Primary pulmonary non‐ Hodgkin's lymphoma ( NHL) is very rare. It represents less than 1% of all NHL, and 0.5–1% of all primary pulmonary malignancies. Almost all cases of primary pulmonary NHL originate from B‐cell lineage. We present a case of a 53‐year‐old man with primary extranodal NK/ T‐cell lymphoma of the bronchus and lung, presented progressive dyspnea caused by right lower lung consolidation, and pleural effusion. Initial chest computed tomography suggested advanced lung cancer. Bronchofiberscopy showed a polypoid tumor on which a biopsy was performed. Histologically, the diffusely infiltrative atypical cells were positive for cytoplasmic CD3, CD56, granzyme B, and negative for cytokeratin, CD20 immunostains, suggesting NK/ T cell lineages. In situ hybridization for Epstein‐ Barr virus encoded ribonucleic acid ( EBER) was positive. Herein, we discuss the clinicopathological features of this case and review the literature on primary extranodal NK/ T‐cell lymphoma of the lung. Compared with other patients, who died after the first cycle of chemotherapy and/or within three months, our patient had longer survival under aggressive chemotherapy and auto‐peripheral blood stem cell transplantation.
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