Laryngeal Extranodal Nasal-type Natural Killer/T-cell Lymphoma

Abstract

Extranodal natural killer/T-cell lymphoma, nasal-type (ENKTL-N) initially presented in larynx is a rare condition without distinctive clinicopathologic features, with a challenging pathologic diagnosis. This study aimed to evaluate the clinicopathologic features and diagnosis of laryngeal ENKTL-N and spread awareness regarding ENKTL-N. A series of 31 cases of laryngeal ENKTL in one Chinese institution over a 9-year interval was retrospectively analyzed. Median age was 50 years (range, 13 to 77 y) with a male/female ratio of 5.2:1 (26/5). All patients initially presented with hoarseness and/or laryngalgia, and 10 patients (32.3%) experienced B symptoms. The supraglottic region was the most common site of occurrence (58.1%), the glottic area being the rarest site (6.5%). The mucosal squamous epithelium was detected in 26 specimens and pseudoepitheliomatous hyperplasia was observed in 8 cases (8/26, 30.8%). “Keratin-pearls” and a “pseudoinvasive” pattern were observed in 2 cases. Follow-up data were available for 26 patients (83.9%), the median survival duration was 9 months, and the overall survival rate at 5 years was 29.6%. Univariate analysis revealed that patients experiencing B symptoms (P=0.019) and age above 60 years had a significantly low survival (P=0.049) and that combined radiotherapy and chemotherapy prolongs overall survival (P<0.001). Laryngeal ENKTL-N is a rare entity with high aggressiveness and a poor prognosis. Multiple biopsies are usually required owing to secondary infection and massive necrosis. Laryngeal EKTL-N may mimic inflammatory lesions or well-differentiated squamous cell carcinoma. Therefore, clinical vigilance is essential to prevent misdiagnosis or a delayed diagnosis.

Intensive therapy can improve long‐term survival in newly diagnosed, advanced‐stage extranodal NK/T‐cell lymphoma: A multi‐institutional, real‐world study

“…In light of prognostication, consistent with multiple studies, 15,[17][18][19][20] ECOG score ≥2 and B symptom(s) were both independent risk factors for OS. In contrast with previous studies, 10,18 Aspa-based regimens are preferred according to guidelines.…”

“…In light of prognostication, consistent with multiple studies, 15,17‐20 ECOG score ≥2 and B symptom(s) were both independent risk factors for OS. In contrast with previous studies, 10,18 age >60 years was not a significant prognostic factor for OS in our study, probably because the median age was only 44 years and patients over 60 years accounted for a relatively small proportion.…”

Primary laryngeal T-cell lymphoma: A case report and review of the literature

“…CD43 was present in 3 cases and neoplastic cells contained CD2 and CD7 present in 2 cases. The Ki-67 index ranged 30%–80% [ 3 ]. Authors suggested that the outcome of laryngeal T lymphoma was bleak, more than half of them (73.1%) died with earlier treatment either from tumor dissemination or severe laryngeal complications, such as bleeding or obstruction [ 5 , 14 ].…”

“…It consists mainly of non-Hodgkin lymphomas (NHLs), represented particularly by diffuse large B-cell and mucosa-associated lymphoid tissues and are predominantly located in the supraglottic region, as this area of the larynx contains follicular lymphoid tissue [ 2 ]. Extranodal natural killer/T-cell lymphoma, located in larynx is a rare condition that accounts for less than 11% of all lymphomas without distinctive clinicopathologic features, as well as challenging pathologic diagnosis [ 3 ].…”

Laryngeal recurrence of extranodal NK/T cell lymphoma

“…ENKTL is an Epstein Barr virus-induced malignancy, which commonly affects the upper respiratory tract, particularly the nasal cavity, and laryngeal ENKTL is rare (incidence of laryngeal ENKTL is 1.7%) [2,3]. Notably, primary laryngeal ENKTL has been reported in <40 patients in the literature, and no studies have described laryngeal…”