Case Report: Primary NK/T Cell Lymphoma Nasal Type of the Colon With Multiple Intestinal Perforations

Abstract

Background: Extranodal NK/T cell lymphoma is a rare non-Hodgkin lymphoma mainly involving the upper aerodigestive tract, even rarer is primary extranasal disease involving the intestine. We present a case of primary intestinal NK/T cell lymphoma with diagnostic challenge, which eventually developed into multiple intestinal perforations.Case Presentation: A 35-year-old man presented with diarrhea and recurrent fever. Abdominal CT revealed multi-segmental intestinal wall thickening. Colonoscopy showed multiple irregular ulcers in colon. During the hospitalization, the patient developed intestinal perforation and an emergency surgery was performed. The resected specimen showed multiple perforations of the colon. The surgical samples underwent pathological analysis, and a diagnosis of extranodal NK/T cell lymphoma nasal type was confirmed. After recovering from surgery, the patient started receiving chemotherapy and PD-1 monoclonal antibody. Fortunately, he was discharged after significant improvement in his general condition. Eleven months follow-up was uneventful.Conclusion: Early diagnosis of primary intestinal NK/T cell lymphoma is frequently difficult. Most patients were definitely diagnosed only after surgical resection following complications, resulting in a poor prognosis. Therefore, doctors should maintain high suspicion of this malignancy for early diagnosis at an early stage clinically.

Primary colon natural killer (NK)/T-cell lymphoma, nasal type, with perforations: a case report and literature review

“…ENKTL-NT occurs primarily in the nasal cavity, and colonic primary cases are even rarer. A MEDLINE search revealed 9 unique cases of colonic primary ENKTL-NT-NT (Table 1) (11)(12)(13)(14)(15)(16)(17)(18)(19) (including the case reported here). In this case report, although the patient was eventually diagnosed with ENKTL-NT, the presenting symptoms were unusual.…”

“…We have no evidence whether the patient had a perforation of the colon before the onset of abdominal pain, but in most cases, the abdominal pain occurred before perforation. Laboratory investigations were not considered to be diagnostic, although lactate dehydrogenase (LDH) above the normal physiological range was specifically highlighted in a few cases, but the sample size was insufficient for statistical analysis (15,19,21). Similarly, imaging studies were unremarkable, with localized thickening of the intestinal wall insufficient to raise suspicion of ENKTL-NT (12).…”

“…Overall, the outcome of primary ENKTL-NT of the colon is not very favorable, especially for patients diagnosed at an advanced stage. Most patients have a poor prognosis, with an average survival of only 2.83-9.5 months (19). Prediction of prognosis and treatment outcome is necessary to enable timely adjustment of the treatment plan based on response.…”