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Background  Primary natural killer (NK)/T cell lymphoma of adrenal glands is an extremely rare tumor with aggressive clinical behavior. There have only been a few cases reported worldwide and the highest reported survival was 90 days. We report the first case of primary unilateral adrenal NK/T cell lymphoma in China with good outcome.    Case presentation  This is a 28-year-old man who presented with abdominal pain and was found to have a large mass on the left adrenal and the top of the renal region. The patient underwent surgical resection and the pathology revealed primary adrenal NK/T cell lymphoma. He received adjuvant sandwich therapy encompassing sequential chemotherapy, radiotherapy and chemotherapy. The patient remains clinically and symptomatically disease-free with over two years follow up.    Conclusion  Given the rarity of this disease, there is limited experience with regard to its diagnosis and treatment. This case report will add to the scant literature on this tumor and will be useful for the differential diagnosis and treatment of adrenal disease.

A case report of primary unilateral adrenal NK/T cell lymphoma: good clinical outcome with trimodality treatment