Carcinomas of the Ampulla of Vater are rare tumors, accounting for % of .. 17 . de Castro SM, van Heek NT, Kuhlmann KF, Busch OR. The complex histological structure of the papilla of Vater gives rise to a Neoptolemos JP, Talbot IC, Carr-Locke DL, Shaw DE, Cockleburgh R, Hall AW. The term ampullary tumor generally refers to either benign or malignant neoplasms that arise from the glandular epithelium of the ampulla of Vater, including 1.

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Ampulla of Vater

Ampullary neoplasms, although rare, present distinctive clinical and pathological features from other neoplastic lesions of the periampullary ampulkma.

No specific guidelines about their management are available, and they are often assimilated either to biliary tract or to pancreatic ampluoma. Due to their location, they tend to become symptomatic at an earlier stage compared to pancreatic malignancies.

This behaviour results in a higher resectability rate at diagnosis. From a pathological point of view they arise in a zone of transition between two different epithelia, and, according to their vaterr, may be divided into pancreatobiliary or intestinal type. This classification ampulomz a substantial impact on vster. In most cases, pancreaticoduodenectomy represents the treatment of choice when there is an overt or highly suspicious malignant behaviour.

In selected situations less invasive approaches, such as ampullectomy, have been advocated, although there are some concerns ve because of a higher recurrence rate associated with limited resections for invasive carcinomas. Importantly, these methods have the drawback of not including an appropriate lymphadenectomy, while nodal involvement has been shown to be frequently present also in apparently low-risk carcinomas. Endoscopic ampullectomy is now the procedure of choice in case of low up to high-grade dysplasia ampulmoa a proper assessment of the T status by endoscopic ultrasound.

In the present paper the evidence currently available is reviewed, with the aim ampuloam offering an updated framework for diagnosis and management of this specific type of disease. In this paper we review current evidence regarding ampullary neoplasm, with a particular focus on diagnosis and treatment.

We are providing a framework for management of these neoplasms that, although rare, display distinctive clinical features. Current evidence about optimal management is reviewed, outlining the role of surgery as compared to newer endoscopic techniques: Neoplasms of the ampulla of Vater account for only 0.

Although ampullary carcinomas are rare neoplasms, they occur more frequently in the ampullary region than elsewhere in the small intestine[ 2 ]. The papilla is a nipple-like structure on the medial aspect apmuloma the second portion of the duodenum, best visualized with a side-viewing endoscope.

Ampullary carcinomas are defined as gland-forming malignant epithelial neoplasms, which originate in the ampullary complex, ampuuloma to the bifurcation of the common bile duct and the pancreatic duct[ 3 ]. One of the possible causes of developing neoplasms in this area is that the ampullary region contains a transition from pancreatobiliary to intestinal epithelium, and such areas of transition are inherently unstable.

The appropriate diagnosis of ampullary neoplasms can amouloma challenging and nowadays different diagnostic modalities can be considered including high-resolution imaging techniques, endoscopy and endoscopic ultrasound EUS [ 5 ]. As a matter of fact, there are no specific guidelines for the diagnosis of these neoplasms. Usually ampullary neoplasms are incorporated into the guidelines of biliary tract[ 6 ] or pancreatic carcinomas[ 7 ]. Regarding treatment, the first local resection of an ampullary lesion was reported in and the first radical resection pancreaticoduodenectomy – PD in The latter approach resulted in a reduced risk of recurrence, even if it maintains still ampullma high morbidity rates.

In Binmoeller[ 8 ] reported the first endoscopic resection of the ampulla with a curative intent. This procedure is technically demanding, but in the last 20 years advances in endoscopic procedures with ablative techniques such as mono, bi and argon plasma coagulation as well as pancreatic and biliary stenting led to a low morbidity and mortality vatee.

Indeed endoscopic ampullectomy is the procedure of choice for ampullary adenomas and can be chosen as an alternative procedure in patients not eligible for surgery. The aim of this paper is to provide a review of the modern diagnostic tools and different treatments for ampullary neoplasms, including both endoscopic and surgical approaches.

The vaterian system is located in the wall of the second part of the duodenum, at the confluence of the common bile duct and the major pancreatic duct.

In consideration of the anatomical characteristics we reported above, it is very difficult to localize the precise origin of tumors once they have invaded adjacent tissue[ 2 ]. The cancer of the ampulla is a rare disease with an incidence of less than one per ; in autopsy series, ampullary neoplasms are seen in 0.


Tumors of ampulla of Vater: A case series and review of chemotherapy options

In the same study, women were found to be less frequently affected 0. The disease is also more common in Caucasians than in Afro-Americans.

In the study by Albores-Saavedra et al[ 9 ] an increase of ampullary cancer incidence from to has been reported, with an annual percentage rate of 0. The rates of incidence of the various histological subtypes of ampullary cancer have been approximately the same across all ages group, suggesting similar or overlapping carcinogenic pathways.

In all of the histological types surveyed, cancer was found predominantly in the older age groups. According to the age-specific rates, the incidence of cancers of the ampulla began to increase after age 30, but increased more rapidly after age 50 in both men and women; average age at diagnosis is between 60 and 70 for sporadic forms.

Although ampullary cancers are generally sporadic, some hereditary syndromes are associated with a higher risk for this type of cancer. The strongest predisposition for ampullary neoplastic disease is represented by the familiar adenomatous polyposis FAP syndrome. Compared to sporadic cases, familiar cases of ampullary cancer also tend to present at a younger age.

Gallstones are present in one third of patients, which may lead to misdiagnosis[ 14 ]. Presence of jaundice is associated with advanced stage of disease and increased risk of tumor recurrence after resection[ 15 – 20 ]. Other common symptoms include weight loss, fatigue and abdominal pain which are present in more than half of patients[ 21 ].

Acute pancreatitis is less frequent, but ampullary cancer should be ruled out in this case[ 22 ]. Up to one-third of patients have chronic, frequently occult, gastrointestinal blood loss but occasionally frank bleeding may occur[ 23 ].

Rarely, large lesions may produce gastric outlet obstruction. Serum CA is elevated in Several classifications of ampullary carcinomas have been developed according to their gross appearance based on duodenal aspect or extension of neoplasm. Three distinct categories of carcinomas are recognized, after the correlation of gross and microscopic features: The ulcerating part frequently corresponds to the invasive component, whereas the vegetating part represents the pre-invasive component; and 3 mixed exophytic and mixed ulcerated lesions[ 23031 ].

The Presence of ulcerations is associated with poor survival rate[ 32 ]. The complex histological structure of the papilla of Vater gives rise to a heterogeneous group of neoplasms with different histologic types, classified according to the predominant component.

The intestinal type, the most common invasive sub-type, is characterized by tubular or cribriform glands similar to those of colon-rectal adenocarcinomas.

Most cases are associated with areas of residual adenoma, within the ampulla and in the surrounding duodenal mucosa. The adenocarcinomas arising in an adenoma adenocarcinoma in villous adenoma, in tubulo-villous adenoma, in adenomatous polyp, and villous adenocarcinomaare usually smaller and show a better prognosis.

Pancreatobiliary carcinomas have a worse prognosis, being frequently associated with unfavourable histopathologic features, such as lymph node invasion, perineural infiltration or areas of poor differentiation[ 25283339 – 42 ]. Some ampullary adenocarcinomas may exhibit mixed features of both intestinal and pancreatobiliary type; the distinction between the two patterns may be difficult in less differentiated cases. Although closely related to the conventional type, distinct variants of adenocarcinomas include[ 43 ]: Since the vast majority of colloid carcinomas of the ampulla express the intestinal markers CDX2 and MUC2, these tumours are regarded as variants of intestinal-type adenocarcinomas; 3 signet-ring cell carcinoma[ 9 ]: Cells may be associated with extracellular mucin but the large pools seen in colloid carcinoma are lacking.

Since this entity is very rare, metastases from other more common signet cell carcinomas, mammary or gastric, should be ruled out; 4 undifferentiated carcinoma: They can occur ex-novo or be associated with other ampullary neoplasms. They are usually large and widely invasive. The spectrum of morphology varies from highly cellular, pleomorphic epithelioid mononuclear cells with abundant cytoplasm, often admixed with bizarre multinucleated giant cells to relatively monomorphic epithelioid and spindle; 5 papillary adenocarcinoma[ 45 ]: The invasive carcinomas show either pancreatobiliary or intestinal phenotype; and 6 neuroendocrine carcinoma[ 46 ]: Their histological features and prognosis resemble those of their pulmonary counterparts.

Tumors of ampulla of Vater: A case series and review of chemotherapy options

The T classification depends on the extension of the primary neoplasm: Staging classification of the ampullary neoplasms according to the American Joint Committee on Cancer TNM classification, 7 th edition[ 47 ]. Regional lymph nodes include the peripancreatic lymph nodes superior and inferior pancreatic head nodes; anterior and posterior pancreatico-duodenal nodes and the lymph nodes along the hepatic artery, proximal mesenteric artery, celiac axis and pyloric regions.


Optimal histological examination of pancreaticoduodenectomy specimen should include analysis of a minimum of 12 lymph nodes[ 47 ]. This observation is particularly important for surgical management of these lesions, as we will discuss in the section about treatment.

Metastasis to lymph nodes outside the regional groups described above, such as nodes of the pancreas tail or para-aortic ones, is considered as metastatic disease M1. Compared to the previous one, the new stage classification has been modified according to new prognostic information; nodal positivity is included in stage IIB, while stage III comprises patients with extensive T4 tumors, with or without nodal disease.

IA, including tumors limited to the ampulla of Vater or sphincter of Oddi, and IB, indicating cancers that invade the duodenal wall. Stage IV is represented by metastatic tumors[ 56 ]. Following surgical resection, recurrence may occur locally involving the tumor bed or the para-aortic lymphatics [ 57 ] or at a distant site.

Peripancreatic lymph nodes are the most frequent site of nodal involvement and, compared to pancreatic carcinoma, disease is more likely to be limited to this region. Vster spreading of ampullary carcinoma generally follows a halsteadian progression: Some data suggest a preferential lymphatic drainage flow from posterior pancreatico-duodenal lymph nodes to nodes located around the superior mesenteric artery, thereby underlining the importance of nodal dissection at least in amuploma areas[ 32 ].

In the diagnostic evaluation of jaundiced patients with suspected malignant bile duct obstruction, benign tumors, inflammatory diseases and gallstones must be excluded first. Then the extent of tumor invasion and spread has to a,puloma established.

During the diagnostic workup, another issue is to differentiate primary ampullary carcinoma from the more common periampullary tumors, including pancreatic, duodenum and bile bater carcinomas. In most cases, the distinction is not essential from a surgical point of view because, if a malignant lesion is suspected in that area, the patients will undergo the same operation pancreaticoduodenectomy.

However ampullary and periampullary tumors have substantially different oncologic implications and prognosis. In the evaluation of patients with jaundice trans abdominal ultrasonography should be the first imaging study, since it can identify intra and extra hepatic bile duct dilatation, a distended gallbladder and gallstones; however the ampullary tumor may not be visualized[ 58 ]. Due to low sensitivity of US, it is necessary not to underestimate indirect signs and to maintain a high level of suspicion.

US evaluation in any case can give a clue to choosing the next most appropriate diagnostic exam: CT scan must be chosen if the mass is solid, whilst when no masses are identified eco endoscopy EUS must be performed. Endoscopic transpapillary forceps biopsies; Vter Transpapillary intraductal ultrasonography; MRI: Ce resonance imaging; Sens: Abdominal CT is more sensitive than US for evaluating the periampullary region, since it is not limited by the presence of bowel gas or obesity and is less operator-dependent.

On the other hand, its diagnostic accuracy is not very high for small ampullary masses, especially if they are located within the duodenal wall or in the lumen.

In order to maximize CT sensitivity, i. A bulging papilla can be encountered in healthy individuals as well as in patients with inflammatory diseases papillitis from passage of biliary stones, parasites, infections or periampullary diverticulabenign or malignant tumors.

Mural thickening and attenuation pattern of contrast medium may be of help for differentiating normal from pathological papilla[ 65 ]. CT spatial resolution is often inadequate vaterr define the local tumor invasion into the surrounding structures[ 66 ].

As a final consideration, CT is however always necessary for staging any malignant disease, as it can identify distant metastatic involvement like regional lymph-nodes, liver, peritoneum, lung and bone. CT virtual endoscopy is a new non-invasive diagnostic tool, which still has to be more extensively evaluated and developed[ 67 ].