Clinicopathological analysis of primary central nervous system <scp>NK</scp>/T cell lymphoma: rare and localized aggressive tumour among extranasal <scp>NK</scp>/T cell tumours

Miyata‐Takata, Tomoko; Takata, Katsuyoshi; Kato, Seiichi; Hu, Lei Ming; Noujima-Harada, Mai; Chuang, Shih Sung; Sato, Yasuharu; Maeda, Yoshinobu; Yoshino, Tadashi

doi:10.1111/his.13223

Cited by 17 publications

(26 citation statements)

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“…[ 1 , 2 ] Extranodal NK/T cell lymphomas are commonly located in the upper respiratory and digestive tracts, such as the nasal cavity, and sometimes occur in the skin or gastrointestinal (GI) tract, but rarely invade the central nervous system (CNS). [ 3 ] Only a few cases of a NK/T cell lymphoma invading the CNS have been reported; [ 4 – 6 ] here, we reported a case of an extranodal NK/T cell lymphoma in the right middle cranial fossa, with diplopia and facial numbness as the initial presenting symptoms.…”

Section: Introductionmentioning

confidence: 78%

“…Several articles on extranodal NK/T cell lymphomas involving the CNS or primary CNS NK/T cell lymphomas reported treatment strategies, including MTX-based chemotherapy and radiotherapy. [ 3 , 6 ] However, this treatment strategy is the standard treatment regimen for B cell lymphomas; the curative effect on extranodal NK/T cell lymphoma remains unknown.…”

Section: Discussionmentioning

confidence: 99%

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Extranodal natural killer/T cell lymphoma, nasal type in the middle cranial fossa

2018

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Introduction:Extranodal natural killer (NK)/T cell lymphomas, nasal type, are aggressive, non-Hodgkin lymphomas. Extranodal NK/T cell lymphomas, nasal type, involving the central nervous system (CNS) are rare; therefore, delayed diagnosis easily occurs and is associated with a poor prognosis. Early diagnosis and patented systemic chemotherapy are necessary.Case presentation:We present a case of 34-year-old male with facial numbness and diplopia. He was diagnosed with extranodal NK/T cell lymphoma, nasal type, involving the CNS. The tumor, located in the right middle fossa, was subtotally removed, and 3 cycles of systemic chemotherapy were given. He later died of severe neutropenia and infection.Conclusion:NK/T cell lymphomas should be considered to be a potential cause of facial numbness and diplopia. A L-asparaginase-based regimen resulted in reasonable tumor suppression, but adverse effects, including fatal neutropenia, should be carefully considered.

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Section: Introductionmentioning

confidence: 78%

Section: Discussionmentioning

confidence: 99%

Extranodal natural killer/T cell lymphoma, nasal type in the middle cranial fossa

2018

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“…T-cell lymphomas account for approximately 2-4% of all primary CNS lymphomas in the West [28,29] with a higher incidence in Asia, ranging from 2 to 17% [30][31][32]. Primary CNS T-cell lymphomas (PCNSTL) include peripheral T-cell lymphoma, not otherwise specified, the most common subtype, ALCL, and extranodal NK/T-cell lymphoma [5,26,32,33]. In a series of 45 PCNSTLs from multiple institutions and countries, Shenkier et al reported a wide age range of presentation from 3 to 84 years with median age of 60 years and male predominance, similar to PCNSLs overall [27,34].…”

Section: Discussionmentioning

confidence: 99%

Central nervous system ALK-negative anaplastic large cell lymphoma with IRF4/DUSP22 rearrangement

et al. 2021

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Anaplastic large cell lymphomas (ALCL) are mature T-cell neoplasms, approximately half of which harbor rearrangements of the ALK gene that confer a good prognosis. Recent studies have demonstrated that a significant proportion of ALK-negative ALCLs demonstrate rearrangements of the IRF4/DUSP22 locus that also are typically associated with a favorable prognosis. ALCL with primary involvement of the central nervous system (CNS) is extremely rare. We report what may be the first case of ALK-negative ALCL with IRF4/DUSP22 rearrangement involving the brain in a 55-year-old man. Magnetic resonance imaging demonstrated signal abnormalities in the periventricular region, corpus callosum and cingulate gyrus. Biopsy revealed a diffuse parenchymal and angiocentric infiltrate of CD30-positive cells that showed IRF4/DUSP22 rearrangement by fluorescence in situ hybridization. We also review the clinical and pathologic features of primary CNS ALK-negative ALCLs in the literature and highlight the need for awareness of this entity to optimize appropriate management.

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“…The diagnoses were established either on biopsy or by the detection of malignant cells in the cerebrospinal fluid. 12 - 25 …”

Section: Discussionmentioning

confidence: 99%

“…In 1 study evaluating 14 cases (4 newly reported and 10 reported reviewed), none of the patients had B symptoms; however, just like our patient’s clinical course, all patients exhibited aggressive clinical behaviors. 22 …”

Section: Discussionmentioning

confidence: 99%

An Unusual Case of EBV-Negative Primary CNS Lymphoma of Natural Killer/T-Cell Lineage

Kajtazi

Bafaquh

Ghamdi

et al. 2021

Clin Med�Insights�Pathol

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Extranodal NK/T-cell lymphoma (ENKTL) is a well-defined cytotoxic lymphoma strongly associated with Epstein–Barr virus (EBV) infection, commonly affecting the nasopharynx and upper aerodigestive tract. Primary central nervous system (CNS) involvement is rare, and only 17 cases were previously reported in the literature. Here, we report the case of a 44-year-old male admitted with a 3-month history of personality changes and progressive right leg weakness. Brain magnetic resonance imaging studies (MRIs) revealed multiple rim-enhancing brain lesions bilaterally. An extensive clinical and laboratory workup was unrevealing, and 2 brain biopsies were initially considered inconclusive. Pertinently, no systemic lymphoproliferative disorder was identified. The patient initially experienced remarkable clinical improvement with dexamethasone, pulse methylprednisolone, and rituximab therapy. However, he eventually had rapid clinical deterioration, was found to have increased brain lesions, and died nearly 6 months after the initial presentation. During this time, the second brain biopsy was found to show involvement by T-cell lymphoma of NK-cell lineage, which was EBV negative. No post-mortem examination was done to identify any systemic lymphoma. This case serves to expand the spectrum of lymphomas involving the CNS.

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Clinicopathological analysis of primary central nervous system NK/T cell lymphoma: rare and localized aggressive tumour among extranasal NK/T cell tumours

Cited by 17 publications

References 20 publications

Extranodal natural killer/T cell lymphoma, nasal type in the middle cranial fossa

Extranodal natural killer/T cell lymphoma, nasal type in the middle cranial fossa

Central nervous system ALK-negative anaplastic large cell lymphoma with IRF4/DUSP22 rearrangement

An Unusual Case of EBV-Negative Primary CNS Lymphoma of Natural Killer/T-Cell Lineage

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