A rare case of monomorphic epitheliotropic intestinal T-cell lymphoma, presenting with spontaneous intestinal perforation.

Introduction And Importance: Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare and aggressive T-cell lymphoma that primarily affects the intestine. It has a poor prognosis and high mortality rate. Symptoms at presentation can be non-specific, and imaging studies may show similarities with nonmalignant conditions. The delayed clinical presentation and lack of targeted therapies contribute to the dismal prognosis of MEITL.

Case Presentation: We present a case of spontaneous intestinal perforation caused by primary intestinal T-cell lymphoma, emphasizing the importance of early recognition of this uncommon cause of perforation. Identifying it is crucial for prompt surgery and chemotherapy for this rare disease.

Clinical Discussion: The most common site of involvement in MEITL is the small intestine, especially the jejunum. The prognosis of MEITL is poor. Early diagnosis of primary intestinal T-cell NHL is challenging due to its rarity and non-specific symptoms. Imaging and endoscopy may show certain features, but a definitive diagnosis relies on biopsy and histopathologic analysis. To date, no efficient therapeutic interventions have been demonstrated for the management of this entity. The standard management strategy consists of induction chemotherapy followed by autologous stem cell transplantation.

Conclusion: This case report highlights that spontaneous perforation with peritonitis could be a potential presenting sign of MEITL. The diagnosis of MEITL is mainly based on histopathologic examination, so an accurate diagnosis necessitates clinical knowledge and thorough biopsy with immunohistochemistry and molecular testing.