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Review
. 2021 Jun;15(2):621-627.
doi: 10.1007/s12105-020-01182-8. Epub 2020 Jun 25.

Extranodal NK/T Cell Lymphoma, Nasal Type with Palatal Involvement: A Rare Case Report and Literature Review

Anastasia Andreou  1 , Grigorios Thermos  2 , Alexandra Sklavounou-Andrikopoulou  1
Affiliations
Review

Extranodal NK/T Cell Lymphoma, Nasal Type with Palatal Involvement: A Rare Case Report and Literature Review

Anastasia Andreou et al. Head Neck Pathol. 2021 Jun.

Abstract

T-cell lymphomas are infrequently encountered in the head and neck area, with the most common subtype being Extranodal NK/T cell lymphoma, nasal type (ENKTL-NT). ENKTL-NT shows a predilection for midline facial structures presenting with ulcerative destructive lesions, whereas palatal involvement is one of the most prominent signs from the oral cavity. Herein, we describe a case of a 76-year-old Greek man with nasal obstruction and an extensive painful necrotic ulcer with ragged borders on the left distal portion of the soft palate and palatine tonsil of 4-months duration. After an initial non-diagnostic biopsy from the nasopharynx, a second incisional biopsy from the palatal lesions was performed. Histopathology was suggestive of an angiocentric lymphoproliferative neoplasm and immunohistochemical examination and in situ hybridization for EBV RNA led to a final diagnosis of ENKTL-NT. The patient was placed under combined chemotherapy and radiotherapy and no recurrence has been noted. Additionally, a retrospective review of the cases in the English literature with an established diagnosis of ENTKL-NT between 2000 and 2019, based on the latest WHO classification of Head and Neck tumors, is performed.

Keywords: EBV; Extranodal NK T cell; Lymphoma; Oral cavity; Palate; Ulcer.

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Conflict of interest statement

No conflict of interest to disclose.

Figures

Fig. 1
Fig. 1
Deep necrotic ulcer with ragged borders covered by a thick pseudomembrane and surrounded by erythema affecting the soft palate
Fig. 2
Fig. 2
a Diffuse infiltration consisting of clusters and sheets of large and/or medium-sized lymphoid cells extending to the adjacent mucous glands (hematoxylin and eosin, × 100 magnification) b Abundant immunohistochemical positivity for CD56 and c Abundant immunohistochemical positivity for CD3 d In situ hybridization. Diffuse positive Epstein-Barr virus–encoded RNA (EBER) staining
Fig. 3
Fig. 3
FDG-PET scan. Hypermetabolic areas in a the soft palate extending to the posterior boarder of the oropharynx and uvula and b left palatine tonsil
Fig. 4
Fig. 4
Complete remission of the oral lesions 5 months post-treatment
See this image and copyright information in PMC

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