evidence for further understanding and designing better treatment strategies.…”

Section: Introductionmentioning

confidence: 99%

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Relapsed Primary Central Nervous System Lymphoma: Current Advances

2021

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Primary central nervous system lymphoma is an invasive malignant lymphoma confined to the central nervous system. Although patients undergoing first-line treatment can achieve complete response, most of them still relapse within two years. Relapsed lymphoma is derived from occult lymphoma cells, and B cell receptor pathway activation and immune escape are the key mechanisms for the pathogenesis of PCNSL. Most relapses are in the central nervous system, a small number of relapses are isolated systemic relapses, and clinical symptoms occur early and vary. Current treatments for relapse include high-dose methotrexate rechallenge and other regimens of chemotherapy, whole-brain radiation therapy, hematopoietic stem-cell transplantation, targeted therapy and immunotherapy, which have become promising treatments. The overall prognosis of relapsed PCNSL is very poor, although it is affected by many factors. This article summarizes the mechanisms, related factors, clinical features, follow-up, treatment and prognosis of relapsed primary central nervous system lymphoma.

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Section: Mechanismmentioning

confidence: 99%

Section: Relapse Rate and Relapse-related Factorsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Relapsed Primary Central Nervous System Lymphoma: Current Advances

2021

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“…Though demonstrating a favorable 2-year progression-free survival rate of 53%, after longer follow-up period, only 1 patient out of 54 remained in remission at 60 months [31]. This was discovered also in a Japanese study with a follow-up for 14 years [33]. Because prospective trials are usually reported with limited follow-up time, a considerable number of patients will relapse after follow-up.…”

Section: Prognosismentioning

confidence: 86%

Primary Central Nervous System Lymphoma Increasing Incidence and Poor Survival: A Finnish Population-based Analysis

Puhakka¹,

Kuittinen²,

Jäkälä³

et al. 2021

Preprint

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PurposeWe report here the first population-based incidence rates and prognosis of primary central nervous system lymphoma (PCNSL) in Finland. MethodsFinnish Cancer Registry data by histological diagnosis and tumor location (2007-2017) for cases with diffuse large B-cell lymphoma.ResultsDuring 2007–2017, 416 new cases of PCNSL were reported (202 males, 214 females). The average age-adjusted incidence in males vs. females for 2007-2009, 2010-2012, 2013-2015 and 2016-2017 was 0.57/100,000 vs. 0.48/100,000 person-years, 0.66/100,000 vs. 0.55/100,000, 0.53/100,000 vs. 0.63/100,000, and 0.76/100,000 vs. 0.66/100,000 respectively. The incidence was highest, 3.27/100,000, among people aged 75–79 years. From 2007–2012 to 2013–2017, the 2-year age-adjusted survival rate increased from 36% to 41%, and the 4-year survival rates from 31% to 37%. Among patients aged 70+ years, the corresponding 2-year survival rates were 25% and 29%, and the 4-year survival rates 25% and 22%.ConclusionAge-adjusted incidence of PCNSL increased from 0.57/100,000 (2007–2012) to 0.63/100,000 (2013–2017). The annual increase was 3.8% being among the highest reported in the world. The prognosis was poor, although a minor improvement in 4-year survival was found.

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“…Löw et al (22), previously reported that administering a high methotrexate dose could lead to a high treatment response rate in PCNSL patients. However, the relapse rate can reach up to 50% with the 5-year survival rate ranging from 22-40% (23,24). In PCNSL, MYD88 L265P is a hot-spot mutation, which alters interleukin-1 and toll-like receptor signaling and leads to the hyperactivation of the NF-κB (25) and JAK/STAT signaling pathways (26)(27)(28).…”

Section: Myd88 L265p Mutation Using Ddpcr Positive Myd88 ------------mentioning

confidence: 99%

Effectiveness of digital PCR for MYD88L265P detection in vitreous fluid for primary central nervous system lymphoma diagnosis

Chen

Ding

et al. 2020

Exp Ther Med

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Primary central nervous system lymphoma (PCNSL) is a rare type of primary extranodal lymphoma (PEL). MYD88 L265P mutation has been observed in up to 75% of PCNSL cases, however, the validity and sensitivity of digital PCR in detecting this mutation remains to be elucidated. A total of 44 PCNSL patients, 15 diffuse large B-cell lymphoma not otherwise specified (DLBCL-NOS) patients and 13 other PEL patients were enrolled in the present study. The abilities of reverse transcription quantitative PCR (RT-qPCR) and droplet digital PCR (ddPCR) to detect the MYD88 L265P mutation in cerebral spinal fluid (CSF) samples were compared. The results suggested that ddPCR showed superior mutation detection sensitivity when compared with RT-qPCR (58 vs. 15%; P<0.05). The MYD88 L265P mutation was significantly associated with increased MYD88 protein overexpression in PCNSL brain tissue samples (P<0.05). Analysis of MYD88 L265P mutation status in CSF and vitreous fluid samples using ddPCR may be a promising technique for minimally invasive confirmation of PCNSL diagnosis.

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Late relapse of primary central nervous system lymphoma

Cited by 12 publications

References 14 publications

Relapsed Primary Central Nervous System Lymphoma: Current Advances

Relapsed Primary Central Nervous System Lymphoma: Current Advances

Primary Central Nervous System Lymphoma Increasing Incidence and Poor Survival: A Finnish Population-based Analysis

Effectiveness of digital PCR for MYD88L265P detection in vitreous fluid for primary central nervous system lymphoma diagnosis

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