Delayed Diagnosis of Nasal Natural Killer/T-Cell Lymphoma

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. 1. Introduction Midline destructive lesions (MDLs) of the face were first reported in the late nineteenth century. These later became known as lethal midline granulomas. Other monikers such as idiopathic midline granuloma, idiopathic midline destructive disease, midline nonhealing granuloma, polymorphic reticulosis, and Stewart syndrome have also been used to describe this destructive process of the nose. Regardless of the name used, an ulcerative process, characterized by loss of both epithelium and cartilage with associated nasal crusting, occurs. Ultimately, there is loss of nasal structure and support, leading to nasal deformity [1]. After thorough workup, the majority of these cases—as in the case presented in this paper—are found to be due to an extranodal lymphoma of natural killer/T-cell lymphoma (NKTL). Though rare, NKTL is the most common form of sinonasal lymphoma and, thus, should be included in the differential for destructive lesions of the nasal cavity or midline face. There is a strong association with Epstein-Barr virus (EBV) and other epidemiologic risk factors including Asian or Central and South American descent, male gender, and age 50 to 55 [2–4]. In the United States and other Western countries NKTL accounts for less than 2% of all non-Hodgkins lymphoma. Presentation is variable but most commonly includes nonspecific complaints of chronic rhinosinusitis, with initial treatments aimed at this presumed diagnosis. As the lesion progresses, patients may have additional complaints such as disruption of normal nasal airflow leading to epistaxis and nasal dyspnea, palatal destruction, associated orbital edema and erythema, and facial pain. Intranasal examination may reveal mild to significant crusting with extensive ulceration and destruction