Primary laryngeal T- cell lymphoma
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Primary lymphoma of the larynx is extremely rare, representing less than 1% of all primary laryngeal neoplasms. It consists mainly of non-Hodgkin lymphomas (NHLs), represented particularly by diffuse large B-cell. Extranodal natural killer/T-cell lymphoma, presented 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.

A 64-years-old man, who was a heavy smoker, was admitted in 2018 to otolaryngology department for hoarseness and shortness of breath for 12 months. The patient did not suffer from fever, weight loss, or night sweats. There was no other personal nor familial medical history. On physical examination, there was no palpable lymphadenopathy and no hepatosplenomegaly.

Flexible fiberoptic nasolaryngoscopy revealed a large exophytic mass limited in the subglottic region without complete airway obstruction; the motion of the vocal folds was normal. A laryngeal computed tomography (CT) confirmed the existence of the subglottic tumor with no involvement of the vocal cords and no lymph node.

A direct laryngoscopy has been performed revealing a subglottic, submucosal lump. No other tumor was found in the rhinopharynx or in the Waldeyer's ring lymphoid tissue. A biopsy revealed a mantle cell type T NHL with the following antigen constellation: cluster of differentiation (CD)3+, CD8+ and the granzyme B + ; cells did not express CD20, CD5, PAX5, CD4, CD10 or CD30. Ki67 estimated at 70%. Full body PET scan did not showed any abnormality. Complete blood count (CBC) was normal thus, bone marrow biopsy did not show any anomalies.

The patient was referred to the hematology department for completion of staging and treatment where he received chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone) and radiation therapy (60 Gy). The patient was disease-free after 18 months follow up.



Read more : https://www.sciencedirect.com/science/article/pii/S2210261221003606?dgcid=rss_sd_all
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