A Rare Case of Solid Pseudopapillary Tumor of the Pancreas

Abstract

Solid Pseudopapillary Tumor (SPT) of the pancreas is a rare epithelial tumor of low malignant potential. Its natural history differs from the more common pancreatic adenocarcinoma in that it has a female predilection, is more indolent, and carries a better prognosis. We are reporting a case of SPT of the pancreas diagnosed in a young female patient. An 18-year-old female presented with abdominal pain. The patient was otherwise healthy. Physical exam was unremarkable. Hematologic and metabolic parameters, including tumor markers, were within normal limits. MRI of the abdomen showed a 2.5 cm x 1.5 cm cystic lesion in the tail of the pancreas. Endoscopic ultrasound (EUS) of the lesion revealed a complex cystic mass with a thick wall and irregular internal solid components, measuring 2 cm x 1.5 cm. Fine needle aspiration was performed and 1.5 ml of serosanguinous fluid was obtained. The cyst was completely drained. Cytology revealed atypical papillary clusters with peri-capillary architecture. The patient underwent a laparoscopic distal pancreatectomy with splenectomy, as the tumor was invading the spleen. Pathology of the resected mass confirmed the diagnosis of SPT of the pancreas. Eight peripancreatic lymph nodes were resected and were all free of malignancy. Since the disease was localized and the excision was complete, the patient was not given adjuvant treatment. SPT is an uncommon pancreatic neoplasm, representing about 1-3% of exocrine pancreatic neoplasms. It usually occurs in young females. It has low-grade malignant potential and a favorable prognosis. A patient might present with abdominal pain, nausea, vomiting, a palpable abdominal mass, and occasionally jaundice due to obstruction of the bile duct. Most tumors are diagnosed through ultrasound or CT, but MRI also defines the hypervascular, well-encapsulated, round tumors with mixed cystic and solid components. EUS and FNA provide pre-operative diagnosis of SPT. Treatment involves complete excision of the tumor, which results in complete cure in majority of the cases. Overall 5-year survival following primary resection approaches 95%. In conclusion, a cystic pancreatic mass in a young female patient should raise suspicion for a solid pseudopapillary pancreatic tumor. The characteristic imaging features can help to differentiate SPT from other pancreatic tumors. Early detection is important, since timely resection usually results in long-term survival.2915_A Figure 1. T2-weighted MR of the abdomen, axial view, showing a solid pseudopapillary tumor in the tail of the pancreas.2915_B Figure 2. Serosanguinous aspirate obtained during EUS-FNA.2915_C Figure 3. H&E stain showing solid pseudopapillary tumor (T) and normal pancreas tissue (P)