Two pp-omas of the head of the pancreas one of which with central necrosis fistulated into the duodenum

Abstract

PP omas are rare, usually malignant tumours of the PP cells of the Langerhan's islets which secrete pancreatic polypeptide. The authors present two women operated for PP-omas of the pancreas. The first was 55 year-old woman in whom we did a cephalic duodenopancreatectomy (Whipple's procedure) for the tumor of the head of the pancreas with central cavity containing gas due to communication with the duodenum. Immunohistochemistry showed a PP oma with strong generalised immunoreactivity with antibodies against Chromogramin A, neuron specific enolasa and PP with more the 95% of tumor cells and coexpression of somatostatine in 35% and VIP in less then 5% of tumor cells. Following uneventful recovery the patient stayed symptom free so far and put 20 kilograms in weight. The second patient was 19 year-old girl with a multinodal tumor of almost the entire pancreas in whom a local excision of the nodal mass of the head of the pancreas had been carried out in the other hospital, three years ago and relaparotomy and tumor biopsy a month before admission to our institution. In her we did a total duodenopancreatectomy and standard lymphadenectomy for a multinodal mass occupying almost the entire pancreas. Immunohistochemistry showed a strong generalised immunoreactivity with antibodies against Chromographin A, Neuron specific enolasa and PP for more then 95% of tumors, cells. Glucagon was expressed in few focuses (in less then 1% of cells), somatostatin was expressed very rarely in single cells while the rest of tumor markers did not show a visible immunologic reactions in the majority of tumors, cells. Three years after surgery she died due to multiple liver secondaries.

Sporadic Pancreatic Polypeptide Secreting Tumors (PPomas) of the Pancreas

“…The four sporadic PPomas presented have had a benign course that is different from that of PPomas presenting the context of MEN-1 syndrome, when the diagnosis implies a malignant tumor. Furthermore, only 8 of the 24 sporadic PPoma patients reported in the literature developed metastatic disease [8][9][10][11][12][13][14][15][16][17][18][19][20][21] when it did not necessarily imply a poor survival. Thus, sporadic PPomas seem to have a more benign course and because of this a limited procedure, such as careful enucleation with a limited normal tissue margin can be considered in selected patients.…”

“…Nonfunctioning neuroendocrine tumors are usually mixed, and increased PP cells are found in 20-67% [6,7]. Pure PPomas are rare, occurring in\1% of neuroendocrine tumors, and only 24 cases have been reported [8][9][10][11][12][13][14][15][16][17][18][19][20][21].…”