. the natural history of this disease entity has not been well established , but it has been postulated that the prognosis is generally good .
in contrast to carcinoid tumors arising in the jejunum and ileum , the clinical and laboratory findings of carcinoid syndrome are absent in patients with a carcinoid tumor of the ampulla of vater , such as duodenal carcinoid tumors . however , in small numbers of carcinoid tumors of the ampulla of vater can show more aggressive behaviors , such as distant metastasis .
. there have been several reports of endoscopic treatment of small carcinoid tumors arising from the duodenum , but there has been no report of pretreatment diagnosis and intentional endoscopic treatment of carcinoid tumors of the ampulla of vater in korea .
the ampulla of vater is a complex structure , and is the confluent portion of common bile duct , pancreatic duct and contains the sphincter of oddi .
this may explain the reason why an ampullary carcinoid often clinically manifests as obstructive jaundice or acute pancreatitis , and an attempt to remove the tumor may result in more frequent procedure - related complications . here ,
a case of a carcinoid tumor of the ampulla of vater , diagnosed before treatment and intentionally treated by endoscopic snare papillectomy , is reported .
a 62-year - old male was admitted with an incidental finding of a carcinoid tumor of the ampulla of vater .
the patient had undergone an endoscopic mucosal resection ( emr ) of early gastric cancer 1 year earlier . every 3 month following the emr ,
he has undergone surveillance gastroscopic examination with forward - viewing endoscopy . during the latest endoscopic examination ,
biopsies were taken from the lesion , which was histologically confirmed as a carcinoid tumor .
he denied weight loss , diarrhea , flushing , or any respiratory difficulties or obstructive biliary symptoms .
he underwent ercp for a more detailed evaluation of the ampulla of vater . an examination with a side - viewing duodenoscope showed prominent , but a preserved configuration of the ampulla , but depressed erosion with hyperemia on the covering mucosa ( figure 1 ) .
contrast agent was injected into common bile and pancreatic ducts , but no abnormality within the lumen of either the bile or pancreatic ducts were seen . following the ercp ,
this revealed a hypoechoic , ovoid shaped lesion less than 1 cm in diameter arising from the mucosal layer ( figure 2 ) .
the patient then underwent endoscopic snare papillectomy for the removal of the carcinoid tumor of the ampulla of vater ( figure 3 ) .
after elevation of the tumor by submucosal injection of saline solution , the ampulla was grasped by snare and a papillectomy performed using blended electrosurgical current ( cut to coagulation ratio 4:1 ) at a setting of 50 watts employing a electrosurgical unit ( ues-20 , olympus optical co. , tokyo , japan ) .
then , plastic biliary ( 7 fr , 3 cm ) and pancreatic ( 5 fr , 3 cm ) stents were inserted to prevent possible cholangitis and pancreatitis .
there was no complication after endoscopic snare papillectomy , i.e. , cholangitis , pancreatitis , bleeding or perforation .
pathologic analysis of the resected specimen revealed a 0.70.50.1 cm sized carcinoid tumor of the ampulla of vater within the mucosal layer ( figure 4 ) .
the patient had an uneventful hospital course and was discharged on the 5 post - papillectomy day following the removal of the biliary and pancreatic stents .
two months after the endoscopic snare papillectomy , a follow - up duodenoscopic examination was performed , which revealed no evidence of tumor recurrence , either macroscopically or microscopically .
six months after the endoscopic snare papillectomy , there was no evidence of local recurrence and metastasis of the tumor , either endoscopically or radiologically .
carcinoid tumors of the ampulla of vater are very rare . according to a review of 90 reported ampullary carcinoid cases , the mean patient age was 52 years , and affected patients were more often male .
jaundice was the most common presenting symptom of a carcinoid tumor of the ampulla of vater and was present in 59% of patients .
it is often apparent at an early stage because of the location ; obstructive symptoms may arise early when the tumor is relatively small . despite frequent regional lymph node metastasis
, the prognosis of carcinoid tumors of the ampulla of vater has generally been considered good . in one study ,
the 5 year survival was 90% , with only 4 ( 6% ) patients dying of a metastatic disease or progressive tumor .
even though there are reports on the benign nature of this tumor , they have malignant potential . unlike carcinoid tumors of the duodenum
, the size of the tumor seems to have no prognostic implications for carcinoid tumor of the ampulla of vater . in one review of 73 cases of carcinoid tumors of the ampulla of vater ,
thirty one were larger than 2 cm in diameter and 48% ( 15/31 ) of these had metastasized .
interestingly , 40% ( 17/42 ) of patients with tumors less than 2 cm also had a metastatic disease .
hence , the tumor size was not a reliable predictor of aggressiveness ; therefore , complete resection of the tumor is mandatory regardless of the size . in our case ,
careful examinations , including eus and abdominal ct scan , were performed before determining the therapeutic options .
the size of the tumor was small ( less than 1 cm ) and there was no evidence of muscularis propria invasion and regional lymph node metastasis on eus .
an in-111 octreotide scan performed to evaluate occult metastases showed negative findings . since the majority of carcinoid tumors express receptors for somatostatin , the detection rate using an in-111 octreotide scan ranges from 80 to 90% , and most investigators agree that this radionuclide scan and conventional imaging , such as ct , are complementary .
the treatment of choice for carcinoid tumors of the ampulla of vater is complete resection , and the standard treatment modality for the tumor has been surgery , such as pancreaticoduodenectomy or local excision of the tumor . in one review of 90 patients with an ampullary carcinoid ,
local excision was generally performed in patients with tumors less than 2 cm in diameter , while a pancreaticoduodenectomy was performed in patients with tumor larger than 2 cm .
on the other hand , 21 of the 22 patients who underwent local excision are alive , with no evidence of recurrence after long - term follow - up , and only one patient died of local recurrence 20 months following the local excision . either a pancreaticoduodenectomy or local excision , via a duodenotomy , may be selected depending on the size of the tumor .
although a pancreaticoduodenectomy enables complete resection of the tumor , this procedure has the disadvantage of relatively higher morbidity .
, local excision may be an option for the treatment of a carcinoid tumor of the ampulla of vater if the size of the tumor is small and there is no evidence of regional lymph node or distant metastasis .
compared to local surgical excision , endoscopic snare papillectomy may be much less harmful to the patient , since it does not require a laparotomy and duodenotomy . in the management of an ampullary adenoma , endoscopic therapy seems to be successful and may be a reasonable alternative to surgical resection . a carcinoid tumor of the ampulla of vater originates from the deep mucosa .
most reports describe it as a round or oval mass , with intact overlying duodenal mucosa , with negative biopsies .
only a few reports present it as a prominent papilla , with a shallow ulcer on the surface .
however , a carcinoid tumor of the ampulla of vater can show mucosal change , theoretically because it originated from the deep mucosa , not the submucosa . in our case , depressed erosion , which may be misinterpreted as papillitis , was found . without a biopsy ,
thorough examination , including the ampulla , is essential in all patients undergoing esophagogastroduodenoscopy , and biopsy of suspicious mucosal changes should always be carried out to diagnose the tumor in early stage . in conclusion ,
endoscopic snare papillectomy may be one therapeutic option in the management of carcinoid tumors of the ampulla of vater in selected patients , such as those with a small sized and localized in the mucosa or submucosal layer , without regionally or distant metastasis . further experience with more cases will be needed to establish the exact indication for endoscopic snare papillectomy .
 here , a case of a patient with incidental finding of a carcinoid tumor of the ampulla of vater , who was treated with endoscopic snare papillectomy , is reported . 
 a 62-year - old male was admitted to our hospital due to a carcinoid tumor of the ampulla of vater , which was found during follow - up endoscopy after an endoscopic mucosal resection of early gastric cancer . 
 no lymphadenopathy or visceral metastasis was found on an abdominal ct scan , in-111 octerotide scan and eus . 
 the ampulla was then en bloc removed by endoscopic snare papillectomy . 
 the resected specimen revealed a 0.70.50.1 cm sized carcinoid tumor . 
 all margins of resection were negative for tumor . 
 after six months of follow - up , there was no evidence of recurrence and metastasis , either endoscopically or radiologically . to our knowledge 
 , this case is the first report of an ampullary carcinoid tumor treated by endoscopic snare papillectomy in korea . 
INTRODUCTION
CASE REPORT
DISCUSSION