For ampullary carcinoma, the only potentially curative treatment is pancreaticoduodenectomy. Preoperative biliary drainage may be needed in the following situations: (i) acute cholangitis and marked pruritus and (ii) delayed surgery. Despite the high rate of potentially curative resection, recurrence is frequent, suggesting the need for adjuvant therapy. Adjuvant therapy with chemotherapy or chemoradiotherapy has been suggested but the results of clinical trials are not definitive. Many clinicians do not recommend adjuvant therapy because of the more favorable prognosis of ampullary as compared to other periampullary tumors and the lack of data proving a survival advantage. Others suggest an approach similar to the one used for resected pancreatic cancer, with adjuvant chemotherapy with gemcitabine or leucovorin – modulated fluorouracil (5-FU). It is mostly used alone after resection, but it can be combined with radiotherapy, although the optimal way to sequence 5-FU-based chemotherapy is unclear.
Endoscopic papillectomy has been suggested to be a possible curative treatment in early stages AC (Tis, T1), well-differentiated, with clear margins of resection and without lymphovascular invasion (49). However, acceptance of this method for definitive treatment is hampered by the inadequacy of biopsy in terms of correct pathologic characterization of the lesion (and therefore its risk of harboring lymph node metastases) and the inability to predict successful, margin-negative endoscopic resection.
Palliative endoscopic biliary drainage
In cases of advanced tumor pathology with a palliative therapy indication, endoscopic drainage of ductal obstruction via papillotomy or transpapillary stent placement has been a widely accepted technique that provides effective symptomatic relief in patients with obstruction of the biliary or pancreatic ducts. If papilla is not approachable, EUS can be used as a guide to obtain access to the bile duct from the duodenum or to the intrahepatic bile duct from the stomach. However, there are only case series reports and some report complications as bile leakage and stent migration. As such, it should be reserved for highly specialized biliopancreatic centers.
Other minimally invasive nonsurgical therapies for palliative intent include preoperative endoscopic debulking, Nd-YAG laser ablation and photodynamic therapy. The literature on these techniques is limited to single-case reports and small series.
EUS-Guided Celiac Plexus Neurolysis
In the case of pain management, neurolysis of the celiac plexus can be performed through EUS. The efficacy and risk of hypotension, increased pain and diarrhea are all transient and similar to those of other access routes. The risk of neurological damage diminishes in procedures that address the celiac plexus through the anterior route and severe complications have not been reported.
For advanced disease, limited data exist to guide physicians in the choice of the optimal regimen for palliative chemotherapy, largely because of the rarity of this disease. Some studies with gemcitabine with cisplatin have shown a significant survival advantage. Others use a single agent gemcitabine initially, followed by an oxaliplatin-based regimen, or vice-versa.
• Prognosis: In general terms, AC have higher rates of resectability and better prognosis than other periampullary cancers, as duodenal, pancreatic head or distal common bile duct (50). The outcome of resected AC depends upon the histology (depth of local invasion), status of the surgical margins and the presence of metastases. Other prognostic factors are obstructive jaundice, intraoperative blood transfusion and tumor marker elevation. Studies estimate a five-year survival rate of 64-80% in patients submitted to pancreaticoduodenectomy and node-negative disease and 17-50% if limited lymph node involvement, in opposition to the less than 10% 2-year survival rate in complete resected node-positive pancreatic cancer. This may be, in part, because the anatomic location allows symptoms and presentation to occur earlier, and also in part because ampullary adenocarcinoma tends histologically to be more differentiated.
PREVENTION? (OR SCREENING AND SURVEILLANCE?)
Patients with FAP almost invariably develop duodenal adenomas and have a higher risk for AC, the most common malignancy and leading cause of death in those who have previously undergone colectomy. Consequently, screening and surveillance of these high-risk patients is desirable. American College of Gastroenterology Clinical Guidelines recommends that “screening for proximal small bowel tumors should be done using upper endoscopy including duodenoscopy starting at age 25–30 years and surveillance should be repeated every 0.5–4 years depending on Spigelman stage of duodenal polyposis”.
Surveillance of AA after endoscopic papillectomy is recommended but it is not standardized. It involves duodenoscopy, multiple biopsies and ductogram to rule out the possibility of intraductal residual or recurrent lesions. Initial surveillance examination has been suggested 1-6 months after the initial procedure, followed by repeat examinations every 3-12 months for 2 years. The periodicity required thereafter is guided by several factors, as histology, age and operability of the patients and history of FAP. In the absence of FAP and firm recommendations, a surveillance strategy similar to the one used in colon polyps is defended. Additionally, screening colonoscopy should be undertaken to exclude colonic polyps. FAP patients should undergo surveillance at 3-year intervals, given the risk for methacronous duodenal lesions.
Posttreatment surveillance of AC is performed at regular intervals, although the optimal surveillance strategy is undefined. Many approaches include a follow-up of every 6 months for 5 years and annually thereafter. It includes history and clinical examination and serum tumor markers CEA and CA 19.9; the role of periodic abdominal CT is not defined.