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.     

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.     

Modes of spread in early ampullary cancer in terms of establishing proper indications for endoscopic papillectomy

Background: Endoscopic papillectomy for adenomas of the ampulla of Vater has been reported and is gaining acceptance as an alternative to surgery in the treatment of early ampullary cancer. However, whether endoscopic treatment is justified as a treatment of choice for early ampullary cancer remains controversial. The aim of the present study was to elucidate the possibility of endoscopic papillectomy as a treatment of early ampullary cancer from the review of pathology of cases treated by surgical resection. Patients and methods: Twenty‐three cases of early ampullary cancer (m—tumor limited to the mucosa of the ampulla 14; od—tumor that invades Oddi's sphincter, 9) treated by surgical resection from January 1984 to March 2003 were investigated as to the following: (i) macroscopic type, maximum size, and histological type of tumor; (ii) main location and extension of tumor; (iii) prevalence of extension into the lower bile duct or pancreatic duct, and relationship between ductal infiltration and macroscopic type, maximum size, main location, or depth of invasion of tumor; (iv) lymphatic permeation, vascular invasion, and lymph node metastasis; and (v) prognosis. Results: All cases were classified macroscopically as exposed‐tumor type or non‐exposed‐tumor type without ulceration. Extension into the lower bile duct or the pancreatic duct was observed in 43% of the cases. There was no correlation between ductal infiltration and macroscopic type, maximum tumor size, main tumor location, or tumor depth. No lymphatic permeation, vascular invasion, or lymph node metastasis were proven in cases with ampullary cancer confined to the mucosa. In the nine cases with involvement of Oddi's sphincter, lymphatic permeation and lymph node metastasis were observed in two cases and one case, respectively. Conclusion: Endoscopic treatment for early ampullary cancer confined to the mucosa without spread to the bile duct or pancreatic duct is justified as a treatment of choice if detailed histological examination of the resected specimen indicated no invasion beyond its margin.     

Endoscopic papillectomy of minor papillar adenoma associated with pancreas divisum

Tumors of the minor papilla of the duodenum are quite rare. We successfully and safely treated an 18-mm adenoma of the minor papilla associated with pancreas divisum using endoscopic papillectomy. A 64-year-old man was admitted to our hospital for treatment of an asymptomatic mass in the minor papilla detected by upper gastrointestinal endoscopy. Endscopic analysis showed an 18-mm, whitish, sessile mass, located in the duodenum proximal to a normal-appearing major papilla. Endoscopic retrograde pancreatography did not reveal the pancreatic duct. Magnetic resonance cholangiopancreatography showed a lack of the ventral pancreatic duct. We suspected this case was associated with pancreatic divisum; therefore, we performed endoscopic papillectomy of the minor papilla tumor. Subsequently, endoscopic pancreatic stent placement in the minor papilla was done to prevent drainage disturbance. The patient has been asymptomatic without recurrence of tumor or stenosis of the Santorini orifice upon endoscopic examination for the past 2 years.     

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.     

Endoscopic Resection of Tumor of Papilla Vater: Our Experiences

Endoscopic papillectomy has become common in recent years as a non‐invasive therapeutic procedure for tumor of the papilla of Vater, although the lesions, which indicate endoscopic papillectomy, are very rare. In order to decide the indication for this procedure, intraductal ultrasonography (IDUS) is useful to obtain the correct diagnosis. For the successful and safe procedure of papillectomy, it is important to use the cutting mode of generator. and it is sometimes useful to inject the saline into the submucosal layer of the duodenum and to place the plastic stent into the bilio‐pancreatic ducts.     

"Duodenal intussusception" due to adenoma of the papilla of Vater

The case of a 55-year-old woman with a pedunculate adenoma of the papilla of Vater is presented. Diagnostic imaging modalities including ultrasonography, CT scan, magnetic resonance of cholangiopancreatography, simultaneous duodenography and cholangiography, and angiography showed a giant tumor protruding intraluminally and moving forward in the duodenum by peristalsis. It had a duodenal intussusception-like appearance, with remarkable left-lower deviation of the common bile duct and major pancreatic duct in the papilla of Vater as far as the left side of the aorta. Episodes of jaundice or ileus were absent, probably because the tumor was mobile in the duodenum. As biopsy specimens showed no malignancy and intraductal ultrasonography in the common bile duct revealed no intraductal invasion of the tumor in the papilla of Vater, the patient underwent transduodenal papillectomy with papilloplasty with pancreatic ductoplasty. Pathological diagnosis of consecutive specimens was a papillary adenoma with moderate atypia and occasional tubular structure. There seems to be an exceptional subtype of the tumor in the papilla of Vater, like this case, demonstrating the duodenal intussusception-like appearance without prominent clinical symptoms.     

Current Status of Endoscopic Papillectomy for Ampullary Tumors

Detection of tumors of the ampulla of Vater, including ampullary adenoma, has been improved by routine screening endoscopic procedures and imaging modalities. Endoscopic resection by endoscopic papillectomy is rapidly replacing classic surgical resection and is a less invasive procedure. Endoscopic resection can have a role not only in the final histopathologic diagnosis but also as a definite therapeutic option. However, the indications for endoscopic resection are not fully established, and endoscopic procedures are not standardized. Significant complications, including severe pancreatitis, intractable bleeding and duodenal perforation, are rare but can occur, especially in less experienced hands. Severe pancreatitis is the most feared complication, but it can be prevented by pancreatic duct stent insertion in most cases. However, in some cases, pancreatic stenting can be challenging after resection. Incomplete resections are sometimes performed to avoid complications. Endoscopic surveillance is also important for identifying and managing remnant adenomatous tissue or recurrent lesions. Further technical development is needed to expand the indications for this procedure, minimize complications and ensure a high success rate.     

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.     

Wire-guided endoscopic snare papillectomy for tumors of the major duodenal papilla

BACKGROUND: Endoscopic excision for adenoma of the major duodenal papilla was introduced as an alternative to surgery, but postprocedure pancreatitis is a serious drawback. This study assessed the feasibility and the safety of endoscopic papillectomy with a guidewire and pancreatic-duct stent insertion to prevent pancreatitis. METHODS: Six patients were enrolled. The snare loop was passed over a guidewire that had been inserted into the pancreatic duct. Immediately after snare resection, a pancreatic stent was placed along the indwelling guidewire. RESULTS: En bloc papillectomy and pancreatic stent insertion were performed successfully in all patients. Pancreatitis did not develop acutely in any patient. Complications included cholangitis (n = 1) and late-onset pancreatitis owing to the pancreatic stent (n = 1). Scant residual adenomatous tissue was present at resection margins in two patients and was treated endoscopically. CONCLUSIONS: Wire-guided endoscopic snare papillectomy in selected patients is a useful technique that maintains pancreatic-duct access for stent placement. This appears to prevent pancreatitis and to improve the outcome for patients undergoing endoscopic resection of papillary tumors.     

Coexistent Ampullary Squamous Cell Carcinoma with Adenocarcinoma of the Pancreatic Duct

Primary squamous cell carcinoma (SCC) of ampulla has seldom been reported. However, metastatic SCC to ampulla of Vater is well known. We report a case of primary SCC of ampulla of Vater coexistent with well-differentiated adenocarcinoma of the distal pancreatic duct. A 50-year-old female presented with evidence of obstructive jaundice. Endoscopic retrograde cholangio-pancreatography revealed bulging papilla with ulcero-infiltrative growth at the ampulla of Vater. An initial endoscopic biopsy of the ampullary mass showed a well-differentiated SCC. The patient underwent Whipple''s operation. Thorough sampling of the dilated portion of the pancreatic duct showed presence of well-differentiated adenocarcinoma of the distal pancreatic duct. Immunohistochemical study with synaptophysin and chromogranin was done with negative result, ruling out neuroendocrine differentiation. Also, a detailed clinical, endoscopic and radiological examination was carried out, that excluded the presence of primary SCC elsewhere.     

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.     

A CASE OF TUMOR‐FORMING PANCREATITIS WITH A RAPID AND AGGRESSIVE CHANGE

An asymptomatic 51‐year‐old man was found to have a low echoic mass by ultrasonography, 12 mm in diameter, at the head of the pancreas during mass screening. Endoscopic ultrasonography revealed that the mass was hypoechoic with clear margins and without main pancreatic duct dilatation. Therefore, islet cell tumor was suspected, but pancreatic carcinoma could not be excluded. After 3 months, follow‐up ultrasonography revealed that the margin had become more clearly defined and the mass had tended to enlarge. Endoscopic ultrasonography and endoscopic retrograde cholangiopancreatography revealed dilatation of the main pancreatic duct. A pylorus‐preserving pancreaticoduodenectomy was performed for the presumed diagnosis of pancreatic carcinoma. Histological examination revealed prominent fibrosis and destruction of the exocrine parenchyma without evidence of malignancy. The diagnosis of pancreatic carcinoma based on tendency of enlargement and dilatation of the main pancreatic duct was difficult in this case.     

Endoscopic resection of benign tumors of the duodenal papilla without and with intraductal growth

BACKGROUND: Endoscopic papillectomy of benign papillary tumor is still not widely practiced. Intraductal growth has been considered a contraindication for endoscopic therapy. This prospective study evaluates endoscopic papillectomy for treatment of benign papillary tumors without and with intraductal growth. METHODS: Monofilament snare and monopolar electrocoagulation were used for papillectomy. A 7F stent was placed in the pancreatic duct. Patients with distal intraductal growth underwent sphincterotomy and endoscopic resection after exclusion of more proximal growth. RESULTS: Between February 1985 and April 2004, 106 patients (109 lesions), 68 women, 38 men, median age 68 years (range 29-88 years) were included. Median tumor size was 2 cm (range 0.5-6 cm) with one session (range 1-8) required for removal. Nine patients had invasive carcinoma (8%). Surgery for incomplete removal or recurrence was performed in 12% of 75 patients without and 37% of 31 patients with intraductal growth (p < 0.01), respectively. Fifteen patients had recurrence (15%); but, only 4 required surgery. Endoscopic resection was curative (median follow-up, 43 months) in 83% without and 46% with intraductal growth (p < 0.001). CONCLUSIONS: Endoscopic papillectomy is safe and effective, and may be feasible in cases of intraductal growth. Surveillance and, if required, re-treatment are mandatory because of the risk of recurrence.     

Long-term survival with carcinoma arising from a congenital choledochal cyst

We report a case of 11-year survival with carcinoma arising from a congenital choledochal cyst. A 34-year-old Japanese woman was admitted due to epigastric discomfort. Ultrasonography revealed a choledochal cyst involving a polypoid tumor. Endoscopic retrograde cholangiopancreatography revealed pancreaticobiliary maljunction in which the main pancreatic duct joined the dilated common bile duct 2 cm above the papilla of Vater. A choledochal cyst containing a polypoid tumor was resected with the regional lymph nodes. Grossly, a 2×2-cm papillary tumor was noted in the posterior wall of the choledochal cyst. Microscopically, the tumor was well-differentiated tubular adenocarcinoma invading the mucosa and fibromuscular layer. Neither anticancer chemotherapy nor radiotherapy was performed. The patient recovered uneventfully after the operation and has been well for 11 years without evidence of recurrent cancer. The good results in this patient may have been due to the early stage of the cancer.     

ENDOSCOPIC SNARE PAPILLECTOMY FOR TUMORS OF THE MAJOR DUODENAL PAPILLA

Safer procedures for performing endoscopic papillectomy on papillary tumors should be established. Fourteen patients underwent endoscopic papillectomy between January 2000 and February 2007. Before papillectomy, tumors were slightly elevated by submucosal injection of glycerol, followed by semi‐circular mucosal incision of the anal border of the lesion in five cases. In nine cases, balloon‐catheter‐assisted endoscopic snare papillectomy was performed. All patients routinely underwent endoscopic nasobiliary drainage tube placement in the bile duct. A pancreatic stent was inserted only in patients with non‐patent minor papilla. The rate of positive margins was lower in patients who received balloon‐catheter‐assisted papillectomy than in patients who received papillectomy without a balloon catheter. Of three patients with local recurrence, only one patient required surgery. Post‐procedural complications included hemorrhage in eight patients, pancreatitis in one patient, and perforation in one patient. No perforation occurred after submucosal injection and mucosal incision. In three of four patients with early hemorrhage, bleeding was noticed as hemobilia through the nasobiliary drainage tube; this appears to be useful for early diagnosis of bleeding. No pancreatitis occurred in patients with patent minor papilla, suggesting that pancreatic stent placement is unnecessary in patients with patent minor papilla.     

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.     

Intraductal papillary-mucinous tumor of the pancreas head with complete absence of the ventral pancreatic duct of Wirsung

A case of intraductal papillary mucinous tumor of the pancreas with complete absence of the ventral pancreatic duct of Wirsung is presented. A 74-year-old Japanese man was admitted to our hospital because of elevated serum amylase concentration. Abdominal computed tomography (CT) scanning revealed diffuse dilatation of the main pancreatic duct and a diffuse and uncircumscribed area with heterogeneous density in the pancreas head. Endoscopic retrograde cholangiopancreatography revealed that the main pancreatic duct was connected with an accessory papilla and was diffusely dilated, without any irregularity of the duct wall being observed in the entire length of the duct. The common bile duct was detected only by cannulation through Vater’s papilla, and no pancreatic duct or its communicating branch was found. Some branches, directed to the dorsal portion of the pancreas head, were found arising from the accessory pancreatic duct. Intraductal ultrasound examination performed through the accessory papilla and the common bile duct revealed a small tumor with a heterogeneous echo level in the pancreas head. From these findings, intraductal papillary-mucinous tumor (IPMT) occurring in the pancreas head was diagnosed, and pylorus-preserving pancreaticoduodenectomy was performed. The resected specimen revealed IPMT in the pancreas head. A roentgenographic study of the resected specimen revealed a defect caused by the tumor located in the pancreatic duct connected with the accessory papilla and showed that there was complete absence of the pancreatic duct connected with Vater’s papilla. Surgical resection enabled us to completely analyze the duct system of pancreas divisum. Although it is not known whether there is a relationship between the pathogenesis of IPMT and embryological anomaly of the pancreatic duct system, this case may provide an insight into the pathogenesis of IPMT.     

A case of early carcinoma of the papilla of Vater confined to the mucosa and continuative epithelium of glands in Oddi's sphincter (m-God) treated by endoscopic papillectomy

We herein report a case of early stage ampullary cancer, treated by endoscopic papillectomy, in which tumor extension was confined to the mucosa and adjacent epithelium of the glands in Oddi's sphincter. A 77-year-old man underwent screening esophagogastroduodenoscopy, which revealed a mass in the papilla of Vater, which was well-differentiated adenocarcinoma as proven by biopsy. The tumor was diagnosed as T1 and endoscopic papillectomy was performed. Histological examination showed adenocarcinoma limited to the mucosa of the common channel and continuative epithelium of the neighboring glands in Oddi's sphincter. No signs of recurrence have been observed during a follow-up of 23 months.     

A long-term surviving patient with cancer of papilla of Vater after local resection

We report successful local resection for cancer of papilla of Vater in an 86-year-old woman. She was referred to our hospital because of right hypochondralgia. Abdominal ultrasonography and computed tomography showed marked dilatation of the common bile duct (CBD). Endoscopic retrograde cholangiography disclosed a small shadow defect in the terminal of the dilated CBD. Biopsy of the papilla revealed well-to-moderately differentiated adenocarcinoma. Considering her extreme old age and keeping in mind her quality of life after the operation, and the finding that the tumor was localized within the papilla and highly differentiated, we performed local resection. In addition, the intrapancreatic portion of the CBD and part of the main pancreatic duct (MPD) were further resected to secure a negative margin, confirmed by frozen section. The MPD was reapproximated to the duodenal mucosa and a choledocho-duodenostomy was performed for CBD reconstruction. Histopathological examination showed the tumor was papillary adenocarcinoma, 10 × 15 mm in size; there was no invasion beyond the sphincter of Oddi, it had partly infiltrated the CBD, but had not invaded to the pancreas or duodenum. The patient's postoperative course was not eventful and she has had good quality of life for the past 6 years since the operation, without any evidence of recurrence. Although radical pancreaticoduodenectomy is now the standard procedure in patients with malignant tumor of the papilla of Vater, local resection is a reasonable alternative for high-risk patients with highly differentiated, apparently localized carcinomas.