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Midline destructive syndrome, a case of extranodal T/NK lymphoma associated with Epstein-Barr virus infection in a young patient
Síndrome destructivo de línea media, a propósito de un caso de linfoma extranodal tipo T/NK asociado a infección por virus de Epstein-Barr en un paciente joven
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Rojas Ríos A, Méndez Peñalosa N, Trujillo-Cárdenas JC, Brigitte Martínez E, Arias Correal J. Midline destructive syndrome, a case of extranodal T/NK lymphoma associated with Epstein-Barr virus infection in a young patient. Rev. colomb. hematol. oncol. [Internet]. 2025 Jun. 27 [cited 2025 Dec. 5];12(1):130-7. https://doi.org/10.51643/22562915.726
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Rojas Ríos A, Méndez Peñalosa N, Trujillo-Cárdenas JC, Brigitte Martínez E, Arias Correal J. Midline destructive syndrome, a case of extranodal T/NK lymphoma associated with Epstein-Barr virus infection in a young patient. Rev. colomb. hematol. oncol. [Internet]. 2025 Jun. 27 [cited 2025 Dec. 5];12(1):130-7. https://doi.org/10.51643/22562915.726
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Introduction: Extranodal T/NK lymphoma (ENKEL-NT) nasal type is a rare and aggressive form of non-Hodgkin's lymphoma (NHL) associated with Epstein Barr virus (EBV) infection, characterized by a neoplastic proliferation of the T/NK cell lymphoreticular system. Its incidence is variable, being common in regions such as Asia and Latin America. Clinical case: 30-year-old male patient, with no significant pathological or family history, call center employee, who consulted for involuntary weight loss of 10 kg associated with mass sensation, obstruction in the right nostril and cacosmia. Imaging studies showed a mass with involvement of the midline, subcutaneous cellular tissue, gums and hard palate. A biopsy of the mass was obtained with findings of atypical lymphoid proliferation and necrotic material, positive immunohistochemistry for CD56, CD4, CD2 and CD8 confirming ENKEL-NK nasal type. EBV was confirmed by serology and viral load. Discussion: ENKEL-NT is a tumor of unusual presentation and given its wide range of differential diagnoses can generate difficulties in diagnostic accuracy. Due to its location it can progress from an indolent and self-limited form to a disseminated form that can impact prognosis and management. Its definitive diagnosis is limited to histopathology and in situ hybridization for EBV. Conclusion: ENKEL NT is an unusual and aggressive form of NHL, being a diagnostic challenge due to its wide differential margin. Its histopathologic diagnosis in early stages could have an impact on disease control and survival.
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