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Bast RC Jr, Kufe DW, Pollock RE, et al., editors. Holland-Frei Cancer Medicine. 5th edition. Hamilton (ON): BC Decker; 2000.

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Holland-Frei Cancer Medicine. 5th edition.

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Chapter 100Neoplasms of the Ampulla of Vater

, MD and , MD.

Cancers of the periampullary region include tumors of the pancreas, distal bile duct, ampulla of Vater, and periampullary duodenum; the later three are very rare and often difficult or impossible to differentiate from adenocarcinoma of pancreatic origin (prior to surgical resection). This chapter will focus on neoplasms arising from the ampulla of Vater.

Nonpancreatic periampullary cancer is felt to have an improved prognosis compared to adenocarcinoma of the exocrine pancreas. This may be secondary to both earlier diagnosis and a more favorable tumor biology. Neoplasms of the ampullary region cause biliary obstruction when the primary tumor is relatively small. To the extent that small tumor size (T stage) is associated with improved survival (increased tumor size associated with decreased survival duration for exocrine and endocrine pancreatic cancer1,2), one would expect patients with neoplasms arising from the ampulla of Vater to be diagnosed earlier in the course of their disease than patients with primary tumors of the pancreas due to the early development of biliary obstruction. Patients who undergo pancreaticoduodenectomy for localized periampullary adenocarcinoma of nonpancreatic origin have a superior survival duration compared with similarly treated patients who have adenocarcinoma of pancreatic origin (Table 100.13–8). However, similar to the natural history of pancreatic adenocarcinoma, extent of disease and the histologic characteristics of the primary tumor predict survival duration. Even in patients who undergo a potentially curative resection, the presence of lymph node metastases, poorly differentiated histology, positive margins of resection, and tumor invasion into the pancreas are associated with a less favorable outcome.3,6,7,9–11

Table 100.1. Survival in Patients with Localized Periampullary Adenocarcinoma Who Underwent Pancreaticoduodenectomy.

Table 100.1

Survival in Patients with Localized Periampullary Adenocarcinoma Who Underwent Pancreaticoduodenectomy.

Histologic evidence of tumor extension from the ampulla into the pancreatic parenchyma appears to reflect the extent of both local and regional disease. In a recent report by Harada and colleagues,6 pathologic variables and clinical outcome were analyzed for 63 patients with adenocarcinoma of the ampulla of Vater treated with pancreaticoduodenectomy; pancreatic invasion was present histologically in 37 (59%) patients’ specimens and absent in 26. The presence of pancreatic invasion was associated with an increased incidence of lymph node metastasis (62 vs. 19%) and a marked reduction in 5-year survival (24 vs. 79%). The prognostic significance of tumor infiltration into the substance of the pancreas is accounted for in the current TNM staging system (Table 100.2).12

Table 100.2. TNM Staging System.

Table 100.2

TNM Staging System.

In contrast to the natural history of adenocarcinoma of pancreatic origin, lymph node metastasis in patients with adenocarcinoma of the ampulla of Vater is not as powerful a predictor of disease recurrence or short survival duration (Table 100.33–7,10). Although most investigators3,7,9 have confirmed an association between the presence of lymph node metastases and decreased survival duration, Allema and colleagues4 found no statistically significant difference in 5-year survival following pancreaticoduodenectomy between 23 node-negative patients (59% 5-year survival) and 35 node-positive patients (41% 5-year survival; p = .36). Yeo and colleagues13 reported 46 patients with ampullary adenocarcinoma and a minimum follow-up of 5 years; the status of regional lymph nodes was not a predictor of 5-year survival (p = .14). Actual 5-year survival in these 46 patients was 39% and actuarial 10-year survival was 25%. However, in a larger report from the same institution, patients with lymph node metastases did have inferior survival duration (5-year survival 31% in node-positive patients vs. 43% in node-negative patients; p = .05).5

Table 100.3. Survival in Patients with Localized, Node-Positive Periampullary Adenocarcinoma Who Underwent Pancreaticoduodenectomy.

Table 100.3

Survival in Patients with Localized, Node-Positive Periampullary Adenocarcinoma Who Underwent Pancreaticoduodenectomy.

It appears that standard pathologic criteria can be used to predict outcome in patients with ampullary adenocarcinoma.7,9,13,14 However, lymph node metastasis should not represent a contraindication to potentially curative pancreaticoduodenectomy.

Diagnostic Evaluation

Unless detected as part of a screening program in patients with familial adenomatous polyposis (FAP), the vast majority of patients with periampullary neoplasms will develop biliary obstruction and present with hyperbilirubinemia. As is true for patients with suspected pancreatic cancer, we prefer to proceed with abdominal imaging in the form of computed tomography (CT) prior to upper endoscopy (Fig. 100.1). In patients with localized nonmetastatic disease, staging is then completed with upper endoscopy to include endoscopic ultrasonography (EUS). Kubo and colleagues15 recently reported an experience with 35 patients who underwent EUS evaluation prior to surgical resection for ampullary neoplasms (adenocarcinoma, 31; adenoma, 3; and carcinoid, 1). The overall accuracy in staging the depth of invasion of the primary tumor was 74%; 6 patients were overstaged and 3 patients were understaged. Only 4 of 10 patients found to have lymph node metastasis on final pathologic assessment of the resected specimen were correctly identified by EUS. Histologic evidence of pancreatic infiltration by the primary tumor was found in 12 patients and correctly identified by EUS in 10 of the 12 patients. However, Menzel and colleagues16 demonstrated that EUS was unable to differentiate adenoma from carcinoma. The presence of a hypoechoic mass with irregular margins and inhomogeneous echo-poor areas invading into surrounding structures was interpreted as consistent with malignancy. Three of eight carcinomas were incorrectly judged to be adenomas, and four of eight adenomas were incorrectly judged to be carcinomas. EUS cannot reliably differentiate adenoma from carcinoma and will understage at least 20% of EUS-staged T1 carcinomas.17

Figure 100.1. Management algorithm employed at The University of Texas M.

Figure 100.1

Management algorithm employed at The University of Texas M.D. Anderson Cancer Center for patients with suspected adenocarcinoma of the periampullary region. Accurate radiographic imaging with contrast-enhanced computed tomography is followed by endoscopic (more...)

Endoscopic retrograde cholangiopancreatography (ERCP) is necessary in the setting of biliary obstruction without an endoscopically visible neoplasm. To prevent cholangitis in patients with extrahepatic biliary obstruction who undergo diagnostic ERCP, we routinely place endoscopic stents. Endoscopic stents are also placed in patients with biliary obstruction in whom surgery will be delayed due to the planned delivery of neoadjuvant therapy.

Treatment

Potentially Resectable Disease

The standard treatment for localized invasive adenocarcinoma of the ampulla of Vater is pancreaticoduodenectomy. Local resection of the ampulla of Vater is a reasonable alternative to pancreaticoduodenectomy in patients with no evidence of carcinoma on endoscopic biopsy and in whom CT and EUS suggest the absence of pancreatic invasion. Local resection of the ampulla of Vater was first described by Halsted in 1899.18 It remains an attractive alternative to pancreaticoduodenectomy due to the prolonged recovery period and defined morbidity associated with pancreaticoduodenectomy.19 Local excision is generally recommended only for benign tumors (or tumors that contain carcinoma in situ) less than 3 to 4 cm in size.20,21 Invasive adenocarcinoma should be treated with pancreaticoduodenectomy unless age, performance status, or medical comorbidies present a contraindication to major abdominal surgery. Even T1 adenocarcinomas of the ampulla of Vater have at least a 20% incidence of lymph node metastasis.9

The application of local excision is made more difficult by the frequent finding of false-negative endoscopic biopsy results. The false-negative rate may be as high as 47%.20 Further, the difficulty in performing a wide, margin-negative resection is evidenced by the frequent development of local recurrence (Table 100.420–24). In the largest series reported to date, Farnell and colleagues20 reported on 52 patients who underwent transduodenal local excision for villous tumors of the duodenum that were benign or contained carcinoma in situ. Local recurrence developed in 17 (32%) of the 52 patients. In 4 (23%) of the 17 patients, the tumor recurred as adenocarcinoma. In contrast, Rattner and colleagues21 reported an experience with local ampullary resection in 14 patients including 2 with adenocarcinoma. There were no local recurrences after a mean follow-up of 19 months. However, lesions treated by local resection were small (1.8 cm ± 1 cm), a 5 to 10-mm cuff of normal duodenum (down to the muscularis propria) was removed with the ampullary neoplasm, and the authors carefully performed frozen section analysis of the biliary and pancreatic duct margins.

Table 100.4. Published Experience with Local Excision for Periampullary Neoplasms.

Table 100.4

Published Experience with Local Excision for Periampullary Neoplasms.

Local resection of the ampulla remains an infrequent surgical procedure due to the inability to accurately exclude invasive adenocarcinoma preoperatively (either by EUS or endoscopic biopsy), the inability to accurately define depth of invasion, and the frequent development of local recurrence even with benign lesions.

Adjuvant Therapy and the Management of Locally Advanced Disease

On occasion, radiation oncologists and medical oncologists are asked to render an opinion regarding the utility of adjuvant chemoradiation or chemotherapy in the setting of locally advanced ampullary adenocarcinoma. Unfortunately, there are few available data on which to base such an opinion. As is often done with other rare malignancies, decisions regarding the use of adjuvant therapy are based on natural history data extrapolated from tumors with a similar phenotype and biologic behavior, that share similar and pathologic prognostic factors. The biology, natural history, and histology of ampullary carcinoma appear most closely related to adenocarcinoma of the pancreas and duodenum.13 For example, in a retrospective review from the Johns Hopkins Hospital, the actual 5-year survival rates for patients with adenocarcinoma of the pancreas, ampulla of Vater, and duodenum were 6%, 39%, and 59%, respectively.13

The presence of lymph node metastases is commonly used to assess the need for postsurgical adjuvant therapy in patients with gastrointestinal malignancies. Although not as powerful a prognostic factor as with pancreatic cancer, metastatic disease in regional lymph nodes clearly increases the likelihood of locoregional and distant recurrence.3,7,8,25 Shirai and colleagues11 reported 18 patients with ampullary adenocarcinoma found to have lymph node metastasis following pancreaticoduodenectomy; 11 patients had 1 to 3 positive nodes, and 7 patients had 4 or more positive nodes. The 5-year survival was 81% for 17 node-negative patients, 71% for the 11 patients with 1 to 3 positive nodes, and 0% for the 7 patients with 4 or more positive nodes. The authors suggested that a subset of patients with adenocarcinoma of the ampulla of Vater and aggressive tumor biology can be identified as those with multiple lymph node metastases. These data suggest that adjuvant therapy should be considered for patients with high-risk histology to include multiple positive regional lymph nodes. This recommendation is in agreement with a previous report by Willett and colleagues,14 who defined patients at high risk for recurrence as those with histologic evidence of tumor invasion of the pancreas, lymph node metastasis, positive margins of resection, or poorly differentiated histology. For 17 high-risk patients in their series who received only pancreaticoduodenectomy with no further treatment, 5-year actuarial local tumor control was 50% and 5-year survival 38%. These patients were compared to 12 high-risk patients who received postoperative adjuvant 5-fluorouracil(FU)-based chemoradiation in whom 5-year actuarial local tumor control was 83% and 5-year survival was 51%. An additional 12 low-risk patients (tumors limited to the ampulla or duodenum, negative margins, negative nodes, moderate- or well-differentiated histology) had no local recurrences and a 5-year survival of 80%.

Local recurrence following pancreaticoduodenectomy can be due to either tumor infiltration into the mesentary at the origin of the superior mesenteric artery or recurrence within peripancreatic and retroperitoneal lymph nodes (Fig. 100.2).26 It is therefore reasonable to consider adjuvant chemoradiation following pancreaticoduodenectomy in patients with lymph node metastases. Treatment schemas using 5-FU-based chemoradiation have been used most commonly.

Figure 100.2. Contrast-enhanced helical computed tomography scan demonstrating a local recurrence 2 years following a pancreaticoduodenectomy for adenocarcinoma of the ampulla of Vater .

Figure 100.2

Contrast-enhanced helical computed tomography scan demonstrating a local recurrence 2 years following a pancreaticoduodenectomy for adenocarcinoma of the ampulla of Vater . The low-density tumor is surrounding the superior mesenteric artery (arrow). The (more...)

In patients with bulky peripancreatic, retroperitoneal, or celiac adenopathy suggestive of regionally advanced disease (Fig. 100.3), chemoradiation prior to surgery should be considered. These patients are at high risk for having systemic metastases that may not be evident at the time of initial diagnosis. A treatment schema using preoperative chemotherapy or chemoradiation attempts to avoid the development of liver metastases early after a major surgical procedure by avoiding surgery in patients with rapidly progressive disease. Following chemoradiation, patients who receive neoadjuvant therapy undergo a repeat staging CT; if progressive disease is demonstrated, a major surgical procedure can be avoided. When delivering multi-modality therapy, it is beneficial, when possible, to deliver the most toxic therapy last, thereby avoiding treatment toxicity in patients who experience rapid disease progression not amenable to available therapies.

Figure 100.3. Contrast-enhanced helical computed tomography scan (A) demonstrating a regionally advanced adenocarcinoma of the ampulla of Vater.

Figure 100.3

Contrast-enhanced helical computed tomography scan (A) demonstrating a regionally advanced adenocarcinoma of the ampulla of Vater. Metastatic disease is strongly suspected in the large low-density retropancreatic lymph node (arrow), which lies just medial (more...)

For patients with localized disease who are not surgical candidates, the use of chemoradiation to maximize local disease control is a reasonable treatment plan. For patients with an acceptable performance status, this approach is clearly supported by studies in unresectable, locally advanced pancreatic cancer.27,28 The decision to proceed with such therapy should be based on an accurate assessment of the patient’s performance status and with the clear informed consent of the patient.

Metastatic Disease

Adenocarcinoma of the ampulla of Vater has a similar pattern of disease recurrence as is seen with duodenal adenocarcinoma; liver metastases, and peritoneal and local recurrence. Unfortunately, there is no published literature to guide the use of systemic therapy for metastatic adenocarcinoma of the ampulla of Vater. Further, little has been published on the use of systemic therapy for duodenal cancer, which is thought to have a similar, although slightly more favorable, biologic behavior. In a patient with metastatic adenocarcinoma of ampullary origin, treatment with 5-FU or 5-FU-based combinations is reasonable. It must be emphasized that such a recommendation relies on isolated reports of successfully treated patients with duodenal cancer who received a variety of drug combinations including 5-FU alone, UFTM (weekly tegafur [400 mg/m2], uracil [1,344 mg/d], and mitomycin [8 mg]) or FAM (5-FU, doxorubicin [Adriamycin], and mitomycin C).29–31

The complex pathophysiologic abnormalities accompanying metastatic upper abdominal malignancies often make specific treatment decisions extremely difficult. Many patients present to the medical oncologist or surgeon with profound debilitation, severe pain, and extensive metastatic disease. For these patients, chemotherapy is unlikely to result in significant improvements in quality of life or survival, and the toxic effects of chemotherapy may create additional complications. Management with supportive care or nontoxic hormonal approaches may be the optimal strategies for these patients. For patients with metastatic disease who present with a good performance status, systemic chemotherapy is appropriate. Because these tumors are so rare, treatment on a site-specific phase II protocol is generally not possible. For selected patients, it may be appropriate to consider treatment on a phase I trial as either initial treatment or after demonstrated lack of response to 5-FU-based therapy.

Associated Syndromes

Periampullary neoplasms also occur as part of two inherited syndromes: FAP and von Recklinghausen neurofibromatosis. In patients with FAP, duodenal polyps develop in 50 to 90% of patients and cluster in the periampullary region. The adenoma-carcinoma sequence has been clearly established, and periampullary adenocarcinoma is a major cause of extracolonic death being responsible for up to 20% of deaths in patients with FAP who have undergone a prior prophylactic colectomy.32,33 FAP is caused by a germline mutation in the tumor suppressor gene APC on chromosome 5q22. There may be an association between the precise mutation and the phenotypic expression of the disease. For example, there appears to be familial segregation in the occurrence of upper gastrointestinal polyposis and periampullary malignancy.34 Polyps (and cancer) may occur in all parts of the upper gastrointestinal tract, demonstrating that the entire epithelium is at risk in patients with FAP. Polyp formation appears to cluster in areas with the highest bile exposure. Bile has been implicated in both the development of mucosal injury and in DNA adduct formation.35

The cumulative probability of a patient with FAP developing a noncolorectal cancer by age 50 is approximately 11%, and one half will be in the periampullary location, mandating endoscopic surveillance in this patient population.36 The treatment of patients with periampullary neoplasms and FAP is complicated by the fact that the polyps are often confluent, flat lesions not amenable to snar excision. Repeated diathermy may result in scar formation leading to duodenal stricture. In addition to careful endoscopic surveillance, investigators have considered photodynamic therapy, argon-beam coagulation, and the use of cyclooxygenase inhibitors (i.e., sulindac).35,37 Surgical options involve local excision and pancreaticoduodenectomy. Due to the multi-focal nature of the duodenal polyps in patients with FAP, local excision results in early recurrence and therefore is rarely performed.38 The management of extensive duodenal polyposis remains somewhat uncertain. Patients with no evidence of invasion by histologic biopsy and EUS are generally subjected to very careful endoscopic surveillance with repeat endoscopies at 6-month intervals. The application of pancreaticoduodenectomy to this patient population is made more complicated by the potential for weight loss and profound nutritional depletion in patients who undergo pancreaticoduodenectomy following total colectomy, and the frequent development of desmoid tumors.

Desmoid tumors are usually slow-growing neoplasms consisting of mature fibroblasts within a collagen matrix. Mitoses are rare, and cellularity is usually scant in the center of the tumor and more dense at the periphery. They have no true capsule and often infiltrate the small bowel mesentery where the majority of desmoid tumors develop. In patients with FAP who have undergone prophylactic colectomy, desmoid tumors may be responsible for up to 30% of deaths due to FAP-related diseases.33 Although their etiology is unknown, there is a strong association with prior surgery, whereas in FAP they can occur spontaneously. Additionally, desmoids have been reported to occur more frequently in FAP patients with a mutation of codon 1444.39 Mesenteric desmoids present a particular problem in the patient who may require surgery for periampullary polyposis in whom invasive cancer is highly suspected or biopsy proven. If such a patient has a mesenteric desmoid, pancreaticoduodenectomy (and the necessary pancreaticobiliary and gastrointestinal reconstruction) may be impossible due to mesenteric forshortening as a result of mesenteric infiltration by the neoplastic desmoid process.40 FAP patients should be carefully evaluated with CT prior to upper abdominal surgery; mesenteric desmoid tumors may exist as diffuse fibromatosis and not the typical round or oval-shaped solid tumor mass one usually associates with this neoplasm.

The association of von Recklinghausen neurofibromatosis with pheochromocytoma and ampullary tumors is well described.41–43 Neurofibromatosis is an autosomal dominant trait and pheochromocytoma occurs in at least 1% of affected patients. The occurrence of periampullary neuroendocrine tumors in patients with neurofibromatosis was first noted by Lee and Garber,44 and Griffiths and colleagues45 demonstrated that the ampullary tumors stained positive for somatostatin by immunohistochemistry. Somatostatin-rich carcinoid (or neuroendocrine) tumors usually occur in the periampullary region of the duodenum causing biliary obstruction or gastrointestinal blood loss. They are most often diagnosed when small; whether their small size at diagnosis is related to their modest malignant potential remains speculative. The majority of somatostatin-rich ampullary carcinoid tumors in patients with neurofibromatosis have not metastasized to liver or lung at the time of diagnosis.41 Similar to other neuroendocrine tumors, regional lymph node metastasis does not imply the subsequent development of distant organ metastatic disease. Standard treatment is pancreaticoduodenectomy; however, an occasionally small superficial neoplasm may be amenable to local excision.

Conclusions

Neoplasms arising from the ampulla of Vater are rare; however, they are often impossible to differentiate from pancreatic adenocarcinoma prior to surgical resection. Whereas surgery alone is curative in up to 40 to 50% of patients with localized nonmetastatic adenocarcinoma of the ampulla of Vater, such is not the case with adenocarcinoma of the pancreas. Multi-modality therapy is clearly superior to surgery alone for patients with localized, potentially resectable pancreatic cancer. Therein lies the importance of accurate pretreatment imaging with CT and EUS. Further, although the technical aspects of pancreaticoduodenectomy are often less complicated in patients with ampullary neoplasms (in contrast to adenocarcinoma of pancreatic origin), current data suggest that pancreaticoduodenectomy should be performed in centers experienced with this surgical procedure; there remains a linear relationship between surgical volume and outcome.46,47

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