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Table 1 Reported cases of ENKTCL with low-grade histological findings

From: Rare case of low-grade extranodal NK/T-cell lymphoma, nasal type, arising in the setting of chronic rhinosinusitis and harboring a novel N-terminal KIT mutation

Case Citation Gender Age Site Presentation Angioinvasion/ necrosis Atypia Ki-67 Immunophenotype Description/Follow-up
1 Seishima et al. [16] F 60 Skin Recurrent swelling upper lip initially diagnosed as “lymphoid proliferation associated with EBV” No No NR CD45RO+, EBER+, CD56-, CD20- Developed ENKTCL-NT with angioinvasion and necrosis involving facial skin and nasal tract 9.5 years later, died despite treatment
2 Zhang et al. [17] F 53 Sinonasal History of chronic rhinosinusitis with nasal polyps removed 20 years prior; Progressive nasal obstruction No Mild 80% cCD3+, CD56+, Granzyme-B+, EBER+, TIA-1+, CD20- Treated with chemotherapy and radiation, disease-free at 4 month follow-up
3 Tabanelli et al. [18] M 52 Sinonasal History of chronic rhinosinusitis for 13 years with eventual sinonasal thickening obliterating right maxillary sinus and nasal turbinate No Mild Moderately high CD2+, CD3+, CD56+, Granzyme-B+, EBER+, CD4-, CD5-, CD7-, CD8- Initially diagnosed as EBV-associated proliferation, persisted for 2 years and diagnosed as ENKTCL-NT on a rebiopsy; long term follow-up not available
4–6 Hasserjian et al. [19] NR NR Sinonasal unknown No No NR NR Remained indolent for 10 years, recurred secondary to immunosuppression for renal transplant
NR NR Sinonasal unknown No No NR NR Bland histologic appearance resembling inflammatory infiltrate, no further description
NR NR Sinonasal unknown No No NR NR
7 Zuriel et al. [20] F 55 Skin Large red violaceous infiltrated plaques No No > 90% CD2+,cCD3+, CD56-, Granzyme B+, EBER+, CD4-, CD5-,CD8- Indolent course over 22 years with only local skin recurrences treated by local radiotherapy
8 Current Case F 71 Sinonasal History of longstanding chronic rhinosinusitis, sinonasal thickening/polyp No Mild < 1% CD2+, cCD3+, CD5(dim)+, Perforin+, Granzyme-B+, EBER+, CD56-, CD30- Treated with radiation, no recurrence at 6 month follow-up
  1. cCD3 Cytoplasmic CD3, NR Not reported