Background/aims: Total pancreatectomy is rarely performed as the treatment of pancreatic carcinoma because of markedly impaired quality of life and poor prognosis. Intraductal papillary-mucinous tumor (IPMT) of the pancreas is characterized by extensive intraductal spread and favorable outcome even in its invasive stage. The role of total pancreatectomy was reappraised in the treatment of IPMT.

Methodology: A total of five Japanese patients with IPMT underwent total pancreatectomy and their clinical follow-up data were reviewed.

Results: Total pancreatectomy was performed due to massive involvement of the entire pancreas in two patients, positive surgical margins on frozen section in one, benign IPMT with concomitant pancreatic cancers in one and recurrent IPMT in the remnant pancreas after distal pancreatectomy for IPMT in the other. Three of them underwent total pancreatectomy of the Whipple type, another underwent total gastrectomy and the other underwent the pylorus-preserving method. Surgical margins were negative by histology and no lymph node metastases were evident. Two patients had severe infection including liver abscess in one and pneumonia in the other. The former died on postoperative day 82 and the latter was controlled by medical treatment and discharged on postoperative day 73. The other three patients had an uneventful postoperative course and were discharged from 29 to 62 days after the operation. Long-term follow-up of the four patients revealed that three patients had hypoglycemic attacks, two diabetic retinopathy and two fatty liver. The four patients were doing well from 683 to 4,140 days after the operation without signs of recurrence.

Conclusions: Total pancreatectomy would be indicated as a treatment of benign or malignant IPMT with extensive involvement when patients' condition permits and gives a chance of cure, although careful long-term medical care and follow-up are essential.

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