E. Panieri et al., OPERATIVE MANAGEMENT OF PAPILLARY CYSTIC NEOPLASMS OF THE PANCREAS, Journal of the American College of Surgeons, 186(3), 1998, pp. 319-324
Background: Papillary cystic neoplasm (PCN) is a rare malignant tumor
of the pancreas that typically occurs in young females and has an exce
llent prognosis.Study Design: We report a retrospective review of 12 p
atients treated during a 16-year period. Pre-, intra-, and postoperati
ve 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 pa
tients presented with an epigastric mass, and three with severe abdomi
nal pain. The correct diagnosis was made preoperatively in only five p
atients. 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 w
ere pylorus-preserving pancreaticoduodenectomy (three), central pancre
atectomy with pancreaticogastrostomy (three), distal pancreatectomy (t
hree), and local resection (three). In one patient two liver metastase
s were resected in addition to the pancreatic primary. One patient pre
sented with tumor rupture and a major bleed into the lesser sac and di
ed of multiple organ failure after resection. Postoperative complicati
ons included a stricture at the hepaticojejunostomy after pancreaticod
uodenectomy, which resolved after temporary stenting, and a pancreatic
duct fistula after local tumor resection, which required a distal pan
createctomy. Eleven patients are well at followup (mean 6.6 years; ran
ge 6 months to 15 years). Conclusions: PCN should be considered in the
differential diagnosis of large pancreatic masses, especially in youn
g females. Conservative resection, where technically feasible, is safe
and effective and represents;he therapy of choice. (C) 1998 by the Am
erican College of Surgeons.