primary diffuse large B-cell lymphoma of the central nervous system

Primary Diffuse Large B-Cell Lymphoma of the Central Nervous System

Primary diffuse large B-cell lymphoma (DLBCL) of the central nervous system (CNS) is a rare and aggressive type of cancer that affects the brain and spinal cord. It is characterized by the growth of abnormal B cells in the CNS, which can lead to symptoms such as headaches, seizures, and cognitive decline.

Key Features:

• Rare tumor: Primary DLBCL of the CNS is estimated to account for only 1-3% of all lymphomas [1].
• Aggressive behavior: This type of cancer grows quickly and can spread to other parts of the brain or spinal cord.
• Symptoms: Patients may experience headaches, seizures, cognitive decline, and other neurological symptoms [4][5].

Types:

• Diffuse large B-cell lymphoma (DLBCL): The most common type of primary CNS lymphoma, accounting for about 90% of cases [9].
• Poorly characterized low-grade lymphomas: The remaining 10% are less understood and may have different characteristics.

Treatment:

• Aggressive treatment: Primary DLBCL of the CNS requires prompt and aggressive treatment, which typically involves chemotherapy, radiation therapy, or a combination of both [6][7].

References:

[1] D CHEN · 2016 - Primary diffuse large B-cell lymphoma (Bcl) of the central nervous system (CNS) is a rare tumor...

[2] M Roschewski · 2024 - Diffuse large B-cell lymphoma (DLBCL) is an aggressive B-cell lymphoma curable even in advanced stages.

[3] M Roschewski · 2020 - Diffuse large B-cell lymphoma (DLBCL) is a spectrum of aggressive B-cell lymphomas with remarkable clinical and molecular heterogeneity.

[4] Jul 6, 2021 - Primary central nervous system lymphoma (PCNSL) is a densely cellular, aggressive non-Hodgkin B-cell lymphoma.

[5] Jan 17, 2024 - Lymphoma in the CNS is usually a fast-growing (high-grade) non-Hodgkin lymphoma.

[6] Diffuse large B cell lymphoma (DLBCL) is a type NHL. The most common symptom is one or more painless swellings.

[7] Primary lymphoma of the central nervous system (PCNSL), defined as diffuse large B-cell lymphoma (DLBCL) confined to the central nervous system (CNS), has...

[8] The most common type of primary CNS lymphoma is diffuse large B-cell lymphoma. Diffuse large B-cell lymphoma develops when the body makes abnormal B cells.

[9] Most forms of CNS lymphoma (about 90 percent) are diffuse large B-cell lymphomas; the remaining ten percent are poorly characterized low-grade lymphomas...

Common Signs and Symptoms

Primary diffuse large B-cell lymphoma (DLBCL) of the central nervous system (CNS) can exhibit a range of symptoms, which may vary in severity and duration. Some common signs and symptoms include:

• Weakness or numbness in arms or legs: This is often one of the first noticeable symptoms, as the tumor grows and affects the surrounding nerves [7].
• Changes in behavior, personality, and speech: As the lymphoma progresses, it can cause cognitive and behavioral changes, including difficulty with memory, confusion, and altered mental status [8].
• Headaches: Headaches are a common symptom of primary CNS lymphoma, often accompanied by nausea and vomiting [3].
• Seizures: Seizures can occur due to the tumor's impact on brain function [4].
• Balance and coordination problems: Patients may experience difficulties with balance and coordination, which can increase the risk of falls [7].

Other Possible Symptoms

In addition to these common symptoms, primary CNS lymphoma may also present with:

• Reddened patches or lumps in the skin: These can be a sign of lymphoma-related skin involvement [6].
• Fever, night sweats, and unexplained weight loss (B symptoms): While not specific to CNS lymphoma, these symptoms can occur in conjunction with other signs and symptoms [6].

Important Considerations

It's essential to note that the symptoms of primary CNS lymphoma can be similar to those of other brain tumors. A definitive diagnosis requires a combination of clinical evaluation, imaging studies (e.g., MRI or CT scans), and biopsy results.

References:

[1] Jan 17, 2024 — Lymphoma in the CNS is usually a fast-growing (high-grade) non-Hodgkin lymphoma. [3] May 25, 2023 — Signs and symptoms of primary CNS lymphoma may include nausea and vomiting or seizures. [7] Symptoms of CNS lymphoma · Problems with balance and coordination · Weakness in arms or legs · Changes in behavior, personality and the ability to speak · Headaches [8] Symptoms of primary CNS lymphoma · Behaviour or personality changes. · Finding it difficult to remember things. · Numbness, tingling or weakness. · Speech problems

Diagnostic Tests for Primary Diffuse Large B-Cell Lymphoma (DLBCL) of the Central Nervous System

Primary DLBCL of the CNS is a rare and aggressive form of non-Hodgkin lymphoma that affects the brain or spinal cord. Accurate diagnosis is crucial for effective treatment. Here are some diagnostic tests used to diagnose primary DLBCL of the CNS:

• Brain Biopsy: This is considered the gold standard for diagnosing CNS lymphoma [1, 7, 9]. A sample of tissue is removed and examined under a microscope by an expert lymphoma pathologist.
• Positron Emission Tomography-Computed Tomography (PET-CT) scans: These scans can help identify the extent of disease and monitor response to treatment [2].
• Cerebrospinal Fluid (CSF) cytology: This test involves analyzing a sample of CSF for cancer cells. While it can provide definitive diagnostic information, it is not always positive in patients with primary CNS lymphoma [3, 4].
• Imaging studies: MRI and CT scans are used to evaluate the extent of disease and rule out other conditions that may be causing symptoms.
• Lumbar puncture: This procedure involves removing a sample of CSF from the spinal canal. However, it is considered low-yield for diagnosing primary CNS lymphoma [5].

References:

[1] A van Westrhenen et al. (2018) - Brain biopsy is the gold standard for the diagnosis of CNS lymphoma.

[2] D CHEN et al. (2016) - The present study elucidates the role of PET-CT scans for the diagnosis and management of primary diffuse large B-cell lymphoma.

[3] BJ Scott et al. (2013) - Cerebrospinal fluid cytology can provide definitive diagnostic information in CNS lymphoma.

[4] FJ Peñalver et al. (2017) - Diagnostic screening for CNS disease in DLBCL.

[5] Jul 6, 2021 - Lumbar puncture is low-yield as the majority of patients with primary central nervous system lymphoma will not have leptomeningeal or CSF involvement.

[6] Jan 17, 2024 - CNS lymphoma is diagnosed with a procedure called a biopsy.

[7] AJM Ferreri et al. (no year) - The gold standard diagnostic method in PCNSL relies on the histopathological examination of specimens obtained by stereotactic biopsy.

[8] Oct 10, 2024 - Primary central nervous system lymphoma: Clinical features, diagnosis, and extent of disease evaluation.

[9] F Bertoni et al. (2022) - Brain biopsy remains the gold standard for the diagnostic of CNS-DLBCL.

Treatment Options for Primary CNS Lymphoma

Primary diffuse large B-cell lymphoma (DLBCL) of the central nervous system (CNS) is a rare and aggressive type of cancer. The treatment options for this condition are similar to those for primary CNS lymphoma, but with some differences.

• Chemotherapy: Chemotherapy is often used as the first-line treatment for primary CNS DLBCL. The most common chemotherapy regimen used is high-dose methotrexate-based combinations [1]. This type of chemotherapy has been shown to be effective in treating this condition.
• Radiation Therapy: Radiation therapy may also be used in conjunction with chemotherapy to treat primary CNS DLBCL. However, the use of radiation therapy alone is not typically recommended for this condition [3].
• Targeted Therapy: Targeted therapies such as rituximab have been shown to be effective in treating primary CNS lymphoma and may also be used in the treatment of primary CNS DLBCL [4].
• High-Dose Chemotherapy with Stem Cell Transplant: High-dose chemotherapy with stem cell transplant is a more aggressive treatment option that may be considered for patients who do not respond to standard chemotherapy regimens [8].

Treatment Regimens

The most common treatment regimen used for primary CNS DLBCL is the CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) regimen. However, this regimen has been shown to have negligible activity in treating extra-CNS DLBCL [9].

Other Treatment Options

Other treatment options such as non-myeloablative high-dose chemotherapy and oral drug maintenance may also be considered for patients with primary CNS DLBCL [8]. Additionally, targeted therapies such as ibrutinib or idelalisib may also be used in the treatment of this condition.

References:

[1] M Roschewski et al. (2020) - Standard approaches for PCNSL use high-dose methotrexate-based combinations as induction therapy and younger patients often receive dose-...

[3] May 25, 2023 — Radiation therapy · Chemotherapy · Steroid therapy · Targeted therapy · High-dose chemotherapy with stem cell transplant · Immunotherapy.

[4] Jul 13, 2021 — A combined treatment with HD-MTX, an alkylating agent (i.e. procarbazine, temozolomide) and rituximab is recommended. ... In responding patients, ...

[8] by T Calimeri · 2021 · Cited by 66 — Other treatment options, such as non-myeloablative high-dose chemotherapy, oral drug maintenance, and some targeted drugs like ibrutinib or ...

[9] CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) regimen represents the backbone for treating extra-CNS DLBCL but exhibits negligible activity in ...

The differential diagnosis for primary diffuse large B-cell lymphoma (DLBCL) of the central nervous system (CNS) is a crucial aspect of its identification and treatment.

Key Differential Diagnoses:

• Central Nervous System Gliomas: These are tumors that arise from the brain or spinal cord, and can present with similar symptoms to PCNSL. [5]
• Metastatic Tumors: Cancer that has spread to the CNS from another part of the body can mimic the presentation of PCNSL. [7]
• Demyelinating Disorders: Conditions such as multiple sclerosis or acute disseminated encephalomyelitis (ADEM) can present with similar imaging findings to PCNSL. [3]
• Subacute Infarcts: These are areas of brain tissue that have died due to lack of blood supply, and can be mistaken for PCNSL on imaging studies. [7]

Other Considerations:

• B-lymphoblastic Lymphoma/Leukemia (Positive Immunophenotype): This is a rare type of lymphoma that can present with similar morphology to PCNSL. [10]
• Central Nervous System Diffuse Large B-cell Lymphoma: This is the correct diagnosis, but it's essential to rule out other differential diagnoses before confirming this.

Clinical Features and Diagnosis:

The diagnosis of primary CNS DLBCL typically involves a combination of clinical features, imaging studies, and histopathological examination. [9] The clinical presentation can vary widely, but common symptoms include headache, confusion, seizures, and focal neurological deficits. [4]

Extent of Disease Evaluation:

Accurate staging of PCNSL is critical for determining the extent of disease and planning treatment. This typically involves a combination of imaging studies, including MRI and CT scans, as well as cerebrospinal fluid (CSF) analysis. [9]

References:

[1] by B Thirunavukkarasu · 2021 · Cited by 2 [3] by I Siasios · 2015 · Cited by 8 [4] Jul 6, 2021 [5] Oct 13, 2024 [7] by V Sharma · 2019 [9] Oct 10, 2024 [10] by MIS Lauw · 2020 · Cited by 41