Extranodal Natural Killer/T-Cell Lymphoma: An Incidental Finding

Abstract

Extranodal natural killer/T-cell lymphoma (ENKTCL) is a rare form of non-Hodgkin lymphoma. This neoplasm is more prevalent in regions of Asia and Latin America and most commonly involves the sinonasal tract, presenting with signs of nasal obstruction, epistaxis, or sinus infection. It is a locally destructive and angioinvasive neoplasm. The treatment of ENKTCL is dependent on the extent of the tumor. For localized disease, the treatment is chemoradiation. For disseminated disease, treatment is mainly chemotherapy-based. This report describes a case of a 41-year-old Hispanic woman who initially presented with signs of nasal congestion for four weeks and was subsequently diagnosed and treated for chronic sinusitis. The patient underwent endoscopic surgery for persistent chronic sinusitis, with a presumptive diagnosis of allergic fungal rhinosinusitis based on clinical and radiographic presentation. The pathologic exam revealed a diagnosis of ENKTCL. The patient underwent three cycles of chemotherapy comprised of steroid (hydrocortisone), methotrexate, ifosfamide, pre-asparaginase, and etoposide (SMILE) followed by radiation, resulting in clinical and radiographic remission. On review of the literature, ENKTCL is very rare in the United States and diagnosis is commonly delayed due to non-specific signs. We report this case to increase awareness of this disease entity and remind clinicians to include this in the differential diagnosis of nasal obstruction.

A case report of Extranodal natural killer / T-cell lymphoma presenting as extensive myiasis

“…Extranodal NK/T-cell lymphoma (ENKTCL) is a rare form of non-Hodgkin lymphoma, more commonly found in Asia and South America, with strong association with EBV infection [ 5 ]. This disease most commonly involves the sinonasal cavity with male predilection (male to female ratio of 3:1) and a mean age of onset of 52 5 .…”

“…Patients with ENKTCL (nasal type) usually present with non-specific symptoms such as nasal obstruction, epistaxis and facial swelling [ 5 ]. However, as the disease progresses, patients may have midline facial destruction with eventual necrosis of upper anterior aerodigestive tract [ 1 ].…”

“…Immunohistochemistry staining of nasal ENKTCL are typically positive for CD2, cytoplasmic CD3e and CD56 but negative for surface CD3 [ 6 ]. Extent of disease are evaluated with computer tomography (CT) scan while magnetic resonance imaging (MRI) are used for visualization of soft tissue involvement [ 5 ].…”

Twelve months of oral ulceration, weight loss and unexplained fevers: a clinicopathologic conference

“…Radiotherapy given concurrently or sandwiched between courses of chemotherapy may confer survival advantage in cases of localised disease. More research is needed in this area to establish whether haematopoietic stem cell transplant may improve overall survival in patients with ENKL following chemotherapy.…”

Angiocentric Centrofacial Lymphoma as a Challenging Diagnosis in an Elderly Man

“…Angiocentric centrofacial lymphoma, now known as nasal-type extranodal natural killer T cell lymphoma (ENK/TL-NT) by the World Health Organization (WHO) since the year 2001 [1,2], is a neoplasm of destructive characteristics, but low incidence. It tends to affect mainly the nasal cavity and palate [3]. According to the 2016 WHO classification, ENK/TL-NT is considered within the category of mature T and NK neoplasms [1,4].…”