Classical Hodgkin Lymphoma involving the central nervous system (brain) – an unusual presentation

Blydenstein, Sarah Alexandra van; Patel, Moosa; Philip, Vinitha; Lakha, Atul; Pather, Sugeshnee; Westgarth-Taylor, T; Thompson, Eddison; Kolloori, Avinash

doi:10.1002/ccr3.66

Cited by 21 publications

(22 citation statements)

References 20 publications

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“…Thus, the clinical features and outcomes are not well described. Our current knowledge and understanding of CNS HL is limited and derived from single reports or small case series, and the optimal therapeutic approach and course of disease after CNS involvement is unknown . To address this challenging and very unusual presentation of HL, we performed a multicenter international retrospective analysis.…”

Section: Introductionmentioning

confidence: 99%

Clinical characteristics and outcomes of patients with Hodgkin lymphoma with central nervous system involvement: An international multicenter collaboration

et al. 2016

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Central nervous system (CNS) involvement is rare in patients with Hodgkin lymphoma (HL). Thus, the clinical features and outcomes are not well described. Cases of histologically confirmed CNS HL diagnosed between 1995 and 2015 were retrospectively identified in institutional (n 5 7), national (n 5 2), and cooperative group (n 5 1) databases. We screened 30,781 patients with HL in our combined databases and identified 21 patients meeting eligibility criteria, an estimated frequency of 0.07%. CNS involvement was present at initial diagnosis in 10 patients (48%) and a feature of relapsed/refractory disease in 11 (52%). Among these 11 patients, the median time from initial diagnosis of HL to development of CNS involvement was 1.9 years (range 0.4-6.6) and the median number of prior lines of therapy was 2 (range 1-7). Altogether, treatments included radiation, multiagent systemic chemotherapy, combined modality therapy, and subtotal resection. The overall response rate was 65%. After a median follow-up of 3.6 years (range 0.8-13.2) from diagnosis of CNS HL, the median PFS and OS were 7.6 and 29 months, respectively. CNS involvement as a feature of relapsed/refractory disease was adversely prognostic for both PFS and OS; however, four patients remain alive and free of relapse at 7-78 months follow-up. CNS involvement in HL is exceedingly rare and has a distinct clinical presentation with predilection for parenchymal lesions with dural extension. Around one-quarter of patients, mostly with CNS involvement at initial HL diagnosis, experience prolonged disease-free survival.

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Section: Introductionmentioning

confidence: 99%

Clinical characteristics and outcomes of patients with Hodgkin lymphoma with central nervous system involvement: An international multicenter collaboration

et al. 2016

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“…Prognosis of this condition is also difficult to ascertain, as there is very limited data about its presentation and natural history. From the limited case reports, primary HL of the spine generally tends to be associated with very low risk of systemic relapse and overall, there is a better prognosis with HL isolated to the CNS, compared with systemic disease [ 7 , 33 ]. However, long-term remission rates are largely unknown and further cases are required to gain insight into this rare condition.…”

Section: Discussionmentioning

confidence: 99%

Primary intradural Hodgkin lymphoma of the conus medullaris and cauda equina: case report

et al. 2020

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Primary Hodgkin lymphoma of the central nervous system is an exceedingly rare condition with very few cases reported in the literature. Isolated intradural involvement of the spine is rarer still, with only two prior cases located in the extramedullary cervical and lumbosacral spine. We present a 48-year-old female who was presented with back pain, radiculopathy and a short history of sphincter disturbance and was subsequently found to have a lobulated homogenously enhancing exophytic lesion involving the conus medullaris and cauda equina on magnetic resonance imaging. Histopathological examination demonstrated the features of classic Hodgkin lymphoma. In this report, we present a case of primary intramedullary Hodgkin lymphoma involving the conus medullaris and cauda equina.

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“…Family history of HL and male gender have been considered risk factors. [13] The role of immunosuppression and Epstein–Barr virus infection in systemic and primary CNS-HL have also been suggested. [14] The presence of EBV in RS cells correlates with increased Tregs (CD4+CD25+FOXP3+) quantification in the tumor microenvironment and decreases the antitumor immunity toward RS.…”

Section: Discussionmentioning

confidence: 99%

Primary central nervous system Hodgkin Lymphoma: A case discussion and a hypothesis on the etiology

Alfaseh

Rajeh

Hamed³

2019

Avicenna J Med

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Hodgkin Lymphoma (HL) is a systemic disease with involvement of the cervical, supraclavicular, and mediastinal lymph nodes. It is commonly diagnosed in patients within the second and third decades of their lives. Diagnosis is usually made based on the distinct morphological and immunohistochemical characteristics, with the tissue biopsy being the cornerstone of workup. Extranodal presentation of HL is unusual and seldom encountered. Primary HL of the central nervous system (CNS) is exceedingly rare. We herein report a case of a 38-year-old male patient who was diagnosed with primary CNS-HL. The patient was treated with complete surgical resection followed by radiotherapy and chemotherapy. The patient was disease-free for 7 years postoperatively without any clinical evidence of relapse. We also discussed a possible role of CNS regulatory T-cells (Tregs) in developmental primary CNS-HL.

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Classical Hodgkin Lymphoma involving the central nervous system (brain) – an unusual presentation

Abstract: Key Clinical MessageIntracranial Hodgkin lymphoma (HL) is considered so atypical that an intracranial space-occupying lesion in a patient with known HL should be thoroughly investigated to rule out a second disease process.

Cited by 21 publications

References 20 publications

Clinical characteristics and outcomes of patients with Hodgkin lymphoma with central nervous system involvement: An international multicenter collaboration

Clinical characteristics and outcomes of patients with Hodgkin lymphoma with central nervous system involvement: An international multicenter collaboration

Primary intradural Hodgkin lymphoma of the conus medullaris and cauda equina: case report

Primary central nervous system Hodgkin Lymphoma: A case discussion and a hypothesis on the etiology

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