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Natural killer cell malignancies: clinicopathologic and molecular features
L L P Siu1, J K C Chan, Y L Kwong
1Department of Medicine, Queen Mary Hospital, Hong Kong.
Histology and Histopathology
|April 19, 2002
Summary
Natural killer (NK) cell malignancies are distinct cancers with specific markers and associations, often affecting the nasal cavity. Further research into genetic changes is needed to understand their development and improve poor prognoses.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Natural killer (NK) cell malignancies are increasingly recognized as distinct clinicopathological entities.
- These tumors exhibit a specific immunophenotype (CD2+, CD3-, cytoplasmic CD3epsilon+, CD56+) and are often associated with Epstein-Barr virus.
Purpose of the Study:
- To characterize the clinicopathological, clinical, and molecular features of NK cell malignancies.
- To identify recurrent genetic aberrations for further investigation into molecular pathogenesis.
- To highlight the need for determining optimal treatment modalities due to poor prognosis.
Main Methods:
- Immunophenotyping (CD markers).
- T cell receptor gene analysis.
- Epstein-Barr virus association studies.
- Cytogenetic and molecular analysis to identify DNA losses and gains.
Main Results:
- NK cell tumors show variable morphology, angioinvasion, angiocentricity, and necrosis.
- Common clinical presentations include nasal/upper aerodigestive tract involvement (nasal NK cell lymphoma), extranasal sites, or aggressive disseminated disease (aggressive NK cell lymphoma/leukemia).
- Recurrent DNA losses at 6q, 11q, 13q, 17p and gains at 1p, 6p, 11q, 12q, 17q, 19p, 20q, Xp were identified.
Conclusions:
- NK cell malignancies represent a heterogeneous group of aggressive lymphoid neoplasms.
- Identified genetic aberrations provide targets for understanding pathogenesis.
- Optimal treatment strategies are urgently needed given the generally poor prognosis across all subtypes.