review of literature on clinical pancreatology - The Pancreapedia

Small cell carcinoma <strong>of</strong> the pancreasA 58-year-old man who complained <strong>of</strong> an abdominal tumor was admitted to hospital.Abdominal CT scan showed that a 15-cm tumor occupied the entire right upper abdomenand that there were ascites and liver metastases. A liver biopsy was performed. The liverbiopsy showed a small cell carcinoma pattern, but no definitive origin <strong>of</strong> the tumor wasdetermined. Considering the extensive peritoneal invasion and multiple liver metastases, thepatient received 2 courses <strong>of</strong> cisplatin/etoposide chemotherapy, but his tumor became largerwith concomitant abdominal pain and nausea. The patient suddenly died due to multipleorgan failure caused by tumor necrosis. The autopsy revealed a pathological diagnosis <strong>of</strong>primary small cell carcinoma <strong>of</strong> the pancreas [619].Non pancreatic periampullary tumorsLymph node ratio (LNR) has been associated with long-term survival in patients withpancreatic adenocarcinoma; however, this has not been demonstrated in other periampullarymalignancies. The purpose <strong>of</strong> one study was to determine if LNR is associated with survivalin other periampullary malignancies. A retrospective <strong>review</strong> <strong>of</strong> a prospective database <strong>of</strong> 522pancreaticoduodenectomies performed between 1988 and 2007 was undertaken. Patientswith positive lymph node status were placed into the following groups: LNR = 0; LNR < 0.2;LNR < 0.4; and LNR >0.4. Of the 364 malignancies identified, there were 219 (60 %)pancreatic adenocarcinomas, 36 (10 %) duodenal adenocarcinomas, 75 (21 %) ampullaryadenocarcinomas, and 35 (10 %) cholangiocarcinomas. Positive lymph node status affectedpatient survival in all malignancies studied. Increasing LNR was significabtly associated withdecreased survival in both pancreatic and perimapullary cancers [620].Papilla <strong>of</strong> Vater tumorsCarcinoids <strong>of</strong> the ampulla <strong>of</strong> Vater are the most rare primary ampullary tumors. There wasnoted a frequent association <strong>of</strong> the endocrine tumors with type 1 neur<strong>of</strong>ibromatosis alsoknown as von Recklinghausen disease. There are only 8 cases <strong>of</strong> papilla duodenalis minorcarcinoids described in the <strong>literature</strong>. Authors describe herein the first carcinoid <strong>of</strong> papilladuodenalis minor case associated with multiple synchronic jejunal leiomyomas and vonRecklinghausen disease, manifested with proximal intestinal obstruction [621].One paper reported a case <strong>of</strong> a carcinoma that probably developed from the peribiliary glandwithin the ampulla <strong>of</strong> Vater based on the histopathological findings <strong>of</strong> the resectedspecimens. There were no malignant findings on gastrointestinal endoscopy and computedthomography. Endoscopic retrograde cholangiopancreatography revealed no tumor in themain pancreatic duct or the common bile duct or ampulla <strong>of</strong> Vater. Pylorus-preservingpanctreaticoduodenectomy was performed with a diagnosis <strong>of</strong> duodenal stenosis <strong>of</strong> unknowncause. The histopathological findings revealed that a moderately to poorly differentiatedadenocarcinoma originating near the peribiliary gland in the ampulla <strong>of</strong> Vater was extensivelydistributed in the submucosal layer <strong>of</strong> the duodenum. Based on these findings, a diagnosis <strong>of</strong>a carcinoma <strong>of</strong> the ampulla <strong>of</strong> Vater arising from the peribiliary gland was most likely [622].A 45-year-old man: pointed out von Recklinghausen disease at 18 years <strong>of</strong> age. He had acheckup in a close inspection purpose <strong>of</strong> a duodenum tumor. It was diagnosed an accessorypapilla carcinoid, and pancreas divisum was doubted. Local resection <strong>of</strong> the accessorypapilla was performed and picked out a carcinoid <strong>of</strong> 7 mm size [623].