Abstract:
:Natural killer (NK)/T-cell lymphomas represent a rare type of lymphoma derived from either activated NK cells or, rarely, cytotoxic T cells. These lesions are most commonly extranodal and tend to present as destructive lesions within the midline facial structures. Other than the nasal cavity and paranasal sinuses, several other extranodal sites of involvement have been reported, including the pharynx, gastrointestinal tract, and testis. Although secondary involvement of the central nervous system has been reported, a convincing case of primary brain NK/T-cell lymphoma has not been previously reported. Here, we report a case of primary brain lymphoma of NK/T-cell type with a characteristic phenotype expressing CD3epsilon, CD56, granzyme B, Epstein-Barr virus-encoded small nuclear RNAs, with germline T-cell receptor gene configuration, and showing an unusual intravascular component. The patient underwent extensive imaging studies, revealing only the brain lesion. The lymphoma failed to respond to therapy and the patient eventually died after transfer to a hospice facility. This unusual case highlights an unusual presentation of a rare disease entity and highlights the need for a better understanding of the biology and treatment of T-cell lymphomas.
journal_name
Hum Patholjournal_title
Human pathologyauthors
Kaluza V,Rao DS,Said JW,de Vos Sdoi
10.1016/j.humpath.2006.01.032subject
Has Abstractpub_date
2006-06-01 00:00:00pages
769-72issue
6eissn
0046-8177issn
1532-8392pii
S0046-8177(06)00078-5journal_volume
37pub_type
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