Esophageal involvement by extranodal natural killer T cell lymphoma, nasal type, mimicking Ebstein Barr viral esophagitis in a tonsillar lymphoma patient undergoing chemoradiation therapy

Abstract

Esophageal involvement by extranodal natural killer (NK)/T cell lymphoma, nasal type, is rare. As a result, esophageal symptoms in these patients might at first be thought to originate from a benign condition, such as viral esophagitis. It is important to note, however, that benign conditions may mask esophageal involvement by lymphoma. Until now, there has been no report documenting esophageal involvement by lymphoma mimicking viral esophagitis in an extranodal NK/T cell lymphoma patient undergoing active treatment. Here, we report a case of esophageal involvement by extranodal NK/T cell lymphoma, nasal type, initially misdiagnosed as Ebstein Barr virus esophagitis. Lymphoma invasion of the esophagus should be considered if esophageal symptoms do not respond to usual medical esophagitis therapy in an extranodal NK/T cell lymphoma, nasal type, patient undergoing chemoradiation.

Radiological and clinical features of primary NK/T-cell lymphoma involving the whole length of the esophagus: A case report

“…Previous studies have demonstrated that Epstein-Barr virus (EBV) infection has been determined in virtually all patients with gastrointestinal NK/T cell lymphoma (20,21). Although the association between EBV and esophageal NK/T cell lymphoma is well-understood through previous clinical studies (5,22), it is typically controversial. Kim et al (20) identified that the frequency of EBV-positivity is decreased in the esophagus due to the limited number of EBV RNA tests available and the inclusion of CD3ε + CD56 − EBV.…”

“…Kim et al (20) identified that the frequency of EBV-positivity is decreased in the esophagus due to the limited number of EBV RNA tests available and the inclusion of CD3ε + CD56 − EBV. However, Lee et al (5) revealed that viral esophagitis may serve a role in the origin and development of NK/T cell lymphoma, and noted benign conditions that mimic viral esophagitis.…”

“…This diagnostic delay is due to the character of vascular invasion, which is a strong factor of inflammatory cell infiltration, and the result of large-scale necrosis is the main cause of misdiagnosing esophageal NK/T cell lymphoma. Esophageal NK/T cell lymphoma is diagnosed by immunophenotyping (5,6,11,12). The typical phenotype is the expression of NK cell markers (including CD56), T cell-associated antigens (including CD3 and CD2) and expression of cytotoxic markers (including TIA-1, perforin and granzyme B), with negative results for CD20 and CD23 (5,6,11,12).…”

“…Esophageal NK/T cell lymphoma is diagnosed by immunophenotyping (5,6,11,12). The typical phenotype is the expression of NK cell markers (including CD56), T cell-associated antigens (including CD3 and CD2) and expression of cytotoxic markers (including TIA-1, perforin and granzyme B), with negative results for CD20 and CD23 (5,6,11,12). The present case demonstrated the immune characteristics of tumor cells are typical of NK/T cell lymphoma: CD3 + , CD56 + , TIA-1 + and granzyme B + .…”

“…Fujihara et al (6) and Zhang et al (4) have suggested that systemic chemotherapy combined with concurrent radiotherapy may be an effective treatment for esophageal NK/T-cell lymphoma. Lee et al (5) identified a 45-year-old male with esophageal NK/T-cell lymphoma who was treated by salvage SMILE chemotherapy (dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide). Primary NK/T cell lymphoma of the esophagus is rare; however, pathological and immunohistochemical examinations of biopsy specimens may be useful in making an early diagnosis.…”