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.     

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.     

Proposed indications for limited resection of early ampulla of Vater carcinoma: clinico-histopathological criteria to confirm cure

Background

Limited resection is reserved for patients with high operative risk or benign adenomas. We aimed to define indications for limited resection of early ampulla of Vater carcinoma with curative intent through detailed preoperative examinations and histopathological evaluations.

Methods

We performed a retrospective cohort study of all consecutive Japanese patients who underwent resection for ampulla of Vater neoplasms at our hospital from 1986 to 2010.

Results

A total of 75 patients were identified. Moderately/poorly differentiated histology, lympho-vascular/perineural invasion, and duodenal/pancreatic invasion were significant risk factors for lymph node metastases. Macroscopically, non-exposed protruded- or ulcerative-type disease did not correlate directly with lymph node metastases; however, these tumor types were associated with other invasive features. In a subset of early carcinomas fulfilling the conditions of exposed protruded adenoma or papillary/well-differentiated adenocarcinoma determined by endoscopic biopsy, negative duodenal invasion determined by endoscopic ultrasonography, no tumor infiltration into the pancreatic duct determined by intraductal ultrasound, and diameter of the pancreatic duct ≤3?mm determined by endoscopic retrograde cholangiopancreatography (N?=?11), the incidence of lymph node metastasis and tumor infiltration into the pancreatic duct was 0%.

Conclusion

Strictly selected patients with early ampulla of Vater carcinomas may benefit from limited resection if the resected specimen is evaluated to confirm all histopathological criteria.     

Surgical technique for complete resection of the extrahepatic portion of the common bile duct and the ampulla of Vater for tumors of the ampulla of Vater

Background

Ampullary tumors have to be completely resected, but substantial morbidity and mortality rates are associated with pancreaticoduodenectomy (PD). Local resection can be the procedure of choice in selected ampullary lesions for high-risk patients.

Methods

Preoperative examination indicated that the ampullary tumor extended into the common bile duct without evidence of pancreatic duct involvement and no definite invasion into either the duodenum or the pancreas. We performed a complete resection of the extrahepatic bile duct and the ampulla of Vater, including the tumor, without performing PD by dissecting the intrapancreatic bile duct from the pancreas both downward towards the ampulla of Vater and upward using a transduodenal approach.

Results

The operation was successfully completed, and the postoperative course was uneventful, with the exception of a minor pancreatic fistula from retropancreatic dissection. The final pathological examination demonstrated well-differentiated tubular adenocarcinoma limited to the mucosa with negative surgical margins.

Conclusion

Complete resection of the extrahepatic bile duct and the ampulla of Vater through a transduodenal approach can be a feasible and safe surgical procedure for selected ampullary tumors in high-risk patients.     

Multiple primary intraductal tumors of Wirsung's duct: demonstration of a relation between benign and malignant tumors

A case of multiple villous and, more rarely, tubulovillous adenomas of the main pancreatic duct, associated with a diffuse infiltrating adenocarcinoma of the pancreas in which evidence for a link between these lesions is supported by histopathologic features, is reported in a 53 year old patient. Clinical presentation included abdominal pain, weight loss and chronic diarrhea with steatorrhea related to pancreatic insufficiency. Retrograde endoscopic pancreatography showed a complete stenosis of the main pancreatic duct located 3 mm beyond the ampulla of Vater. CT scan showed an heterogeneous cephalic pancreatic tumor with extensive enlargement of the main pancreatic duct. After total pancreatectomy, recovery was maintained (follow-up 18 months). By analogy to colorectal tumors, a new pathologic classification of these rare neoplasms may be proposed.     

内镜下十二指肠乳头切除术在乳头部肿瘤诊断中的价值

目的:评价内镜下十二指肠乳头切除术(endos-copic papillectomy,EP)在乳头部肿瘤诊断中的临床价值.方法:分析2008-01/2012-03来我院的16例经病理检查确诊的十二指肠乳头部肿瘤患者的相关资料,评价EP在十二指肠乳头部肿瘤诊断中的临床价值.结果:16例患者中,内镜活检诊断为腺瘤者12例,慢性炎症改变者4例.EP将乳头部肿瘤组织切除后整体送检行病理学检查确诊为低分化腺癌者2例,高分化腺癌1例,腺瘤13例(其中1例腺瘤恶变,4例常规内镜活检诊断为慢性炎症改变者术后均确诊为腺瘤),EP术后准确率明显高于内镜活检法(P<0.05).超声内镜(endoscopic ultrasonography,EUS)检查提示病变均起源于黏膜层,胰胆管扩张者9例,未见黏膜下层连续性中断及胰胆管受累者,无腹膜后淋巴结肿大者;12例高回声病变者术后病理检查结果均为腺瘤,1例高回声病变局部见低回声区者术后病理检查结果为腺瘤恶变,3例低回声病变者术后病理检查结果均为腺癌.EP术后2例低分化腺癌者、1例腺瘤恶变者及1例腺瘤者胆管开口处见病变残留,提示胆管受累,但术前EUS检查均未提示胆管受累;1例高分化腺癌及11例腺瘤者病灶均完整切除,术后切缘阴性.EP术后除2例出现黑便外,无急性胰腺炎、穿孔及其他并发症发生,无患者死亡.结论:EP术将乳头部肿瘤组织切除后整体送检行病理学检查可提高诊断准确率,为治疗方法的抉择提供科学依据.     

Primary acinar cell carcinoma of the ampulla of Vater

Acinar cell carcinoma of the pancreatobiliary system is a relatively rare malignant neoplasm arising usually in the pancreatic parenchyma. We experienced a 68-year-old woman who presented with obstructive jaundice due to an ampullary mass 1.0 cm in diameter, detected by abdominal computed tomography and endoscopic examination. The patient underwent a curative surgical operation, and histopathological examination revealed that the tumor was confined to the ampulla of Vater with no continuity to the pancreatic parenchyma. The tumor cells showed acinar or tubular arrangement with eosinophilic to basophilic granular cytoplasm, findings identical to those of acinar cell carcinoma of the pancreas. Immunohistochemically, the tumor cells were positive for lipase. From these findings, we concluded that the tumor was primary acinar cell carcinoma arising in the ampulla of Vater, probably originating from heterotopic pancreatic tissue. This is the first reported case of primary acinar cell carcinoma in the ampulla of Vater.     

Pancreatic tuberculosis with obstructive jaundice—a case report

Isolated pancreatic tuberculosis (TB) is very rare and its treatment somewhat controversial. We report a case of pancreatic TB diagnosed as pancreatic carcinoma. An 82-yr-old man presented with right upper abdominal pain and obstructive jaundice, without fever or weight loss. Ultrasonography, computed tomography, and endoscopic retrograde cholangiopancreatography showed a mass lesion in the pancreatic head, which caused stricturing of the distal common bile duct and pancreatic duct in the head of the gland. As malignancy was suspected, he underwent a Whipple procedure (pancreaticoduodenectomy). Histological examination of the resection specimen disclosed typical features of tuberculosis in the pancreatic head, lymph nodes, and at the ampulla of Vater. The rest of the abdominal cavity was unremarkable. After receiving antimicrobial therapy for tuberculosis for 6 months, he remains well, without jaundice or a recurrent mass visible by ultrasound.     

Case of early ampullary cancer treated by endoscopic papillectomy

We herein report a case of ampullary cancer in a 65‐year‐old man who underwent endoscopic papillectomy. Duodenoscopy revealed an exposed‐type tumor mass at the ampulla of Vater. Histology of the biopsy specimen demonstrated well‐differentiated adenocarcinoma. Endoscopic ultrasonography and intraductal ultrasonography showed a hypoechoic mass limited to the ampulla of Vater (clinical stage, T1). Endoscopic papillectomy was performed after informed consent was obtained. Histological examination of the resected specimen revealed adenocarcinoma limited to the ampulla of Vater (final stage, pT1). Both accurate preoperative T staging and proper histological evaluation of the resected specimen appear to justify endoscopic treatment of early ampullary cancer.     

Small-cell neuroendocrine carcinoma of the ampulla of Vater

We report a patient (an 80-year-old woman) with anemia and fecal occult blood, who had an emergency operation for carcinoma of the cecum (well-differentiated adenocarcinoma without local lymph node metastasis). Postoperative magnetic resonance cholangiopancreatography, cholangiography, and upper gastroduodenal endoscopy showed a tumor of the ampulla of Vater, and pylorus-preserving pancreatoduodenectomy was performed. Histology of the resected tumor was that of small-cell carcinoma, and immunohistochemistry showed positive staining for neuron-specific enolase, chromogranin A, and synaptophysin, confirming the neuroendocrine nature of the tumor. As the histology of the tumor was distinct from cecal carcinoma, and no tumors were found in other organs, the tumor was diagnosed as primary small-cell neuroendocrine carcinoma of the ampulla of Vater. The patient died due to multiple liver metastases of the carcinoma of the ampulla of Vater 7 months after the pancreatoduodenectomy. The clinical and morphological features of this disease have been reported in nine individuals previously.     

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.     

Intraoperative fiberoptic choledochoscopy for malignant biliary tract obstruction

A prospective study to determine the safety and effectiveness of intraoperative fiberoptic choledochoscopy in the management of malignant obstruction of the biliary tree was conducted in 44 patients. There were 12 patients with cancer of the pancreatic head, 9 with adenocarcinoma of the distal common bile duct, 4 with adenocarcinoma of the ampulla of Vater, 8 with cholangiocarcinoma of the common hepatic duct, 9 with intrahepatic bile duct carcinoma, and 2 with hepatocellular carcinoma. Five patients with intrahepatic bile duct carcinoma and two with hepatocellular carcinoma were found during a search for intrahepatic duct stones in patients with recurrent cholangitis. In nine patients with bile duct carcinoma (seven extrahepatic and two intrahepatic), histopathological diagnosis was made at operative endoscopy. Treatment strategy was altered in seven patients based on findings at operative choledochoscopy. Complications were few with no mortality. Intraoperative fiberoptic choledochoscopy was safe and useful in the management of malignant obstruction of the biliary tree.     

Mucinous carcinoma of Vater's ampulla with a unique extension along the main pancreatic duct

We report a case of mucinous carcinoma of Vater's ampulla with a unique extension along only the main pancreatic duct (MPD) and microinvasion to the pancreas. A 52‐year‐old man was referred to our hospital for the evaluation and treatment of acute pancreatitis. Abdominal computed tomography (CT) demonstrated swelling in the head of the pancreas with a mass in the duodenum. Hypotonic duodenography and endoscopic examination revealed a well‐defined mass, measuring about 25 mm in size, in Vater's ampulla. A biopsy specimen of the tumor showed moderately differentiated adenocarcinoma. A pylorus‐preserving pancreaticoduodenectomy with a regional lymphadenectomy was performed, under a preoperative diagnosis of adenocarcinoma of Vater's ampulla with direct invasion into the head of the pancreas. The resected specimen of the duodenum confirmed the presence of the mass, which measured 22 × 15 mm in size, in Vater's ampulla. Microscopically, the tumor consisted of two components: moderately differentiated adenocarcinoma in the peripheral region of the tumor Vater's papilla and mucinous carcinoma in the central region of the tumor. The mucinous carcinoma component uniquely extended along only the MPD with microinvasion to the pancreas. Immunohistochemically, both the moderately differentiated adenocarcinoma and the mucinous carcinoma were positive for cytokeratin 20 (CK20) and negative for cytokeratin 7 (CK7) which is the pattern of intestinal‐type carcinoma of Vater's ampulla. We concluded that the original site of this tumor may have been the duodenal epithelium of Vater's ampulla originally moderately differentiated adenocarcinoma—which subsequently changed to mucinous carcinoma that extended along only the MPD with microinvasion to the pancreas.     

Duodenal duplication cyst of the ampulla of Vater

A 35-year-old man presented with the complaint of epigastric discomfort. Gastrointestinal endoscopy and endoscopic ultrasonography revealed a cystic lesion 20 mm in size at the ampulla of Vater. Endoscopic retrograde cholangiopancreatography (ERCP) revealed that the cystic lesion communicated with both the common bile duct and pancreatic duct via the common channel. Choledochocele was ruled out by close examination of the ERCP findings. The cystic lesion was surgically resected. Since histological findings revealed that the mucosa inside the lesion was duodenum-like and contained a layer of smooth muscle, the lesion was diagnosed as a congenital duplication cyst of the duodenum.     

Gallstones in the pancreatic duct: endoscopic retrograde pancreatographic demonstration

A gallstone blocking the ampulla of Vater or the common bile duct is the usual cause of gallstone pancreatitis. A gallstone in the pancreatic duct itself, leading to pancreatitis, has rarely been documented. We describe such a case and show what seems to have been the obstructing stone by endoscopic retrograde cholangiopancreatography (ERCP).     

Signet-ring-cell carcinoma of the ampulla of Vater: a case report

We encountered a rare variant of carcinoma of the ampulla of Vater in a 68-year-old man with postprandial abdominal pain and nausea. Ultrasonography and computed tomography showed a dilated common bile duct and main pancreatic duct. At duodenoscopy, the papilla of Vater was enlarged, and biopsy specimens taken from the papilla revealed signet-ring-cell carcinoma. Endoscopic ultrasonography showed a hypoechoic tumor without pancreatic invasion. Pylorus-preserving pancreatoduodenectomy was performed. Histologic examination of resected specimens indicated lymphatic and vascular invasion, but surgical margins were tumor-free. The patient is alive and disease-free 10 months after the operation.     

A case of primary duodenal cancer near the papilla of Vater which had initial symptoms of obstructive jaundice]

A 63-year-old man visited our hospital complaining of brown urine. A physical examination showed jaundice of the skin and conjunctiva bulbar. Blood tests showed elevated serum levels of bilirubin and hepatobiliary enzymes. A type 2-like mass lesion was found near the papilla of Vater during the endoscopic retrograde cholangiopancreatography and was histologically proven to be a well-differentiated adenocarcinoma. A diagnosis of obstructive jaundice due to primary duodenal cancer arising near the papilla of Vater was made. After the jaundice was decreased by endoscopic biliary stenting, a pancreatoduodenectomy was performed. A histopathological examination of the resected specimen concerning the location and manner of invasion of cancer cells revealed that the cancer arose from the duodenal mucosa near the papilla of Vater.     

Papillary adenoma of the pancreas with excessive mucin secretion.

A 68-year-old woman with papillary adenoma of the pancreas with excessive mucin secretion is reported. The patient was initially diagnosed as having chronic pancreatitis because of a history of repeated attacks of pancreatitis and localized dilatation of the main pancreatic duct. Four years later, endoscopic retrograde pancreatography showed markedly diffuse dilatation of the entire main pancreatic duct with amorphous filling defects of mucin. Excretion of mucin was observed through the enlarged orifice of Vater's ampulla. The patient was treated with distal pancreatectomy, and papillary adenoma with abundant mucin in the cytoplasm was histologically demonstrated. We describe unique clinical features of "mucin-producing pancreatic tumor" and discuss an important role of endoscopic retrograde pancreatography in the diagnosis.     

Non-pancreatic periampullary adenocarcinomas: an explanation for favorable prognosis

BACKGROUND/AIMS: Our previous studies demonstrate that patients with non-pancreatic periampullary adenocarcinomas have a favorable prognosis relative to those with pancreatic adenocarcinoma. This study investigates histopathologic factors that contribute to the superior outcome of these patients. METHODOLOGY: A retrospective review of all patients explored for periampullary neoplasms at a single institution over a 20-year period. RESULTS: 291 patients with periampullary neoplasms underwent exploratory laparotomy, of which 185 had resectable tumors. Periampullary adenocarcinomas were resected in 120: pancreatic head (n=74), distal common bile duct (n=10), duodenum (n=5), and ampulla of Vater (n=31). The resection rate for non-pancreatic adenocarcinomas was 90%, while that of pancreatic cancers was 44% (p<0.01). Median survival for resected non-pancreatic adenocarcinomas was 38.8 months; that of pancreatic tumors was 15.3 months (p<0.01). Non-pancreatic adenocarcinomas were significantly smaller (p<0.001), better differentiated (p<0.001), and less likely to have involved nodes (p<0.001), margins (p<0.001), perineural invasion (p<0.001), or vascular invasion (p<0.2) than pancreatic adenocarcinomas. CONCLUSIONS: Histopathologic features of non-pancreatic periampullary adenocarcinomas significantly differentiate them from pancreatic adenocarcinoma and contribute to their relatively favorable long-term outcome following resection.     

A case of intraductal papillary mucinous neoplasm of the pancreas arising from pancreas divisum without ventral pancreatic duct of Wirsung]

Most reported cases of intraductal papillary mucinous neoplasms (IPMNs) originate from Wirsung's duct or their branches. IPMNs arising from Santorini's duct and its branches have rarely been reported. Eight cases of IPMN arising from Santorini's duct have been published worldwide. However, these cases are associated with incomplete type of pancreas divisum. Recently, one report of IPMN with complete absence of Wirsung's duct has been reported. This patient was a 57-year-old woman who was admitted to the hospital due to progressive jaundice. On endoscopic retrograde cholangiopancreatography, there was a severely bulging ampulla of Vater and patulous minor papilla draining mucinous material and a cystic lesion communicating with the dilated Santorini's duct without any communication with Wirsung's duct. A pancreaticoduodenectomy was performed and the pathologic examination of resected specimen showed no evidence of Wirsung's duct, but an IPMN arising from Santorini's duct with peripancreatic lymph node metastasis. Herein, we report a case of invasive IPMN arising from pancreatic head without ventral pancreatic duct with a review of the relevant literatures.