Primary Pancreatic Lymphoma: The Role of Surgical Treatment

Abstract

Primary pancreatic lymphoma (PPL) is a rare disease that is difficult to diagnose preoperatively. We describe the youngest case of PPL treated by surgical excision and chemotherapy. A 16-year-old male presented with abdominal pain and jaundice. Abdominal computed tomography showed a 3.0 × 4.5 cm homogeneously enhanced mass localized between the inferior vena cava and pancreatic head; the common pancreatic duct was dilated and the common bile duct was stenosed. Magnetic resonance imaging findings showed a 4.5 cm tumor localized between the inferior vena cava and pancreatic head with low signal intensity on T1W images and high intensity on T2W images, which enhanced inhomogeneously. Endoscopic retrograde cholangiopancreatography findings were compatible with smooth stenosis of the common bile duct. He was diagnosed as pancreatitis secondary to pancreatic tumor and pylorus-preserving pancreaticoduodenectomy was performed. Postoperative diagnosis was PPL and chemotherapy was performed. After 4 years of treatment he has no signs of recurrence.

Fig. 1

Abdominal CT findings. A 3.0 × 4.5 cm tumor was localized between the inferior vena cava and pancreatic head which enhanced homogeneously with contrast administration (a). The common pancreatic duct was dilated (arrow) and the common bile duct was stenosed (b).

Fig. 2

MRI findings. There was a 4.5 cm tumor localized between the inferior vena cava and the pancreatic head which demonstrated low signal intensity on T1-weighted images (a) and high signal intensity on T2-weighted images and which enhanced inhomogeneously (b).

Fig. 3

ERCP findings. The common bile duct and the main pancreatic duct were stenosed smoothly without findings of infiltration (arrow).

Fig. 4

Pathology findings. Histopathology showed small tumor cells without cell adhesion or tissue structure proliferation (a, HE, ×400), and immunochemical staining was positive for B cell markers CD20 and CD79 and negative for T cell marker CD10 (b-d, ×400) compatible with the diagnosis of Hodgkin's diffuse B cell lymphoma.