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Laryngeal extranodal nasal-type natural killer/T-cell lymphoma: A clinicopathologic study of 31 cases in China

American Journal of Surgical Pathology Jun 18, 2019

Xiang CX, et al. - The clinicopathologic features and diagnosis of laryngeal extranodal natural killer/T-cell lymphoma, nasal-type (ENKTL-N) were evaluated in addition to spreading awareness regarding ENKTL-N via retrospectively analyzing a series of 31 cases of laryngeal ENKTL [median age: 50 years (range, 13 to 77 y); male/female ratio: 5.2:1 (26/5)] over a 9-year interval from a Chinese institution. The initial presentation comprised hoarseness and/or laryngalgia in all cases, and B symptoms in 10 patients (32.3%). The most common site of occurrence was the supraglottic region (58.1%) and the rarest site being the glottic area (6.5%). Twenty-six specimens showed the mucosal squamous epithelium with 8 cases displaying pseudoepitheliomatous hyperplasia (8/26, 30.8%). Two cases had “Keratin-pearls” and a “pseudoinvasive” pattern. As per the available follow-up data of 26 patients (83.9%), the median survival duration of 9 months, and the overall survival rate at 5 years of 29.6% were noted. As per the univariate analysis, significantly low survival was suggested for patients experiencing B symptoms and age above 60 years and prolongation of overall survival could be achieved with combined radiotherapy and chemotherapy. Researchers identified laryngeal ENKTL-N as a rare entity but with high aggressiveness and a poor prognosis. Due to secondary infection and massive necrosis, usually multiple biopsies are required. Laryngeal EKTL-N may mimic inflammatory lesions or well-differentiated squamous cell carcinoma. Therefore, they recommend being vigilant clinically to prevent misdiagnosis or a delayed diagnosis.
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