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Background: Primary lymphomas amount to less than 1% of the primary laryngeal tumors. It is the second most common primary hematologic tumor of the larynx after plasmacytoma. Although squamous cell carcinoma amounts to 90% of the laryngeal tumors, diagnosis of laryngeal lymphoma is very important as nonsurgical management is indicated in all stages of this disease. Case report: A case of 10-year-old boy with hoarseness of voice with direct laryngoscopic biopsy reported as diffuse B-cell lymphoma. The sections were positive for epithelial membrane antigen (EMA), LCA, CD138, CD4 and Mum1 and ALK. He was managed under MCP 842 protocol. He had complete response after the treatment and was symptom free after 13 months of therapy. Conclusion: Primary laryngeal lymphoma is a rare entity and should be managed with current chemotherapeutic protocol. Differentiating it between squamous cell carcinoma is important as management protocol in lymphoma is nonsurgical. Aid of immunohistochemistry helps in early diagnosis and management of these cases.

Anaplastic Lymphoma Kinase-positive Primary Diffuse Large B-cell Lymphoma of the Larynx: A Rare Clinical Entity