Enteric NK/T Cell Lymphoma-induced Recurrent Perforation in the Intestine: A Case Report

To report a rare case of recurrent intestinal perforation caused by primary intestinal extranodal NK/T-cell lymphoma (ENKTL), analyze the diagnostic challenges of this rare and aggressive malignancy, and raise clinical awareness for its early identification and optimal management in patients with unexplained gastrointestinal perforation.

A 31-year-old female presented with 3 days of persistent hypogastric abdominal pain and a 1-day history of high fever up to 39 ℃. She had undergone emergency surgery for jejunal perforation 20 months prior, with a postoperative pathological diagnosis of Epstein-Barr virus (EBV)-associated T- and NK-cell lymphoproliferative disorders. Multiple follow-up colonoscopies and bone marrow aspiration examinations failed to confirm the diagnosis of ENKTL, due to negative immunohistochemical staining of cluster of differentiation (CD)56 and EBV-encoded small RNA (EBER) in biopsy specimens. Abdominal contrast-enhanced computed tomography on this admission confirmed intestinal perforation, and emergency partial ileectomy was performed. Histopathological examination of the resected specimen showed diffuse full-thickness intestinal infiltration of atypical lymphoid cells, with positive immunohistochemical staining for CD2, CD3, CD56, T‑cell intracellular antigen‑1 (TIA-1) and EBER, and a Ki-67 proliferation index of approximately 70%. The final diagnosis of ENKTL (Ann Arbor stage IVA) was confirmed, and the patient received systemic chemotherapy with a regimen of methotrexate, etoposide, dexamethasone and pegaspargase after surgery.

The patient's general condition improved significantly after combined surgical intervention and systemic chemotherapy. During the 26-month continuous follow-up, the patient remained in good general condition, with no recurrence of abdominal pain, intestinal perforation, disease progression, or severe treatment-related adverse events.

Primary intestinal ENKTL is a rare and highly aggressive subtype of non-Hodgkin lymphoma, whose non-specific clinical and imaging manifestations pose major challenges for early diagnosis, which may lead to delayed treatment and unfavorable prognosis. This disease should be included in the core differential diagnosis for young patients, especially Asian populations, presenting with unexplained gastrointestinal perforation. Repeated deep tissue biopsy, EBER detection combined with comprehensive imaging evaluation are essential for early definitive diagnosis, and timely surgical intervention combined with standardized systemic chemotherapy can effectively improve the clinical outcomes of affected patients.