Extranodal NK/T-cell lymphoma

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Extranodal NK/T-cell lymphoma

• Monirath Hav, MD, PhD fellow
• Pathology Department
• Ghent University Hospital

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General overview on hematopoiesis
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Relative frequencies of B-cell lymphoma subtypes
Anon. (1997). Blood 89 3909-3918
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Relative frequencies of mature T-cell lymphoma
subtypes
Armitage J et al.J Clin Oncol 26 4124-4130
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Introduction

• Extranodal NK/T-cell lymphoma
• More prevalent in Asians
• Often in adults (6th decade)
• More common in males (31)
• gt 95 associated with EBV
• Sites of involvement
• upper aerodigestive tract (nasal cavity )
• Extranasal including skin, soft tissue, GI tract,
  and testis
• Prognosis aggressive lymphoma
• Poor response to chemotherapy (MDR-1 )
• Median survival 12.5 months
• High local recurrence rate (21.4 Vs 5 in T cell
  lymphoma of nasopharynx)

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Morphology

• Angiocentric angiodestructive diffuse growth
  pattern
• Fibrinoid changes in Vx
• Coagulative necrosis
• Admixed apoptotic bodies
• Mitotic figures easily found
• Most often medium-sized cells
• Pale to clear cytoplasm
• Irregular, folded nuclei
• Inconspicous nucleoli

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Immunophenotype

• CD2
• Surface CD3 but cytoplasmic CD3
• CD56
• Granzyme B, TIA1, or perforin
• Occasional cases show CD7 CD30
• EBV

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Differential Diagnosis

• CD56 lymphoid neoplasms
• Aggressive NK cell leukemia systemic involvement
  (PB, LN, BM )
• Enteropathy-associated T-cell lymphoma
  associated with IEL
• Hepatosplenic T-cell lymphoma typical sites of
  involvement
• PCTCL subsets

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Case report

• 41 M
• Obstructing tumour of the nasal cavity
• Destruction of septal trabeculae