Natural killer-like T-cell lymphoma appearing in the duodenum with recurrence in a variety of extranodal organs

A 48-year-old man was admitted to Keio University Hospital in April 1995 with complaints of right abdominal pain and weight loss. Hypotonic duodenography showed a mass located in the 3rd portion of the duodenum. Endoscopic biopsy specimens disclosed diffuse large cell non-Hodgkin's lymphoma of the duodenum. The patient was initially treated with 2 courses of CHOP, albeit with no response. A pancreatoduodenectomy and radiotherapy yielded a complete remission. A year later, lymphoma recurred in the right mandible salivary gland, and a second complete remission was obtained after 6 courses of CHOP and radiation. However lymphomas also recurred in the intestine, and lungs, and the patient died of disease progression 38 months after diagnosis. Lymphoma cells were surface CD3 and CD56 positive. An examination of resected intestinal tissues disclosed lymphoma cells morphologically resemble large granular lymphocytes with rearranged TcR genes. These findings indicated the diagnosis of natural killer-like (NK-like) T-cell lymphoma. Compared with previously reported cases of NK-like T-cell lymphoma, this case was noteworthy for an unusual clinical course characterized by initial appearance in the duodenum, recurrence in a variety of extranodal organs, and the relatively long-term survival of the patient.