Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma: A Case of Extranodal Non-Hodgkin Lymphoma in the Jejunum with Invasion of the Abdominal Wall and Transverse Colon.

INTRODUCTION

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare extranodal lymphoma, accounting for <1% of non-Hodgkin lymphomas, which most commonly affects Asians. MEITL mainly occurs in the small intestine and is characterized by an aggressive clinical course with a poor prognosis. MEITL shows intractable symptoms and frequently causes perforation and bleeding of the gastrointestinal tract. Bowel perforation and/or bleeding often require emergency surgery.

CASE PRESENTATION: A 77-year-old woman developed left-sided pain nearly at the umbilicus. CT revealed jejunal wall thickening measuring 60 mm. The lesion was hypervascular and showed gradual enhancement. The irregularly shaped tumor invaded the abdominal wall and transverse colon. Endoscopic findings showed a large ulcerative mass accompanied by severe necrosis. Radiographic findings of a small bowel series revealed a long, narrowed segment. Partial resection of the jejunum and planned dissection of the regional lymph nodes were performed, and partial resections of the abdominal wall and transverse colon were performed simultaneously. Histopathologically, diffuse and monotonous proliferation of medium-sized atypical lymphocytes was observed, and invasion into the abdominal wall and transverse colon was confirmed. A definitive diagnosis of MEITL was made based on histopathological assessment and immunohistochemistry. PET/CT revealed multiple areas of increased uptake in the small intestine. Serum levels of soluble interleukin-2 receptor were progressively elevated over time. The patient refused further aggressive treatment for MEITL. She died because of disease progression 142 days after symptom onset.

CONCLUSIONS

Clinicians should be aware that Asian patients who have small intestinal lesions accompanied by refractory symptoms, including bowel perforation and/or bleeding, may have MEITL. Surgeons should work closely with clinicians, and emergency surgery should be considered for suspected bowel perforation and/or bleeding in MEITL patients.