Diagnosis, prevention and treatment of central nervous system involvement in peripheral t-cell lymphomas

Zing, Natalia Pin Chuen; Fischer, Thaís; Federico, Massimo; Chiattone, Carlos S.; Ferreri, Andrés J.M.

doi:10.1016/j.critrevonc.2021.103496

Cited by 4 publications

(8 citation statements)

References 75 publications

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“…[139][140][141][142][143][144] T-cell lymphomas Primary CNS T-cell lymphomas (PCNS-TCL) account for between 2% and 8.5% of all primary lymphomas within the CNS, [145][146][147] with a reported higher incidence in Eastern countries. 148 Estimates of average age at diagnosis range from 55.8 to 60 years. 149,150 Interestingly, a recent systematic review described a much lower median age at diagnosis for those with primary CNS anaplastic large cell lymphoma (PCNS-ALCL), at 21 years (range 1-82 years).…”

Section: Burkitt Lymphomamentioning

confidence: 99%

Rare central nervous system lymphomas

2022

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Central nervous system (CNS) lymphomas are rare malignancies characterised by lymphoid infiltration into the brain, spinal cord, cranial nerves, meninges and/or eyes in the presence or absence of previous or concurrent systemic disease. Most CNS lymphomas are of the diffuse large B‐cell lymphoma (DLBCL) subtype for which treatment strategies, particularly the use of high‐dose methotrexate‐based protocols and consolidation with autologous stem cell transplantation, are well established. Other histopathological subtypes of CNS lymphoma are comparatively less common with published data on these rare lymphomas dominated by smaller case series and retrospective reports. Consequently, there exists little clinical consensus on the optimal methods to diagnose and manage these clinically and biologically heterogeneous CNS lymphomas. In this review article, we focus on rarer CNS lymphomas, summarising the available clinical data on incidence, context, diagnostic features, reported management strategies, and clinical outcomes.

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Section: Burkitt Lymphomamentioning

confidence: 99%

Rare central nervous system lymphomas

2022

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“…Central nervous system (CNS) involvement in peripheral T-cell lymphoma (PTCL) is a rare event when comapred with adult T-cell leukemias and HIV-associated T-cell lymphomas. Primary CNS lymphoma exclusively involves CNS at presentation and secondary CNS lymphomas are diagnosed in patients with systemic lymphomas where CNS involvement can occur at presentation or relapse as an isolated site of recurrence or associated with systemic disease ( 1 ). Primary CNS T-cell lymphomas (PCNSTCLs) occur in 7.4%–8.5% in Asia and in 3.5%–5% in Western countries ( 2 – 5 ).…”

Section: Introductionmentioning

confidence: 99%

“…Based on retrospective studies, several risk factors associated with a higher risk of CNS dissemination have been documented: involvement of >1 extranodal site; involvement of gastrointestinal tract, skin, nose, or paranasal sinuses; advanced disease (Ann Arbor clinical stage III or IV); International Prognostic Index (IPI) score ≥3; and elevated serum lactate dehydrogenase (LDH) ( 1 ). A new CNS prognostic index of natural killer lymphoma (CNS-PINK) was developed for patients with ENKTCL treated with non-anthracycline-based chemotherapy ( 19 ).…”

Section: Introductionmentioning

confidence: 99%

“…When the CNS-IPI commonly used in aggressive B-cell lymphomas is used in PTCL, the diagnostic sensitivity is only 6% ( 6 ). Therefore, it should not be used routinely in patients with T-cell lymphomas ( 1 ).…”

Section: Introductionmentioning

confidence: 99%

“…Patients with T-cell lymphomas at high risk of CNS dissemination who receive a high dose of MTX as part of the first-line treatment do not need additional prophylaxis ( 1 ). There is a lack of data supporting drugs other than MTX as CNS prophylaxis in T-cell lymphomas.…”

Section: Introductionmentioning

confidence: 99%

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Peripheral T-Cell Lymphomas Involving the Central Nervous System: A Report From the Czech Lymphoma Study Group Registry

et al. 2022

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IntroductionWe analyzed the incidence, risk factors of central nervous system (CNS) relapse, and outcome of CNS involvement in patients with peripheral T-cell lymphomas (PTCL) from the Czech Lymphoma Study Group Registry NiHiL (Clinical Trial gov. NCT03199066).Materials and MethodsOut of 1,040 patients with PTCL, we identified 29 patients (2.79%) with CNS involvement: 2 patients with primary CNS T cell lymphoma, 11 patients with CNS and systemic disease at diagnosis, and 16 patients (1.54%) at CNS relapse. The most common histology with CNS disease was PTCL, not otherwise specified. Progression-free survival (PFS) was defined as the time interval from diagnosis to progression or death. PFS-2 was defined as the interval from the date of a new relapse until the next relapse.ResultsPatients with testicular involvement received intrathecal prophylaxis with methotrexate. High-dose methotrexate-based treatment was administered in 44.8% of patients with CNS disease. Median follow-up was 71.3 months. The difference between the median PFS of 1,027 patients without initial CNS disease (32.6 months) and 11 patients with initial CNS and systemic disease (4.8 months) was significant (p = 0.04). The difference between the median PFS2 in CNS relapses (10.1 months) and 493 relapses outside of CNS (9.1 months) was not significant (p = 0.6). Risk factors for CNS relapses included the following: involvement of more than one extranodal site (p = 0.008), soft tissue involvement (p = 0.003), testicular involvement (p = 0.046), and the presence of B symptoms (p = 0.035). The difference between the median OS of 1,027 patients without initial CNS disease (46.0 months) and 11 patients with initial CNS and systemic disease (18.2 months) was significant (p = 0.02). The median OS2 in CNS relapses was 11.8 months and that in relapses outside of CNS was 21.3 months. CNS involvement was not associated with a significantly worse OS compared to relapsed/refractory patients without CNS involvement (p = 0.1).ConclusionsThe incidence of CNS disease at the time of diagnosis and at relapse in PTCL is low and usually associated with other systemic involvement. The prognosis of PTCL with initial CNS involvement is significantly worse when compared to patients without CNS disease at diagnosis. The outcome of CNS relapse is comparable with relapsed PTCL outside of CNS. The optimal treatment is not defined yet.

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Rare CNS Metastasis from Peripheral T Cell Lymphoma

Pirich,

Calabek-Wohinz,

Freydl

et al. 2023

psychopraxis. neuropraxis

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ZusammenfassungDie Metastasierung in das zentrale Nervensystem (ZNS) bei T‑Zell-Lymphomen ist ein seltenes klinisches Szenario. Zu den häufigsten Subtypen mit ZNS-Beteiligung gehören periphere T‑Zell-Lymphome (PTCL) und anaplastische großzellige Lymphome (ALCL). Neurologische Symptome bei einer sekundären ZNS-Beteiligung entwickeln sich in der Regel subakut bis akut. Die diagnostischen Maßnahmen umfassen die klinisch neurologische Untersuchung, eine Gadolinium-verstärkte MRT der gesamten Neuroachse, eine Liquoranalyse und gegebenenfalls eine Hirnbiopsie. Die ZNS-Metastasierung von T‑Zell-Lymphomen stellt, aufgrund ihrer Seltenheit, eine diagnostische Herausforderung dar und muss von anderen Erkrankungen, wie infektiologischen, autoimmunen oder anderen neoplastischen Ursachen, abgegrenzt werden. Zu den Behandlungsoptionen gehören die systemische Chemotherapie, die intrathekale Chemotherapie und die Strahlentherapie. Die Prognose für eine sekundäre ZNS-Beteiligung bei T‑Zell-Lymphomen ist, trotz der zahlreichen Behandlungsoptionen, in der Regel ungünstig. In diesem Fallbericht wird über eine durch Biopsie nachgewiesene ZNS-Metastase eines peripheren T‑Zell-Lymphoms berichtet. Ziel der Literaturübersicht inklusive des Fallberichts ist es, das Verständnis für die klinischen Merkmale, die diagnostischen Modalitäten, die Behandlungsmöglichkeiten und die Prognose der ZNS-Metastasierung von peripheren T‑Zell-Lymphomen zu verbessern.

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Diagnosis, prevention and treatment of central nervous system involvement in peripheral t-cell lymphomas

Cited by 4 publications

References 75 publications

Rare central nervous system lymphomas

Rare central nervous system lymphomas

Peripheral T-Cell Lymphomas Involving the Central Nervous System: A Report From the Czech Lymphoma Study Group Registry

Rare CNS Metastasis from Peripheral T Cell Lymphoma

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