Primary natural killer/T-cell lymphoma presenting as leptomeningeal disease

Liao, Bing; Kamiya-Matsuoka, Carlos; Gong, Yun; Chen, Merry; Wolf, Brian; Fowler, Nathan

doi:10.1016/j.jns.2014.05.015

Cited by 14 publications

(26 citation statements)

References 20 publications

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“…Three of the 8 cases (Cases 2, 6, and 8 in Table) were treated with systemic high-dose MTX that did not prevent ENKL development; those patients died 2-18 months after diagnosis. In one patient (Case 8 reported by Liao et al), however, sequential chemotherapy including high-dose MTX (dose not shown) was effective (20). In our patient, high-dose MTX at 3.5 g/m 2 was also successful in achieving sustained CR for 8 months.…”

Section: Discussionmentioning

confidence: 97%

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Development of Extranodal NK/T-cell Lymphoma Nasal Type in Cerebrum Following Epstein-Barr Virus-positive Uveitis

et al. 2017

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A 74-year-old woman developed bilateral uveitis with high Epstein-Barr virus (EBV) DNA load in the vitreous fluid without lymphoma cells. Four years after the onset, T2-weighted contrast-enhanced MRI revealed hyperintense lesions in the right occipital and parietal lobe. A biopsy resulted in the diagnosis of extranodal NK/T-cell lymphoma nasal type (ENKL). The repeat region of LMP1, an EBV gene, detected in the brain lesion was identical to that detected in the vitreous fluid. ENKL of the central nervous system is quite rare, and the pathogenesis has not been determined. The lymphoma in this case might have been closely associated with the EBV-positive uveitis.

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Section: Discussionmentioning

confidence: 97%

“…The patient passed away due to hemophagocytic lymphohistiocytosis 26 days after the first visit. There have been eight reported cases of ENKL with primary CNS lesions (Table) (13-20). Case 3 reported by Cobo et al had acquired immune deficiency syndrome (AIDS) (15).…”

Section: Discussionmentioning

confidence: 99%

Development of Extranodal NK/T-cell Lymphoma Nasal Type in Cerebrum Following Epstein-Barr Virus-positive Uveitis

et al. 2017

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“…A total of 10 cases were collected from case reports in the English language literature (Table ) and analysed . There were nine IC cases and one IS case that was infected with HIV.…”

Section: Discussionmentioning

confidence: 99%

“…according to the 2008 World Health Organization (WHO) classification . Moreover, published case reports were reviewed, and 10 additional cases were selected for further analysis . The clinical and pathological information was obtained from a review of the papers.…”

Section: Methodsmentioning

confidence: 99%

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

et al. 2017

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“…The nasal cavity is the most common and prototypic site of involvement, but other primary sites have also been reported, including the paranasal sinuses, pharynx, skin, gastrointestinal tract, testis and CNS. [12][13][14] Metastasis to the brain from nasal NK/T-cell lymphomas has been reported in less than 3% of cases. 15 ENKL developing within the CNS is rare (Table 3).…”

Section: Discussionmentioning

confidence: 99%

Extranodal NK/T‐cell lymphoma, nasal type, manifesting as rapidly progressive dementia without any mass or enhancing brain lesion

et al. 2016

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Among the many potential etiologies for rapidly progressive dementia (RPD), primary central nervous system extranodal NK/T-cell lymphoma, nasal-type (ENKL) is a rare entity. We present the first reported case of autopsy-proven RPD due to ENKL without any mass or enhancing lesion of the brain. A 54-year-old immunocompetent man presented with RPD, myoclonus and ataxia. The mini-mental state examination (MMSE) score was 22/30. His brain MRI revealed progressive brain atrophy without gadolinium enhancement or mass lesion. Five months after the initial evaluation, cognitive impairment further worsened with an MMSE score of 3/30. At the advanced stage, lumbar MRI showed swollen cauda equina with gadolinium enhancement. The number of Epstein-Barr virus (EBV) DNA in cerebrospinal fluid had gradually increased. Twelve months after onset, the patient died of respiratory failure. Pathological findings revealed that lymphoma cells had diffusely invaded the meninges, parenchyma of the brain, spinal cord and cauda equina. Cells were positive for CD3, CD56 and EBV-encoded small RNAs and negative for CD20. No evidence of malignancy was identified in the visceral organs. This report indicates that ENKL should be recognized as one of the rare causes of RPD. Early testing for EBV-DNA in cerebrospinal fluid and imaging of cauda equina would be useful diagnostic tools.

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Primary natural killer/T-cell lymphoma presenting as leptomeningeal disease

Cited by 14 publications

References 20 publications

Development of Extranodal NK/T-cell Lymphoma Nasal Type in Cerebrum Following Epstein-Barr Virus-positive Uveitis

Development of Extranodal NK/T-cell Lymphoma Nasal Type in Cerebrum Following Epstein-Barr Virus-positive Uveitis

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

Extranodal NK/T‐cell lymphoma, nasal type, manifesting as rapidly progressive dementia without any mass or enhancing brain lesion

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