Primary NK/T cell lymphoma in the uterus: report of an unusual case

Natural killer/T-cell lymphoma (NKTCL) is a highly invasive non-Hodgkin lymphoma (NHL) characterized by extranodal onset, commonly observed in the nose and nasopharynx. NHL in the female reproductive system is rare, mostly secondary, occurring in the ovaries, cervix, uterine body, and endometrium. The prognosis of this disease is extremely poor, with most patients dying within a few months of diagnosis. Owing to its rarity, the prognostic factors are difficult to ascertain. Here, we report a case of extranodal NK/T-cell lymphoma in the uterus related to pregnancy.

A 31-year-old woman sought treatment for continuous vaginal discharge after cesarean section, however, anti-inflammatory treatment had no effect. A lump was found on the anterior wall of the uterus on transvaginal ultrasonography, so uterine lesion resection was performed. Owing to pathological misdiagnosis as uterine fibroids, treatment was delayed for 10 months. After readmission with a clear diagnosis, radiotherapy, chemotherapy, and autologous stem cell transplantation were done, and the disease was put into completely remission.

Primary uterine NKTCL is rare, and special attention should be given to women infected with Epstein-Barr Virus (EBV) during their pregnancy and post-partum period, especially patients with abnormal uterine bleeding, vaginal discharge, fever, or high LDH.