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Introduction:
Marginal zone lymphoma corresponds to a sub-type of non-Hodgkin lymphoma stated to arise from post-germinal center memory B cells of marginal zone type that derive from and multiply anatomically in extranodal, splenic, and nodal tissues respectively in MALT, splenic, and nodal type lymphomas (1). Furthermore, Extranodal marginal zone lymphoma or MALToma is most commonly localized in the stomach; other common sites include the eyes and ocular adnexa, skin, lungs, salivary glands, breasts, and thyroid. It is rarely seen in the larynx which has only been reported in approximately 30 cases so far after the first case reported in 1990. (2) Herein, we present a case of supraglottic low grade marginal zone lymphoma of the larynx.
CLINICAL REPORT:
A 78-year-old male was evaluated in the ENT OPD of our hospital with the only complaint of progressive hoarseness for 6 weeks. He had no history of weight loss, fever, and night sweats. The past, family, and medical history of the patient were not of significant note. On physical examination, the superficial cervical lymph nodes were not enlarged. Plain and contrast-enhanced MDCT of the neck showed a welldefined lesion of size 22 x 17 mm predominantly involving the right side of the supra glottis of the larynx. In the post-contrast study, the lesion showed significant enhancement involving the right aryepiglottic fold and causing the ipsilateral pyriform sinus narrowing.
On nasopharyngolaryngoscopy, a right false vocal cord mass was seen. The specimen after direct laryngoscopy biopsy revealed multiple bits of gray-white to gray-brown soft tissue measuring 1x1x0.5 cm. Histologically, the section showed multiple fragments of tissue lined by stratified squamous epithelium. The subepithelium showed sheets of small monomorphic lymphoid cells having round hyperchromatic nuclei, inconspicuous nucleoli, and scant cytoplasm.(Figure 1 A) Extensive crushing artifactual changes were noted. These cells are also seen infiltrating in between mucus glands. (Figure 1 B) Few skeletal muscle fragments are also observed. Furthermore, immunohistochemistry revealed small B cells positive for CD20, BCL2, and CD21. Immunohistochemistry for CD3, CD10, CD5, CD43, CD23, CD138, and Cyclin D1 were negative. The above findings confirmed the diagnosis of low-grade extranodal marginal zone lymphoma.