Recurrent Epstein-Barr Virus-positive (EBV+) Primary Central Nervous System Lymphoma (PCNSL) in a Patient with Clinical Features of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS)

Nagano, Masahiro; Ayaki, Takashi; Koita, Natsuko; Kitano, Toshiyuki; Nishikori, Momoko; Goda, Naokazu; Minamiguchi, Sachiko; Ito, Akio; Takaori‐Kondo, Akifumi; Takahashi, Ryōsuke

doi:10.2169/internalmedicine.1246-18

Cited by 8 publications

(7 citation statements)

References 19 publications

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“…In addition, mixed T- and B- lymphocytes infiltrated in the perivascular space and glial stroma. Not only our case, but also a few previous case studies have reported minimal inflammation and a mixture of T- and B-cells without demyelination [ 3 , 12 ]. Thus, T-cell dominant lymphocytic infiltration and demyelinating features are not mandatory characteristics of heralding inflammatory lesions.…”

Section: Discussionsupporting

confidence: 69%

Inflammatory Brain Lesions as Omen of Primary Central Nervous System Lymphoma: A Case Report and Literature Review

Kim

Jung

et al. 2021

Brain Sciences

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We report a rare case that was initially diagnosed as an inflammatory lesion and ultimately confirmed as primary central nervous system lymphoma (PCNSL) in an immunocompetent patient who was not treated with corticosteroid prior to the initial biopsy. A 70-year-old female patient presented with numbness in the left side of face, arm, and leg. Brain magnetic resonance imaging (MRI) revealed a lesion with intense gadolinium (Gd)-enhancement in the ventral portion of the midbrain. A stereotactic biopsy demonstrated mixed T-cell and B-cell infiltrating inflammatory lesions without demyelination. Three months after postoperative treatment with steroid, the lesion markedly decreased on follow-up MRI. Twenty-six months after the initial attack, she complained of dysarthria and urinary incontinence. Repetitive MRI showed a lesion with homogeneous enhancement, extensively involving the bilateral cerebral hemisphere, corpus callosum, and the right middle cerebellar peduncle. The confirmed diagnosis was diffuse large B-cell lymphoma on the second biopsy. Despite our best efforts, she died 38 months after disease onset. Based on review of the literature and our case, preceding inflammatory lesions are not always demyelinating and T-cell dominant inflammatory lesions. When the initial biopsy reveals an inflammatory lesion in an old-aged patient, the clinician should keep in mind the development of PCNSL and perform close clinical and radiological observations for a timely diagnosis.

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Section: Discussionsupporting

confidence: 69%

Inflammatory Brain Lesions as Omen of Primary Central Nervous System Lymphoma: A Case Report and Literature Review

Kim

Jung

et al. 2021

Brain Sciences

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“…Nonetheless, the second histological analysis showed a mixed infiltration of T-and B-lymphocytes. Not only our case but also a few previous case studies have reported a mixture of T-and B-cells without demyelination [4,25]. Thus, the predominance of T-cell infiltration and demyelinating features are not mandatory characteristics of preceding inflammatory lesions.…”

Section: Discussionsupporting

confidence: 59%

Inflammatory brain lesions preceding primary central nervous system lymphoma: a case report and genetic analysis

et al. 2023

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Background Primary central nervous system lymphoma (PCNSL) is an aggressive extranodal lymphoma exclusively occurring within the central nervous system. Inflammatory brain lesions as “sentinel lesions” of PCNSL are very rare. We present a rare case of PCNSL with preceding inflammatory lesions in an immunocompetent patient who underwent two biopsies, one craniotomy and two genetic testing. Case report A 66-year-old male patient presented with left limb weakness and ataxia. Brain magnetic resonance imaging showed a contrast-enhancing lesion with perifocal brain edema in the near midline of right frontal lobe. Histological examination of a brain biopsy specimen revealed inflammatory lesion characteristics with infiltration of T-cell dominant lymphocytes and few B-cell. Given that the patient developed cerebral hematoma after biopsy, lesion resection by craniotomy was performed. An excised sample demonstrated mixed T-cell and B-cell infiltrating inflammatory lesions. Four months after total resection of the right frontal lobe lesion, another lesion appeared in the left frontal parietal lobe, which was diagnosed as diffuse large B-cell lymphoma by biopsy. In addition, genetic testing of the lesions at two different locations was performed, and the results showed that the inflammatory lesions had the same three gene (RELN, PCLO, and CREBBP) mutations as PCNSL. Interestingly, the three mutated genes are associated with tumor. Conclusion Our present case is the first to demonstrate inflammatory brain lesions heralding PCNSL from genetic and pathological perspectives. This may help clinicians to select new auxiliary diagnostic methods for timely diagnosis of patients with suspected PCNSL.

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“…Different hypotheses have been proposed regarding the relationship between lymphoma and CLIPPERS, which need further validation: (a) Lymphoma may be misdiagnosed as CLIPPERS at the onset or recurrence of symptoms. Studies reporting these cases highlight the role of biopsy and red flags such as inadequate response to corticosteroids in differentiating CLIPPERS from lymphoma 6–8,16 19 after a variable time in response to EBV reactivation, 11,20 chronic stimulation of perivascular antigens, 12 blood brain barrier dysregulation, 21 or compromised immune system resulting from treatment of CLIPPERS 9,10,12 ; (c) CLIPPERS may be a lymphocytic response through immune reconstitution or autoimmune reaction to various triggers such as HBV infection, influenza vaccination, or natalizumab withdrawal that may resolve or progress to more severe diseases such as lymphoma depending on management and other unknown prognostic factors 22,23 ; (d) CLIPPERS may be a spectrum rather than a disease caused by heterogeneous mechanisms.…”

Section: Discussionmentioning

confidence: 99%

“…3,5 To find previous lymphoma-associated cases up to November 2023, PubMed and Scopus online databases were searched using the keywords "CLIPPER" and "Lymphoma". To the best of our knowledge, 20 patients with lymphoma-associated CLIPPERS have been reported [6][7][8][9][10][11][12][13][14][15][16] since the first introduction of CLIPPERS. 2 A review of 10 patients by Zhang et al showed that patients with lymphoma-associated CLIPPERS had worse clinical outcomes and higher risk of mortality compared to those without lymphoma.…”

Section: Discussionmentioning

confidence: 99%

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids triggered by Hodgkin's lymphoma: A case report and brief literature review

Shervedani,

Tabibian,

Khotbesara

et al. 2023

Clinical Case Reports

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Key Clinical MessageWe presented a patient, diagnosed with lymphoma‐associated CLIPPERS, 11 years after lymphoma treatment. Therefore, CLIPPERS may be paraneoplastic neurological syndrome of lymphoma, which needs to be considered in the follow‐up of lymphoma cases.AbstractChronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare central nervous system disorder with a recent increase in incidence. There are few reports of lymphoma‐associated CLIPPERS, although the relationship between these two diseases and the pathophysiology of CLIPPERS in general need further investigation. Here, we present a patient with a history of Hodgkin's lymphoma (HL) more than 10 years before the onset of CLIPPERS, in contrast to the majority of previously reported lymphoma‐associated cases, and discuss the possibility that CLIPPERS is the paraneoplastic neurological syndrome of HL. This highlights the need to consider CLIPPERS as a differential diagnosis during follow‐up of patients with a history of lymphoma.

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Recurrent Epstein-Barr Virus-positive (EBV+) Primary Central Nervous System Lymphoma (PCNSL) in a Patient with Clinical Features of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS)

Cited by 8 publications

References 19 publications

Inflammatory Brain Lesions as Omen of Primary Central Nervous System Lymphoma: A Case Report and Literature Review

Inflammatory Brain Lesions as Omen of Primary Central Nervous System Lymphoma: A Case Report and Literature Review

Inflammatory brain lesions preceding primary central nervous system lymphoma: a case report and genetic analysis

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids triggered by Hodgkin's lymphoma: A case report and brief literature review

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