Journal of Cytology
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Year : 2021  |  Volume : 38  |  Issue : 4  |  Page : 233-234
Extranodal NK/T cell lymphoma presenting as premaxillary swelling: A cytological diagnosis

Department of Pathology, Maulana Azad Medical College, Delhi, India

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Date of Submission22-Sep-2020
Date of Decision22-Aug-2021
Date of Acceptance12-Nov-2021
Date of Web Publication20-Nov-2021

How to cite this article:
Sinha P, Kushwaha P, Gupta K, Singh M, Jain S. Extranodal NK/T cell lymphoma presenting as premaxillary swelling: A cytological diagnosis. J Cytol 2021;38:233-4

How to cite this URL:
Sinha P, Kushwaha P, Gupta K, Singh M, Jain S. Extranodal NK/T cell lymphoma presenting as premaxillary swelling: A cytological diagnosis. J Cytol [serial online] 2021 [cited 2023 Feb 2];38:233-4. Available from:

Dear Editor,

A 24-year-old woman presented with rapidly progressing painless left cheek swelling for 2 months. There was no history of fever, trauma, or pus discharge. On examination, the swelling was 4 cm × 4 cm, firm, fixed, non-tender and involved the lower eyelid. Contrast-enhanced computed tomography (CT) scan of a paranasal sinus with nose showed a heterogeneously enhancing mass in the maxillo-facial region destroying the left maxillary sinus and orbital floor [Figure 1]a.
Figure 1: (a) CECT Nose showing heterogenous mass. (b) FNAC smear showing cells with cytoplasmic vacuolation, nuclear notching and convulations. Giemsa stain ×600. (c) Cell Block showing similar cells. H and E ×100

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Fine-needle aspiration cytology (FNAC) was performed as per standard protocol using a 21-G needle; a cell block (CB) was also prepared. Giemsa-stained smears were moderately cellular, comprising predominantly medium to large atypical lymphoid cells in a hemorrhagic background. The cells had scant vacuolated cytoplasm, hyperchromatic irregular nuclei, nuclear notching, and multi-nucleation [Figure 1]b. Occasional cells showed tongue-like cytoplasmic projection and atypical mitosis. The findings were suggestive of high-grade non-Hodgkin's lymphoma (NHL). Immunohistochemistry (IHC) on CB was performed, which showed positivity for leucocyte common antigen (CD45) and CD3 (cytoplasmic staining) and was negative for cytokeratin, CD19, and LMP [Figure 1]c.

On flow cytometry (FC), these cells were immunoreactive for CD4, CD56, HLA-DR, CD34, and nonreactive for CD7, surface CD3, CD8, CD38, CD20, and CD19 [Figure 2]. EBV early RNA (EBER) in situ hybridization was positive for EBV infection. A diagnosis of extranodal NK/T cell lymphoma (ENKTL) was made. The biopsy attempted was inconclusive due to extensive necrosis. The patient received radiotherapy, showed improvement, and is on follow-up.
Figure 2: Flow cytometry on FNA sample showing immunoreactivity for CD4, CD56, HLADR and CD34

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ENKTL is an aggressive NK/T-cell neoplasm. It was earlier known as lethal midline granuloma as the lesions were mostly midline facial. The reported incidence is 3-6% of all NHL in Asia and Latin America.[1] ENKTL occurs mostly in the nose, nasopharynx, oropharynx, Waldeyer's ring, and upper aerodigestive tract; however, 20% of them occur in non-nasal sites e.g., skin, testis, breast, gastrointestinal tract, muscle, and salivary gland. Rarely, it can involve the liver, spleen, lymph node, and bone marrow.[2] It occurs in any age group (6-86 years) with a male-to-female ratio of 2:1 and commonly presents with nasal obstruction, purulent rhinorrhea, epistaxis, and sore throat.[3]

Cytological features as described in previous studies include moderate to highly cellular smears comprising of medium to large-sized cells with irregularly folded and indented nuclei and abundant pale blue to clear cytoplasm in a background of small lymphocytes.[4],[5] In some studies, a more polymorphic background with presence of neutrophils, eosinophils, and reactive histiocytes with emperipolesis is described. Mitoses is usually frequent. Some authors have also described cytoplasmic azurophilic granules and tongue-like cytoplasmic projections as characteristic features of ENKTL.[4],[5]

On FC, the cells are reactive for CD2, cytoplasmic CD3, CD56, HLA-DR, and cytotoxic molecules (granzyme B, TIA1, and perforin); nonreactive for surface CD3, CD4, CD8, and CD16, whereas CD7 is variable.[1] ENKTL is positive for EBV confirmed by in-situ-hybridization for EBER; LMP1 is usually negative.[1]

To conclude, ENKTL should be suspected on FNAC in a patient with sinonasal mass showing features of a high-grade NHL. Adequate material for FC and/IHC must be considered at the time of aspiration for timely and confirmative diagnosis to avoid rapid systemic dissemination.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Chan JKC, Quintanilla- Martinez L, Ferry JA. Extranodal NK/T-cell lymphoma, Nasal type. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Revised 4th ed. 2017. p. 368-71.  Back to cited text no. 1
Tse E, Kwong YL. The diagnosis and management of NK/T-cell lymphomas. J Hematol Oncol 2017;10:85.  Back to cited text no. 2
Mardi K, Madan S. Cytodiagnosis of extranodal natural killer/T-cell lymphoma, nasal type: Report of two cases. Clin Cancer Investig J 2014;3:398-400.  Back to cited text no. 3
  [Full text]  
Cho EY, Gong G, Khang SK, Kang YK, Huh J. Fine needle aspiration cytology of CD56-positive natural killer/T-cell lymphoma of soft tissue. Cancer 2002;96:344-50.  Back to cited text no. 4
Kaur K, Kakkar A, Bhardwaj N, Sakthivel P, Singh CA, Jain D, et al. Spectrum of cytomorphological features of extranodal NK/T-cell lymphoma, nasal type. Cytopathology 2019;30:393-401.  Back to cited text no. 5

Correspondence Address:
Dr. Meeta Singh
Room No 260, Maulana Azad Medical College, Bahadur Shah Zafar Marg, Delhi - 110 031
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JOC.JOC_225_20

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