Operative management of papillary cystic neoplasms of the pancreas.

BACKGROUND

Papillary cystic neoplasm (PCN) is a rare malignant tumor of the pancreas that typically occurs in young females and has an excellent prognosis.

STUDY

DESIGN

We report a retrospective review of 12 patients treated during a 16-year period. Pre-, intra-, and postoperative data were evaluated in all patients to determine optimal management with specific reference to surgical strategy.

RESULTS

All 12 tumors occurred in young women (mean age 22 years, range 14-36 years). Six patients presented with an epigastric mass, and three with severe abdominal pain. The correct diagnosis was made preoperatively in only five patients. Incorrect diagnoses included hepatoma, pancreatic pseudocyst, and hydatid cyst. The PCNs had a mean diameter of 12.5 cm (range 8-20 cm), and occurred in the head (four), neck (three), body (three), and tail (two) of the pancreas. All were resected. Operations performed were pylorus-preserving pancreaticoduodenectomy (three), central pancreatectomy with pancreaticogastrostomy (three), distal pancreatectomy (three), and local resection (three). In one patient two liver metastases were resected in addition to the pancreatic primary. One patient presented with tumor rupture and a major bleed into the lesser sac and died of multiple organ failure after resection. Postoperative complications included a stricture at the hepaticojejunostomy after pancreaticoduodenectomy, which resolved after temporary stenting, and a pancreatic duct fistula after local tumor resection, which required a distal pancreatectomy. Eleven patients are well at followup (mean 6.6 years; range 6 months to 15 years).

CONCLUSIONS

PCN should be considered in the differential diagnosis of large pancreatic masses, especially in young females. Conservative resection, where technically feasible, is safe and effective and represents the therapy of choice.