Rare cases of primary central nervous system anaplastic variant of diffuse large B-cell lymphoma

Xu, Tianqi; Jia, Qingge; Wang, Yingmei; Liu, Yixiong; Han, Donghui; Li, Peifeng; Ma, Jing; Fan, Linni; Qu, Yan; Guo, Shuangping; Li, Mingyang; Wang, Zhe

doi:10.1186/s13000-019-0826-0

Cited by 6 publications

(4 citation statements)

References 35 publications

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“…Furthermore, ALK+ LBCL is a rare subgroup of DLBCL characterized by diffuse infiltration of medium to large neoplastic cells with round centric or eccentric nuclei, and is usually negative for CD30 and CD20 with a strong positivity to ALK, in contrast to the findings of our case [ 16 , 18 , 20 ]. Also, the negative expression of CD15 in addition to the absence of inflammatory background and band-like fibrosis was essential to exclude CHL [ 15 , 16 ].…”

Section: Discussioncontrasting

confidence: 99%

“…ALCL is defined as a CD30-positive neoplasm of T- or null-cell lineage, characterized by the proliferation of large pleomorphic cells with kidney-shaped nuclei. The negative expression of T-cell markers (CD5, CD3) in our case, in addition to the strong expression of the B-cell marker (CD20), was crucial to exclude ALCL [ 16 , 18 , 19 ].…”

Section: Discussionmentioning

confidence: 95%

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Primary splenic anaplastic variant of diffuse large B-cell lymphoma: a case report

et al. 2021

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Background Primary splenic lymphoma represents a rare entity that constitutes less than 1% of non-Hodgkin lymphomas, and less than 2% of all lymphomas. Diffuse large B-cell lymphoma (DLBCL) is the most common histological subtype of primary splenic lymphomas. DLBCL encompasses a heterogeneous entity with distinct morphological variants. The anaplastic variant of DLBCL was first defined in the 2017 World Health Organization classification as a rare histological subtype that constitutes less than 3.4% of DLBCL cases. Case presentation A 65-year-old Syrian man presented to our hospital with constant dull localized left upper quadrant abdominal pain for about 20 days accompanied by general weakness, loss of appetite, and rapid weight loss. Clinical examination revealed isolated splenomegaly and left upper abdominal tenderness. Following physical, laboratory, and radiologic examinations, the patient underwent splenectomy. Interestingly, pathological and immunohistochemical examinations of the resected spleen confirmed the diagnosis of a primary anaplastic variant of DLBCL. Conclusions Herein, we aimed to present an unusual combination of a rare splenic neoplasm and a unique lymphoma subtype. Furthermore, we aimed to highlight the difficulties in differential diagnosis and the importance of histological and immunohistochemical examinations with clinical correlation.

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Section: Discussioncontrasting

confidence: 99%

Section: Discussionmentioning

confidence: 95%

Primary splenic anaplastic variant of diffuse large B-cell lymphoma: a case report

et al. 2021

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“…Patients with A-DLBCL follow an aggressive disease course and have poor prognosis ( 3 ). In our study on 3 cases of primary CNS A-DLBCL, patients also had MYC/BCL2 co-expression, and concurrent MYC and BCL2 and/or BCL6 genetic abnormalities, and constitutive NF-κB pathway activation ( 4 ). Our results suggest that A-DLBCL displays many genetic alterations and biological features that differ greatly from ordinary DLBCL.…”

Section: Introductionmentioning

confidence: 84%

Tumor Immune Microenvironment Components and Checkpoint Molecules in Anaplastic Variant of Diffuse Large B-Cell Lymphoma

Chai

Wang

et al. 2021

Front. Oncol.

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BackgroundAnaplastic diffuse large B-cell lymphoma(A-DLBCL) is a rare morphological subtype characterized by the presence of polygonal, bizarre-shaped tumor cells. Our previous research found that A-DLBCL displays many genetic alterations and biological features that differ greatly from those of ordinary DLBCL. However, the status of tumor immune microenvironment components and checkpoint molecules in A-DLBCL remains unclear.MethodsThirty A-DLBCL patients were enrolled to study tumor immune microenvironment components and checkpoint molecules and their associations with clinicopathological features and prognosis.ResultsPatients with A-DLBCL presented higher expression of PD-L1 (40% vs 10%, P=0.004) than patients with ordinary DLBCL. FISH analysis showed that extra copies of PD-L1 were more frequent in A-DLBCL cases than in ordinary DLBCL cases (23.3% vs 4.0%, P=0.001). The numbers of PD-1+ TILs (tumor infiltrating lymphocytes) and CD8+T cells were significantly lower in A-DLBCL versus ordinary DLBCL. In contrast, the numbers of GATA3+ Th2 cells, FOXP3+ Tregs and CD33+ myeloid-derived suppressor cells (MDSCs) were significantly higher in A-DLBCL than in ordinary DLBCL. The associations between clinicopathological features and tumor immune microenvironment cell frequency were analyzed in A-DLBCL patients. Briefly, the number of PD-1+ TILs was lower and the number of CD33+ MDSCs was higher in patients with mutated TP53 compared to those with wild-type TP53. The number of FOXP3+ Tregs was much lower in patients with a noncomplete response (CR) to chemotherapy than in those with a complete response. The number of CD8+ T cells showed a decreasing trend in patients with high International Prognostic Index (IPI) scores and in those with concurrent MYC and BCL2 and/or BCL6 abnormalities. Univariate survival analysis showed that patients with PD-L1+, mPD-L1+(PD-L1+ nonmalignant stromal cells) or mPD-L1+ status had a significantly poorer overall survival (OS) than those with PD-L1- status. An increase in the number of CD3+ T cells, FOXP3+ Treg cells and T-bet+ Th1 cells was significantly associated with prolonged OS in patients with A-DLBCL.ConclusionOur study suggests that A-DLBCL displays a distinct pattern of tumor immune microenvironment components and checkpoint molecules that distinguish it from ordinary DLBCL. The analysis of tumor immune microenvironment components and checkpoint molecules could help in predicting the prognosis of A-DLBCL patients and determining therapeutic strategies targeting the tumor immune microenvironment.

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“…[1] It involves areas such as the brain, spinal cord, brain nerves, eyes, and meninges, representing merely 1% of all non-Hodgkin lymphoma cases and accounting for around 3% of all brain tumors. [2,3] In recent decades, its incidence has shown an upward trend, primarily affecting older individuals with compromised immunity, while occurrences among young individuals with intact immune function are rare. PCNSL typically infiltrates the cerebral hemispheres, thalamus/basal ganglia, corpus callosum, and periventricular regions, with rare instances involving the cerebellum.…”

Section: Introduction Primarymentioning

confidence: 99%

Experience of multi-disciplinary treatment of multiple cerebellar diffuse large B-cell lymphoma: A case report

Guo,

Li,

et al. 2024

Medicine

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Rationale: Primary central nervous system lymphoma (PCNSL) is a rare, highly malignant form of non-Hodgkin lymphoma categorized under the diffuse large B-cell type. It accounts for merely 1% of all non-Hodgkin lymphoma cases and comprises approximately 3% of all brain tumors. The involvement of the cerebellum is observed in only 9% of these cases. Recently, we came across an unusual instance: a young man presenting with multiple lesions located specifically within the cerebellum. Patient concerns: A 26-year-old male was admitted to the hospital due to severe headaches. He has a medical history of sporadic headaches, accompanied by dizziness, nausea, and vomiting persisting for a month. Over the last 10 days, his headaches have intensified, coupled with decreased vision and protrusion of the eyeballs. Magnetic resonance imaging (MRI) revealed abnormal signals in both cerebellar hemispheres. Diagnoses, interventions, and outcomes: Diagnostic procedures included cerebellar biopsy, posterior fossa decompression, and lateral ventricle drainage. Histopathological examination identified diffuse large B-cell lymphoma (DLBCL) with high proliferative activity. To minimize neurotoxicity, chemotherapy involved intrathecal methotrexate (MTX) injections combined with the CHOP program. The patient has shown good tolerance to the treatment so far. Lessons: While the definitive optimal treatment approach remains elusive, current chemotherapy centered on high-dose MTX stands as the standard induction therapy. Integrating surgery with radiotherapy and chemotherapy significantly extends patient survival.

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Rare cases of primary central nervous system anaplastic variant of diffuse large B-cell lymphoma

Cited by 6 publications

References 35 publications

Primary splenic anaplastic variant of diffuse large B-cell lymphoma: a case report

Primary splenic anaplastic variant of diffuse large B-cell lymphoma: a case report

Tumor Immune Microenvironment Components and Checkpoint Molecules in Anaplastic Variant of Diffuse Large B-Cell Lymphoma

Experience of multi-disciplinary treatment of multiple cerebellar diffuse large B-cell lymphoma: A case report

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