Primary Peripheral T-Cell Lymphoma of the Central Nervous System

Stark, Alejandro; Tiemann, Markus; Dörner, Lutz; Melnikowa, E.; Mehdorn, H. Maximilian; Blömer, Ulrike

doi:10.1055/s-2004-822786

Cited by 7 publications

(3 citation statements)

References 6 publications

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“…The prognosis for T-cell lymphoma is controversial. There are several reports about primary TPCNSL with poor prognosis [18, 19, 29], and several with good response rates and overall survival time [10, 27]. Of our sample of nine patients, two died due to progressive disease after HD-MTX-based chemotherapy.…”

Section: Discussionmentioning

confidence: 97%

Primary CNS lymphoma other than DLBCL: a descriptive analysis of clinical features and treatment outcomes

et al. 2011

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Diffuse large B-cell lymphoma (DLBCL) constitutes most primary central nervous system (CNS) lymphoma (PCNSL), whereas T-cell, low-grade and Burkitt’s lymphomas (BL) are rarely encountered. Due to the paucity of cases, little is known about the clinical features and treatment outcomes of PCNSL other than DLBCL. The objective of this study was to describe the clinical characteristics and outcomes for patients with PCNSL other than DLBCL. Fifteen patients, newly diagnosed with PCNSLs other than DLBCL between 2000 and 2010, were included. The male to female ratio was 0.67:1 with a median age of diagnosis of 31 years (range 18–59). Pathologic distributions were as follows: peripheral T-cell lymphoma (PTCL; n = 7), marginal zone B-cell lymphoma (MZBCL; n = 1), lymphoplasmacytic lymphoma (LPL; n = 2), Burkitt’s lymphoma (n = 1), other unspecified (T-cell lineage, n = 2; B-cell lineage, n = 2). Thirteen patients (87%) showed Eastern Cooperative Oncology Group performance score (ECOG PS) 1–2. The remaining two were one PTCL patient and one Burkitt’s lymphoma patient. Of the nine patients with T-cell lymphoma, five (56%) had multifocal lesions, and one (20%) with LPL of the five patients with B-cell lymphoma showed a single lesion. Leptomeningeal lymphomatosis was identified in two patients (one with Burkitt’s lymphoma and one with unspecified B-cell lymphoma). Two patients (22%) with T-cell lymphoma died 7.7 and 23.3 months later, respectively, due to disease progression, despite HD-MTX-based therapy. Six patients with T-cell lymphoma (6/9, 66.7%) and four patients with low-grade B-cell lymphoma (4/5, 80%) achieved complete response and have survived without relapse (Table 3). One patient with Burkitt’s lymphoma showed poor clinical features with ECOG PS 3, deep structure, multifocal, and leptomeningeal lymphomatosis, and died 7.6 months after the initiation of treatment. In comparison with previously reported DLBCLs (median OS 6.4 years, 95% CI 3.7–9.1 years), T-cell lymphoma showed equivocal or favorable clinical outcomes and low-grade B-cell lymphomas, such as MZBCL and LPL, had a good prognosis. However, primary CNS Burkitt’s lymphoma presented poor clinical outcomes and showed a comparatively aggressive clinical course. In conclusion, primary CNS lymphoma other than DLBCL occurred more in younger patients and showed a generally good prognosis, except for Burkitt’s lymphoma. Further research on treatment strategies for Burkitt’s lymphoma is needed.

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Section: Discussionmentioning

confidence: 97%

Primary CNS lymphoma other than DLBCL: a descriptive analysis of clinical features and treatment outcomes

et al. 2011

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“…6 Although T-cell lymphomas have been reported as primary CNS tumors, they are infrequent in PCNSL, accounting for less than 5% of all cases. 15,26,31,38,40,44 Unlike B-cell lymphomas, most peripheral (nonlymphoblastic) T-cell lymphomas lack a distinctive morphological appearance, and consist of small, intermediate, or large cells, in variable combinations. Given this heterogeneous composition, which may suggest reactive T-cell proliferations, definitive diagnosis depends on clinical history, unequivocal T-cell phenotype and clear cytological demonstration of malignancy, overt infiltration with tissue destruction, or molecular evidence of a clonal T-cell population.…”

Section: Gross Pathological Characteristicsmentioning

confidence: 99%

Molecular pathogenesis of primary central nervous system lymphoma

Kadoch¹,

Treseler²,

Rubenstein³

2006

FOC

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✓ Primary central nervous system lymphoma (PCNSL) is an aggressive form of non-Hodgkin lymphoma (NHL) typically associated with a worse prognosis than other localized extranodal lymphomas with similar histological characteristics. The defining feature of PCNSL is its confinement to the central nervous system (CNS), with proclivity for growth within the leptomeningeal as well as intraocular compartments. Primary CNS lymphoma rarely disseminates outside the CNS and accounts for less than 5% of all primary brain neoplasms. At least 95% of PCNSLs are of large B-cell histology, the most common subtype of NHL. Consistent with the trend seen in systemic NHLs, the incidence of PCNSL has markedly increased over the past three decades, both in immunocompromised and immunocompetent patients. Because PCNSL is relatively rare, the identification of molecular prognostic biomarkers and the definition of a standard therapeutic strategy have been challenging. The authors discuss the current knowledge of the molecular pathogenesis of CNS lymphomas and review the recent advances in gene expression profile analysis and identification of novel prognostic biomarkers.

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“…Allerdings ist die histopathologische und immunhistochemische Untersuchung gerade bei niedriggradiger Morphologie wie im vorliegenden Fall nicht immer ausreichend, um ein PTZL ZNS sicher von entzündlichen demyelinisierenden Läsionen, Vaskulitiden, viralen oder bakteriellen Schädigungen sowie von diffus großzelligen B-Zell-Lymphomen des ZNS mit zahlreichen reaktiven TLymphozyten zu differenzieren [3,7,10]. In einigen Fällen wurde primär aufgrund der erhöhten Lymphozytenzahl in der Liquorpunktionsflüssigkeit die Verdachtsdiagnose einer viralen Meningoenzephalitis gestellt und erst nach zeitintensivem Ausschluss viraler, bakterieller und fungaler Infektionen (einschließlich Herpessimplex-Virus, Zytomegalievirus, Varizella-Zoster-Virus, EBV, HIV, Borrelien und Toxoplasma gondii) der Weg zur Diagnose eines PTZL ZNS verfolgt [9].…”

Section: Differenzialdiagnose Mittels Immunhistochemieunclassified