Delayed Diagnosis of Nasal Natural Killer/T-Cell Lymphoma

Abstract

Midline destructive lesions of the face have multiple possible etiologies. The majority of these cases are found to be due to an extranodal lymphoma of natural killer/T-cell-type non-Hodgkins lymphoma (NKTL). Unfortunately, diagnosis is often delayed. With variable presenting complaints, including nonspecific issues like chronic rhinosinusitis or nasal congestion, initial treatments are aimed at these presumed diagnoses. Only as the lesion progresses do overt signs of destruction occur. As with our patient, who was initially treated for presumed infection and abscess, final diagnosis often does not occur until several months, and several antibiotic courses, from initial presentation. As such, it is important for this rare entity to remain in the clinician's differential diagnosis for nasal lesion.

Differential Diagnosis of Necrotizing Sinonasal Lesions

“…[30][31][32][33][34] Other names for this lymphoid neoplasm include lethal midline granuloma, polymorphic reticulosis, and midline malignant reticulosis, although the preferred terminology currently is extranodal NK/T-cell lymphoma, nasal type. These tumors are more commonly seen in males and most commonly seen in Asia as well as Central and South America.…”

“…These tumors are more commonly seen in males and most commonly seen in Asia as well as Central and South America. [30][31][32][33][34] The diagnosis is often difficult to make and is especially challenging early in the disease because patients initially present with nonspecific symptoms including nasal discharge, sinusitis, and headaches. Over time, the lesion becomes more destructive with extensive mucosal ulceration and destruction of bone and cartilage.…”

“…The lesional cells are positive for CD56, a marker for NK cell differentiation; CD2; CD3e; CD7; and CD8, and are often negative for CD3, CD5, CD16, and CD57. [30][31][32][33][34] The NK/T-cell lymphomas often express TIA-1, granzyme, perforin, and FAS. Epstein-Barr virus is almost always identified in lesional cells.…”

Determinants of response to daratumumab in Epstein-Barr virus-positive natural killer and T-cell lymphoma

“…Natural killer T-cell lymphoma (NKTL) is a highly malignant subtype of mature T and NK neoplasm characterized by Epstein-Barr virus (EBV) infection and often associated with destructive lesions in the sinonasal cavity and the upper aerodigestive tract. 1 This neoplasm makes up 5%-15% of all non-Hodgkin's lymphoma (NHL). Prognosis remains relatively dismal with a 5-year overall survival rate of 54% in patients with nasal disease and 34% in patients with extranasal disease.…”

Nasal Extranodal Natural Killer/T‐cell Lymphoma Presenting as a Nonmidline Lesion: A Case Report

“… 1 , 2 It presents as a midfacial ulceration resulting in progressive facial destruction with functional and aesthetic deformity. 3 It has a high prevalence among males in Asia and America. However, it is rare in the Western world, amounting to around 1% of the population.…”