Duodenum-preserving total pancreatic head resection for IPMN, MCN, serous cystadenoma (SCA), and cystic EN lesions is a safe and beneficial surgical procedure. Segmental resection of the duodenum was applied for an oncologically complete resection. In regard to long-term outcome, the procedure is, additionally, a pancreatic cancer preventive strategy.

We report on three female patients with solid pseudopapillary tumors of the pancreas. The histogenetic origin of this entity is still unclear. The tumor, usually occurring in young women, forms large masses (up to 10 cm in diameter) before becoming symptomatic. Metastases have very rarely been reported. In contrast to other pancreatic tumors, the main pancreatic duct was displaced, but of normal caliber without stenosis, in all our patients. Despite the large size of the tumors, they were curatively resected in all three cases. Two of the tumors infiltrated the parenchyma or adipose tissue of the pancreas but did not spread into the lymph nodes or other organs. All of the patients are alive and without signs of tumor recurrence up to 8 years after surgical resection.

This case is the first presentation of the coincidence of a neuroendocrine carcinoma of the duodenum with a jejunal bleeding gastrointestinal stromal tumor in neurofibromatosis type1 which led to hemorrhagic shock. In neurofibromatosis -- even if non-symptomatic -- the increased incidence of tumor needs to be considered.

A duodenum-preserving total pancreatic head resection is a limited surgical procedure for patients who suffer a local monocentric, cystic neoplastic lesion in the pancreatic head. Absence of an advanced pancreatic cancer and completeness of extirpation of the benign tumor determine the long-term outcome. In regards to the location of the lesion in the pancreatic head, several modifications have been applied with low hospital morbidity and mortality below 1%.

Cystic neoplasms of the pancreas are being detected and surgically treated increasingly more frequently. Intraductal papillary mucinous neoplasms (IPMN) and mucinous cystic neoplasms (MCN) are primary benign lesions; however, the 5-year risk for malignant transformation has been estimated to be 63 % and 15 %, respectively. Surgical extirpation of a benign cystic tumor of the pancreas is a cancer preventive measure. The duodenum-preserving total pancreatic head resection technique (DPPHRt) is being used more frequently for cystic neoplasms of the pancreatic head. The complete resection of the pancreatic head can be applied as a duodenum-preserving technique or with segmental resection of the peripapillary duodenum. Borderline lesions, carcinoma in situ or T1N0 cancer of the papilla and the peripapillary common bile duct are also considered to be indications for segmental resection of the peripapillary duodenum. A literature search for cystic neoplastic lesions and DPPHRt revealed the most frequent indications to be IPMN, MCN and SCA lesions and 28 % suffered from a cystic neoplasm with carcinoma in situ or a peripapillary malignoma. The hospital mortality rate was 0.52 %. Compared to the Whipple type resection the DPPHRt exhibits significant benefits with respect to a low risk for early postoperative complications and a low hospital mortality rate of < 1 %. Exocrine and endocrine pancreatic functions after DPPHR are not impaired compared to the Whipple type resection.