Rare central nervous system lymphomas

Abstract

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.

Intracranial Involvement of Systemic Hodgkin Lymphoma: A Case Report and Literature Review

“…Hodgkin lymphoma (HL), a hematopoietic malignancy, affects lymph nodes throughout the body [ 1 2 3 ]. While it is a significant malignancy, its progression to the central nervous system (CNS) is remarkably rare, with an incidence of only 0.02% [ 4 5 6 ]. Primary or secondary lymphomas account for 2.2%–4.0% of all brain tumors [ 5 7 8 9 10 ].…”

“…While it is a significant malignancy, its progression to the central nervous system (CNS) is remarkably rare, with an incidence of only 0.02% [ 4 5 6 ]. Primary or secondary lymphomas account for 2.2%–4.0% of all brain tumors [ 5 7 8 9 10 ]. In the spectrum of CNS lymphomas, diffuse large B-cell lymphoma (DLBCL) is the predominant histological subtype, comprising an estimated 90%–95% of cases [ 7 8 10 ].…”

Posterior fossa Hodgkin’s lymphoma radiographically mimicking an arteriovenous malformation: illustrative case

“…Hodgkin’s lymphoma (HL) is a hematological malignancy arising from B lymphocytes, in which central nervous system (CNS) involvement is extremely rare, seen in only 0.02%–0.5% of cases. 1 , 2 CNS HL typically presents as a supratentorial parenchymal lesion associated with various focal neurological deficits, seizures, and mass effect symptoms similar to those in other intracranial space-occupying lesions. 2 Histopathological diagnosis requires a tissue sample demonstrating the classic Reed-Sternberg cells expressing CD15 and CD30.…”

“… 1 , 2 CNS HL typically presents as a supratentorial parenchymal lesion associated with various focal neurological deficits, seizures, and mass effect symptoms similar to those in other intracranial space-occupying lesions. 2 Histopathological diagnosis requires a tissue sample demonstrating the classic Reed-Sternberg cells expressing CD15 and CD30. 1 , 2 The literature regarding the treatment of CNS HL is limited due to the disease’s rarity; however, case reports and series generally describe resection followed by a combination of radiation therapy (RT) and chemotherapy, such as doxorubicin, bleomycin, vinblastine, and dacarbazine.…”

“… 2 Histopathological diagnosis requires a tissue sample demonstrating the classic Reed-Sternberg cells expressing CD15 and CD30. 1 , 2 The literature regarding the treatment of CNS HL is limited due to the disease’s rarity; however, case reports and series generally describe resection followed by a combination of radiation therapy (RT) and chemotherapy, such as doxorubicin, bleomycin, vinblastine, and dacarbazine. 2 – 4 …”