3019 Solid Pseudopapillary Tumor of the Pancreas: A Typical Presentation in an Atypical Patient

Abstract

INTRODUCTION: Solid pseudopapillary tumor (SPT) of the pancreas is a rare neoplasm. It accounts for 1% of all tumors of the pancreas. It is a benign tumor with a low malignant potential and a favorable prognosis. It typically presents in young women, and on imaging it can be seen as a large encapsulated mass with solid and cystic components. The female to male predominance is 10:1. The following case shows a typical presentation of SPT in a male patient. CASE DESCRIPTION/METHODS: A 25-year-old man from Guatemala with no medical history presented with epigastric pain, nausea, vomiting, weight loss and poor oral intake. The abdominal pain was stabbing, intermittent, and radiating to the back. Family, social and surgical history were negative. Vitals were normal. On physical examination he appeared dehydrated with epigastric tenderness and a palpable left upper quadrant mass. Labs showed normal chemistry, liver function tests, lipase, CA 19-9, CEA and CA 125. Complete blood count showed an elevated white blood cell count of 23,000. Computerized tomography revealed a 7.6 × 10 × 8 cm heterogenous mass in the distal body and tail of the pancreas, with solid, and cystic components and calcifications. The patient underwent esophagogastroduodenoscopy (EGD) and endoscopic ultrasound with fine needle aspiration (FNA) of the mass. EGD showed a small non-bleeding duodenal ulcer. Pathology on the FNA revealed SPT. The patient underwent a distal pancreatectomy and splenectomy with negative margins, and a normal spleen. Lymph nodes were negative for malignancy. The patient did well post operatively with resolution of his symptoms. DISCUSSION: SPT is a rare tumor predominately seen in females. In men it tends to occur at an older age with an aggressive behavior. The origin of SPT is not clear. 60% of SPT are found in the body and tail of the pancreas. SPT is primarily benign but about 15% exhibit malignant features such as metastasis or invasion of adjacent structures. The most common sites of metastasis are the liver and peritoneum. Typical presentation is a palpable abdominal mass with symptoms of abdominal pain, nausea, vomiting and early satiety. The histopathology of SPT reveals solid nests of poorly cohesive cells resulting in a pseudopapillary architecture with positive nuclear staining for beta catenin, positive immunostaining for CD10, CD56, vimentin; they are negative for chromogranin. Cystic fluid is often bloody with low CEA and low amylase. Surgical resection is curative. The five-year survival rate is 95%.