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

Andreou, Anastasia; Thermos, Grigorios; Sklavounou‐Andrikopoulou, Alexandra

doi:10.1007/s12105-020-01182-8

Cited by 7 publications

(5 citation statements)

References 36 publications

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“…This reflects the fact that the nasal cavity and paranasal sinuses are the predilection sites for ENKTL, which is one of the predominant malignant lymphomas in the head and neck region, including the oral cavity. 7,8,[10][11][12] However, these reports were limited to cases in which lesions were identified on the soft or hard palate, and only a few, including this case, were found on the gingiva. 8 is moderate in volume and often pale, and (5) observation of azure granules in Giemsa-stained specimens.…”

Section: Discussionmentioning

confidence: 99%

A case of extranodal NK/T‐cell lymphoma, nasal type, diagnosed by scraping cytology of the maxillary gingiva

Niwa,

Mukai,

Kimura

et al. 2023

Diagnostic Cytopathology

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Extranodal NK/T‐cell lymphoma (ENKTL), nasal type, is often seen in the head and neck region, but there have been rare instances of this disease with initial presentation as a lesion in the oral mucosa. The patient, a woman in her seventh decade of life, presented with an ulcer in the maxillary gingiva, and scraping cytology and biopsy were performed. Cytological specimens showed solitary or small aggregating cells with marked atypia in a necrotic background. Tumor cells were detected that had various nuclear shapes and azure granules in the cytoplasm. Biopsy showed that the tumor cells had diffusely infiltrated or interdigitated into the subepithelium. Immunohistochemistry revealed that the tumor cells had T‐ and NK cell phenotypes and were Epstein–Barr virus‐encoded small RNA (EBER) positive, leading to a diagnosis of ENKTL. Thus, when nonepithelial tumor cells in a necrotic background and prominent atypia are found, as in this case, it is important to carefully observe for azurophil granules in the cytoplasm for differential diagnosis considerations.

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Section: Discussionmentioning

confidence: 99%

A case of extranodal NK/T‐cell lymphoma, nasal type, diagnosed by scraping cytology of the maxillary gingiva

Niwa,

Mukai,

Kimura

et al. 2023

Diagnostic Cytopathology

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“…Although early-stage localized NK/TL is curable with timely diagnosis, patients with more advanced diseases have a poor prognosis [ 12 ]. Furthermore, due to the rarity of extranodal NK/TL and the lack of sufficient clinical studies, there are a variety of treatment modalities for NK/TL, and the prognosis depends on the disease stage, location of involvement, and the patient's age [ 12 , 13 ].…”

Section: Discussionmentioning

confidence: 99%

Intraoral Extranodal Natural Killer Cell/T-Cell Lymphoma of the Hard Palate

Ahmadi-Motamayel,

Najar-Karimi,

Abbasi

et al. 2023

Case Reports in Dentistry

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Natural killer cell/T-cell lymphoma (NK/TL) is a rare subtype of non-Hodgkin’s lymphoma (NHL) associated with the Epstein-Barr virus (EBV) and requires early detection, which can be very challenging. NK/TL often arises in the nasal cavity and can then spread to the oral cavity, but the case presented here is an unusual presentation of NK/TL in a 66-year-old edentulous male patient who initially presented an intraoral exophytic lesion in the palate that appeared within 3 months. We report the present case to highlight the challenges of reaching an accurate diagnosis given the diverse clinical manifestations of NK/TL.

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“…Frontline chemotherapies such as dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide (SMILE) demonstrate some promise in relapsed and advanced-stage ENKL. In addition, these therapies exhibit a higher 5-year overall survival (OS) when combined with radiation, vs conventional regimens that integrate anthracycline regimens such as cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP), which show lower and suboptimal 5-year OS [ 2 , 3 , 5 – 7 , 17 – 21 ]. Retrospective studies appear to demonstrate superior outcomes from CMT compared with radiation or chemotherapy alone, and to our knowledge, there have been no prospective, controlled studies to date that draw direct comparisons.…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Aggressive Disseminated Nasal-Type Epstein-Barr Virus-Positive Extranodal Natural Killer/T-Cell Lymphoma with Bone Marrow Involvement

et al. 2023

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Patient: Female, 38-year-old Final Diagnosis: Nasal-type extranodal natural killer/T-cell lymphoma Symptoms: Facial pain • dyspnea • non-productive cough • nausea • vomiting • green nasal discharge • dark-brown eye discharge Clinical Procedure: — Specialty: Oncology Objective: Rare disease Background: Nasal-type extranodal natural killer/T-cell lymphoma (ENKL) is an exceedingly rare and aggressive subtype of non-Hodgkin lymphoma. The malignancy has both a high morbidity and mortality and is most commonly discovered in patients with advanced stages of the disease. As a result, early detection and treatment is tantamount to improving survival and minimizing lasting effects. Case Report: Herein, we report a case of nasal-type ENKL in a woman with facial pain and associated nasal and eye discharge. We highlight the histopathologic features from nasopharyngeal and bone marrow biopsy, which demonstrated Epstein-Barr virus-positive biomarkers of diffuse and subtle involvement, respectively, with associated chromogenic immunohistochemical staining. We also highlight existing therapy utilizing a combination of chemotherapy with radiation, as well as consolidation therapy, and suggest the need for further research of allogeneic hematopoietic stem cell treatment and the potential of programmed death ligand 1 (PD-L1) inhibition in managing nasal-type ENKL malignancy. Conclusions: Nasal-type ENKL is a rare subtype of non-Hodgkin lymphoma that is infrequently associated with bone marrow involvement. The malignancy has a poor prognosis overall and typically is discovered late in the disease course. Current treatment favors utilization of combined modality therapy. However, previous studies have been inconsistent in determining whether chemotherapy or radiation therapy can be used alone. Additionally, promising results have also been shown with chemokine modulators, including antagonistic drugs that target PD-L1, in refractory and advanced cases.

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

Cited by 7 publications

References 36 publications

A case of extranodal NK/T‐cell lymphoma, nasal type, diagnosed by scraping cytology of the maxillary gingiva

A case of extranodal NK/T‐cell lymphoma, nasal type, diagnosed by scraping cytology of the maxillary gingiva

Intraoral Extranodal Natural Killer Cell/T-Cell Lymphoma of the Hard Palate

A Rare Case of Aggressive Disseminated Nasal-Type Epstein-Barr Virus-Positive Extranodal Natural Killer/T-Cell Lymphoma with Bone Marrow Involvement

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