Abstract：A boy aged 14 years had abdominal pain as the major manifestation, with elevated serum amylase and lipase. Abdominal ultrasound performed early after onset in another hospital showed enlargement of the pancreas and a reduction in echo. Magnetic resonance cholangiopancreatography (MRCP) showed pancreatic duct dilation and an unclear image of the head of the pancreas. Acute pancreatitis was considered. However, his symptoms were not relieved after fasting, fluid infusion, anti-acid therapy, and somatostatin therapy. Then, abdominal CT scan and MRCP found multiple low-density lesions of the pancreas and enlargement of the hilar and retroperitoneal lymph nodes. Exploratory laparotomy found pancreatic edema and multiple hilar nodules with unclear boundaries, and pathological biopsy showed anaplastic large-cell lymphoma. Since the liver, the spleen, bone marrow, and the central nervous system were not involved, he was diagnosed with stage Ⅲ primary pancreatic lymphoma. After vindesine and dexamethasone were used to reduce tumor load, the patient underwent vindesine-pirarubicin-asparaginase-dexamethasone chemotherapy once and vinorelbine-dexamethasone chemotherapy 8 times. Imaging examination still showed multiple low-density lesions of the pancreas and retroperitoneal lymph node enlargement. His parents discontinued treatment. It is concluded that the rare causes of acute pancreatitis with poor response to conventional treatment should be considered, especially for patients with abdominal lymph node enlargement. Extranodal lymphoma should be considered, and lymph node biopsy should be performed as early as possible to confirm diagnosis. The prognosis of pancreatic lymphoma is associated with clinical stage and pathology.
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