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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