Undifferentiated carcinoma of the duodenal ampulla

This report demonstrates a case of undifferentiated carcinoma of the duodenal ampulla. A 74-year male experienced jaundice lasting for 3 weeks. An upper gastrointestinal series demonstrated a polypoid, ovoid filling defect in the second portion of the duodenum, and duodenoscopy disclosed a protruding mass involving the orifice of the papilla of Vater. Cholangiography demonstrated obstruction due to compression in the terminal common bile duct. Pylorus-preserving pancreatoduodenectomy was performed on the diagnosis of ampullary carcinoma. The gross specimen showed a polypoid mass, measuring 3.5 cm in diameter, in the ampulla, located mainly in the duodenal submucosal layer and invading the terminal common bile duct. Histologically, the tumor was small cell type, undifferentiated carcinoma, arising from the duodenal epithelium adjacent to the ampulla.     

Local resection of a gangliocytic paraganglioma near the papilla of Vater

A 69-year-old man was admitted to our hospital with epigastric discomfort. Upper gastrointestinal endoscopy showed a submucosal tumor near the papilla of Vater. Abdominal CT and MRI showed a small, well-enhanced tumor. Endoscopic tumor biopsy was performed before the operation, but pathologic findings showed normal duodenal musosa. Nevertheless, since malignancy could not be ruled out, we resected the tumor with the sphincter of the papilla of Vater, followed by plasty of the orifice for the common bile duct and main pancreatic duct. We identified 3 parts with tumor cells; epithelioid cells, spindle cells, and ganglion-like cells. The tumor was diagnosed as gangliocytic paraganglioma of the duodenum. Treatment by resecting the tumor with the sphincter of the papilla of Vater, followed by the plasty of the orifice for the common bile duct and main pancreatic duct, was selected considering the patient's safety and to achieve radical cure.     

An inflammatory myofibroblastic tumor of the ampulla of vater successfully managed with endoscopic papillectomy: report of a case

Inflammatory myofibroblastic tumors (IMTs) are solid neoplastic mesenchymal proliferations composed of myofibroblastic spindle cells admixed with inflammatory infiltrates. The documented sites in the gastrointestinal tract include the esophagus, small intestine, colon, appendix, rectum, pancreas, spleen, liver, and Meckel's diverticulum. Biliary IMTs are rare, and IMTs arising from the ampulla of Vater have not been reported previously. Herein we report the case of a 65-year-old woman with an extrahepatic biliary obstruction due to IMT of the ampulla of Vater, and a successful therapeutic approach using endoscopic ultrasonography and endoscopic papillectomy.     

Sarcoidosis causing duodenal obstruction

Conclusions We present an unusual case of sarcoidosis in which the clinical presentation is best explained by an inflammatory mass in the region of the ampulla of Vater causing obstruction of the bile duct and pancreatic duct as well as intermittent duodenal obstruction. It is clear from previous reports that gastrointestinal exacerbations of sarcoidosis may occur during quiescent pulmonary disease. We are confident of the diagnosis because (1) the patient had a well-documented history of systemic sarcoidosis; (2) the obstruction responded to high-dose steroids promptly on two separate occasions; (3) biopsy of peripancreatic lymph nodes in the inflammatory mass was consistent with sarcoidosis; and (4) there was no clinical or radiographic evidence for Crohn's disease or any other granulomatous disorder. Our report indicates that small bowel obstruction must be added to the list of rare intra-abdominal complications of sarcoidosis.     

Hemobilia due to biliary intraepithelial neoplasia associated with Zollinger–Ellison syndrome

A 58-year-old man was transferred to us from his local hospital because of failure to control his gastrointestinal bleeding by endoscopic hemostasis. Abdominal imaging suggested a hypervascular tumor of the pancreatic head (36?mm diameter), and laboratory testing showed an elevated serum gastrin level (17,800?pg/mL). Gastroduodenal endoscopy revealed multiple duodenal ulcers and active bleeding from the ampulla of Vater. The selective arterial secretagogue injection test suggested a gastrinoma in the pancreatic head, but no gastrinoma in the pancreatic tail. The patient was diagnosed with solitary pancreatic head gastrinoma complicated by hemosuccus pancreaticus, and pancreaticoduodenectomy was performed. Intraoperatively, the diagnosis was changed to primary peripancreatic lymph node gastrinoma without pancreatic involvement. The gastrointestinal bleeding stopped postoperatively and serum gastrin levels returned to normal. Histological examination of the surgical specimens revealed a small submucosal gastrinoma in the duodenum (7?mm diameter). The final diagnosis was microgastrinoma of the duodenum with peripancreatic lymph node metastasis. The cause of bleeding from the ampulla of Vater was initially obscure, but eventually a hemorrhagic erosion with moderate atypia was found in the common bile duct, indicating biliary intraepithelial neoplasia (BilIN). This is the first report of hemobilia due to BilIN with gastrinoma.     

Periampullar gangliocytic paraganglioma resected by surgical ampullectomy: a case report

We report the case of a 40-Year-old woman with duodenal gangliocytic paraganglioma of the ampulla of Vater. Preoperative diagnosis was periampullar tumor. Final diagnosis of duodenal gangliocytic paragangioma of the ampulla of Vater with negative margins was made by histological and immunohistochemical study of surgical ampullectomy specimen. This case report stresses the yield of immunohostochemical study in the diagnosis of duodenal gangliocytic paraganglioma, a rare entity.     

Duodenal Crohn''s Disease Complicated by Common Bile Duct Obstruction: Report of a Case and Review of the Literature

A 28-year-old woman with Crohn's disease involving the duodenum and terminal ileum presented with biochemical evidence of incomplete, extrahepatic biliary obstruction. Percutaneous transhepatic cholangiography showed partial obstruction of the intramural duodenal portion of the common bile duct. Cholangiography was complicated by cholangitis. Surgical decompression of the biliary system and bypass of the affected duodenal segment were performed after intraoperative confirmation of Crohn's disease involving the ileum, duodenum, and ampulla of Vater. The patient made a complete recovery and all previously abnormal cholestatic liver function test results returned to normal.     

Primary duodenal diffuse large cell non-hodgkin lymphoma with involvement of ampulla of Vater: Report of 3 cases

Primary gastrointestinal system lymphomas constitute about one third of all extranodal lymphomas. Duodenal involvement of the lymphoma is a rare condition. Periampullary lymphoma or lymphomatous involvement of ampulla of Vater is even rarer. Since, periampullary lymphoma is not easy to differentiate from epithelial carcinoma of these sites clinically and radiologically, accurate histopathological diagnosis is essential to plan optimal treatment strategy. Obstructive jaundice and the need for some form of drainage procedure and dose modification of the chemotherapy are additional challenges. In this report, we present three cases of duodenal diffuse large cell lymphoma with involvement of ampulla of Vater, two of whom presented with the initial signs of obstructive jaundice. One of the icteric patients was only diagnosed histopathologically following an explorative laparotomy with the initial diagnosis of carcinoma.     

Primary duodenal diffuse large cell non-hodgkin lymphoma with involvement of ampulla of Vater: report of 3 cases

Primary gastrointestinal system lymphomas constitute about one third of all extranodal lymphomas. Duodenal involvement of the lymphoma is a rare condition. Periampullary lymphoma or lymphomatous involvement of ampulla of Vater is even rarer. Since, periampullary lymphoma is not easy to differentiate from epithelial carcinoma of these sites clinically and radiologically, accurate histopathological diagnosis is essential to plan optimal treatment strategy. Obstructive jaundice and the need for some form of drainage procedure and dose modification of the chemotherapy are additional challenges. In this report, we present three cases of duodenal diffuse large cell lymphoma with involvement of ampulla of Vater, two of whom presented with the initial signs of obstructive jaundice. One of the icteric patients was only diagnosed histopathologically following an explorative laparotomy with the initial diagnosis of carcinoma.     

A case report: endoscopic enucleation of gastrointestinal stromal tumor of the ampulla of Vater

Objective

To report a case of endoscopic enucleation of a gastrointestinal stromal tumor of the ampulla of Vater.

Design

The tumor resection was performed by needle knife endoscopic submucosal dissection.

Results

The tumor was resected completely without serious complications and the patient recovered rapidly.

Conclusion

Endoscopic enucleation may be an alternative treatment for gastrointestinal stromal tumors of the ampulla of Vater for patients who fulfill certain requirements.     

Expression of hedgehog proteins in periampullary cancer]

BACKGROUND/AIMS: Hedgehog protein is an essential molecule for gastrointestinal tract development, and disruption of hedgehog signaling pathway is linked to some gastrointestinal tumorigenesis. Here, we performed hedgehog immunostaining in periampullary cancer to evaluate the differences according to the location type of cancer and the differentiation of adenocarcinoma. METHODS: We retrieved surgical specimens from 43 periampullary cancer patients (15 ampulla of Vater cancer, 12 distal common bile duct cancer, 13 pancreatic head cancer, and 3 combined ampulla of Vater/bile duct cancer). Immunohistochemical stain was performed in both normal and cancerous tissue portions of each case using Sonic hedgehog (H-160) rabbit polyclonal antibody. Immunohistochemical stain results were grouped into three groups according to the percentage of positive cytoplasmic stain in tumor volume (unstained: <5%, weakly stained: 5-50%, and strongly stained: >50%). RESULTS: All of the normal tissue revealed negative immunohistochemical stain while cancerous tissue revealed positivity in 95.3% (41/43 cases). Strongly stained cases were more frequently seen in ampulla of Vater cancers (13/15) and in combined ampulla of Vater/bile duct cancers (3/3) than in distal common bile duct cancers (4/12) and in pancreatic head cancers (3/13) (p=0.002). In addition, strongly stained cases were more frequently seen in well-differentiated adenocarcinoma than the others (p<0.001). CONCLUSIONS: Most of the periampullary cancers show hedgehog protein expression. In addition, hedgehog protein immunostainings shows stronger expression in ampulla of Vater cancers and in well-differentiated adenocarcinoma.     

Upper digestive hemorrhage caused by ulcerated periampullary leiomyoma

Duodenal leiomyoma represents a very unusual cause for acute gastrointestinal bleeding. The authors report the case of a 49-year-old male with a massive bleeding from an ulcerated duodenal leiomyoma involving the ampulla of Vater. An emergency Whipple's pancreaticoduodenectomy was carried out under suspicion of local malignancy. It is emphasized that the proper diagnosis of smooth muscle tumors is often difficult, both clinically and from the pathological point of view.     

Duplication cyst of the duodenum in the adult

A duodenal duplication cyst in a 45-year-old male is reported. A hypotonic duodenography showed a smooth spherical defect in the descending portion of the duodenum. A fiberoptic duodenoscopy disclosed a smooth well-defined tumor which was located orally from the ampulla of Vater. A retrograde pancreatocholangiography indicated the tumor was not in communication with the pancreatic duct or biliary tract. At operation, a cystic spherical mass, 3 cm in diameter, was located in the posterior wall of the duodenum corresponding to the above described diagnosis. The combination of a greater awareness of this condition as well as improved X-ray and endoscopic techniques has made preoperative diagnosis more accurate.     

A case of duodenal carcinoid tumor accompanied by liver metastasis and lymph node metastases in a patient with von Recklinghausen disease

We report a case of duodenal carcinoid tumor accompanied by liver metastasis and lymph node metastases in a patient with von Recklinghausen disease. A 48-year-old woman with von Recklinghausen disease was referred to our hospital because of a submucosal tumor at the ampulla of Vater detected by upper gastrointestinal endoscopy. The lesion was diagnosed as a carcinoid tumor based on the pathology of the biopsy specimen. At operation, although there were liver metastasis on the surface of S3 the liver and regional lymph node metastases, we selected pancreatoduodenectomy with lymph node dissection and enucleation of the liver metastasis. The postoperative course was good and the woman was discharged on postoperative day 33. No recurrence has been seen at 24 months since surgery.     

Somatostatinoma of the papilla of Vater with multiple gastrointestinal stromal tumors in a patient with von Recklinghausen's disease

We report a case of somatostatinoma of the papilla of Vater with multiple gastrointestinal stromal tumors in a patient with von Recklinghausen's disease (VRD). A 64-year-old woman who had had recurrent attacks of acute pancreatitis and cholangitis was found, on gastroduodenal endoscopy, to have a tumor of the papilla of Vater and multiple submucosal tumors of the stomach and duodenum. Numerous submucosal tumors were observed in the stomach, duodenum, and jejunum, and total excision of the papilla of Vater and resection of the duodenal and jejunal submucosal tumors was performed. The tumor of the papilla of Vater showed the histologic appearance of a dense proliferation of tumor cells in acinar form, from the duodenal mucosa to the muscle layer, and psammoma bodies were revealed within the tumor. Immunohistologically, the tumor cells were intensely positive for somatostatin. The submucosal tumors of the duodenum and jejunum were negative for smooth muscle actin, s-100, and neuron-specific enolase (NSE), and positive for CD34 and c-kit, and they were diagnosed as gastrointestinal stromal tumors (GISTs) according to the strict definition. The only 25 cases of papilla of Vater somatostatinoma associated with VRD to have been reported in the English-language literature since 1982 are reviewed, as well as our own case. Received: January 22, 2001 / Accepted: August 10, 2001 Reprint requests to: M. Usui     

Paraganglioma of the ampulla of Vater: a potentially malignant neoplasm

Paragangliomas are rare tumours originating from neuroectodermic remnants and are usually considered as benign. We present two cases of paraganglioma of the ampulla of Vater that were treated surgically by pancreaticoduodenectomy. In one case, histopathology revealed malignant characteristics of the tumour with invasion of the pancreas and simultaneous duodenal lymph-node metastases. Both patients had a favourable outcome without disease recurrence at 40 and 44 months postoperatively. Only 21 cases of ampullary paraganglioma have been reported in the literature, 7 of them with malignant characteristics. In conclusion, paragangliomas of the ampulla of Vater have malignant potential. Surgical therapy of these tumours should not be limited to local resection, as disease recurrence and lymph node involvement have been reported. We propose that paragangliomas of the ampulla of Vater should be operated by cephalic pancreaticoduodenectomy, which allows long-term and disease-free survival.     

Carcinoid tumour of the ampulla of Vater presenting as acute pancreatitis.

The first report of a carcinoid tumour of the ampulla of Vater causing acute pancreatitis is presented. The pancreatitis resulted from ampullary obstruction and diversion of the bile through the pancreas. Endoscopic retrograde cholangiopancreatography established the correct diagnosis, making this the first occasion in which a carcinoid tumour of the ampulla of Vater has been correctly diagnosed before surgery.     

Duodenal and ampullary obstruction by a Peutz-Jeghers polyp

We report a case of Peutz-Jeghers syndrome presenting with obstruction of the second part of the duodenum and the ampulla of Vater by a large intra-luminal polyp leading to duodenal obstruction and obstructive jaundice. CT scan of the abdomen showed a large polypoidal lesion, a caecal polyp and jejuno-jejunal intussusception. At surgery, two intussusceptions were reduced and leading polyps were excised via two enterotomies; the caecal polyp was excised via caecotomy. The duodenal polyp was excised by limited duodenectomy after frozen section has shown no evidence of malignancy. Histopathological study of all the excised polyps including that of the duodenum showed hamartomatous polyps with no malignant changes. Apart from acute bleeding, this case highlights many of the surgical gastrointestinal complications of Peutz-Jeghers syndrome. It also highlights the unusual combined duodenal and common bile duct obstruction by a large Peutz-Jeghers polyp. The controversial association of this syndrome with cancer and management options is also discussed.     

A case of ampullary cancer with pancreas divisum treated by endoscopic papillectomy

A 60's man underwent a medical check-up and esophagogastroduodenoscopy revealed an exposed-type tumor at the ampulla of Vater. Endoscopic ultrasonography and intraductal ultrasonography showed a hypoechoic mass limited to the ampulla of Vater. Endoscopic retrograde cholangiopancreatography (ERCP) revealed a slightly dilated ventral pancreatic duct not connected to the dorsal duct. Endoscopic papillectomy was performed without pancreatic stent placement and his postprocedural course was uneventful. The specimen was histologically diagnosed as well-differentiated adenocarcinoma limited to the mucosa of the ampulla of Vater. Endoscopic papillectomy without pancreatic stent placement can be performed without a risk of post-ERCP pancreatitis for ampullary tumor limited to the mucosa of the ampulla of Vater associated with pancreas divisum.     

胰十二指肠动脉弓移位对胰头-壶腹区肿瘤的鉴别诊断

目的分析胰头-壶腹区肿瘤与胰十二指肠动脉弓的关系,提高对该区肿瘤组织来源的定性诊断水平。 方法收集2009年1月至2018年1月在中山大学附属第一医院术前行64排螺旋CT检查并经病理证实的胰头-壶腹区36例肿瘤,包括胰头癌10例,胰头神经内分泌肿瘤8例,胰头实性假乳头状瘤2例,胰头淋巴瘤1例,十二指肠乳头癌8例,十二指肠淋巴瘤1例,十二指肠间质瘤1例和胆总管癌5例。回顾分析其不同组织来源的肿瘤与胰十二指肠动脉弓的关系。 结果胰头来源肿瘤使胰十二指肠动脉弓向右移位,恶性肿瘤对血管有包埋;胆总管来源肿瘤使胰十二指肠动脉弓向右移位,但肿瘤对血管无有包埋;十二指肠壶腹部来源肿瘤使胰十二指肠动脉弓向左右移位,恶性肿瘤对血管无有包埋。 结论胰十二指肠动脉弓的移位和包埋征象在胰头-壶腹区肿瘤的CT鉴别诊断中有重要价值。