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Treatment 5yr survival = 50 %. Adjuvant therapy RT +/-combination chemoradiotherapy with a fluoropyrim - dine (eg.(5-FU)) or capecitabine if positive surgical margins. Guidance if negative margins is contraversial Prognosis 70 % for patients with lymph node-negative disease. 25 % for node-positive disease. Prognostic factors: High-grade (poorly differentiated) histology. Residual tumor identified at the surgical margins. TNM. Obstructive jaundice- worse prognosis. Intraoperative blood transfusion (>3U intra-operatively have a worse prognosis). Tumour marker elevation- preoperative levels of CA 19-9 >150 U/mL in non-jaundiced patients or >300 U/mL in the presence of cholestasis is associated with unresectable disease in periampullary carcinomas. Preoperative elevated levels of CA 19-9 and high platelet-lymphocyte ratio- are predictors of worse survival and of the need for adjuvant therapy. Periampullary lesion Definition: Neoplasms that arise in the vicinity of the ampulla of Vater. Adenomas (villous and tubulovillous) Also... Hemangiomas Leiomyomas Best route is undecided: Excision (advocated as high rate of focus of adenocarcinoma) vs surveillance (unlikely to progress and less mortality/morbidity) Treatment Of Ampullary Carcinomas
Incidence increased 200-300x with FAP and HNPCC Pathology 15% of ampullary adenomas have a focus of adenocarcinoma. If negative biopsies for cancer one should still remain suspicious. These display the adenoma- carcinoma sequence observed in colorectal neoplasia. K-ras mutations are an early event in ampullary carcinogenesis (in 37%). High COX-2 expression has been detected in 78 % of ampullary carcinomas.
Endoscopic signs indicating malignant transformation: Induration and rigidity of the papilla on probing. Ulceration of the lesion. A submucosal mass effect that leaves the overlying mucosa intact but signifies tumour extension into the duodenal wall. Failure to achieve a cleavage plane with submucosal injection. Clinical features Usually present > age 40 if sporadic; earlier if have FAP. Patients usually present with obstructive jaundice.
K-ras
COX-2
Mucosal Origin:
Minimally-invasive nonsurgical therapies Photodynamic therapy- less healthy tissue destruction Argon plasma coagulation (APC) Nd:YAG laser ablation Offers the potential for control of local tumor growth May result in prolonged survival if tumor is not extensive. Endoscopic snare resection- Reduces tumor bulk Benefit is short-lived because of incomplete tumor removal Procedure is complicated by hemorrhage. Local resection Lower morbidity than Whipple’s but higher recurrence and worse survival. Used for: a) Elderly with significant co-morbidities b)Sometimes for early, low-grade tumors (well-differentiated small (<6 mm) tumors that do not penetrate through the ampullary musculature (ie, Tis, pT1)) (LN mets found in <4 % with early, minimally-invasive tumors so lack of LN resection usually not a problem)- most surgeons prefer Whipples for this c)For patients with an adenoma, or cancer in an adenoma with a low possibility of invasion into the sphincter of Oddi (otherwise undefined). Pancreaticoduodenectomy (Whipple operation)- preferably pylorus-preserving Curative resection in 85 % Consider Whipples even as palliative procedure (better quality of life and longer survivial) Peri-operative mortality < 5 % Perioperative morbidity rates : 30 % >50% die from recurrent disease after whipples alone. Complications a) Pancreatic fistula (20 %) usually from the pancreaticodu - denal anastomosis- higher in ampullary cancer than pancreatic as normal pancreas in the former b) Delayed gastric emptying, c) Haemorrhage d) Sepsis (pneumonia/ leaks/collections) e) Postoperative diabetes as a result of pancreatic resection. Duodenum Bile Duct Pancreas Leiomyofibromas Lipomas Lymphangiomas Neurogenic tumors. Best means of excision also contraversial: Whipple’s-low recurrence rate, higher morbidity+mortality. Local surgical excision safer but higher recurrence rate and need surveillance Snare ampullectomy Has lower mortality and morbidity than local surgical excision But... May need multiple procedures (mean 2.3) to effect complete excision Recurrence rates approaching 30 % Requirement for continued endoscopic surveillance. Abdominal CT Pancreatic protocol CT is more sensitive than US for evaluating the periampullary region. Magnification endoscopy with narrow band imaging Visualises abnormal vessels on the surface of adenomas/adenocarcinomas MRCP (= filling defects protruding into duodenum, with characteristic delayed enhancement) Overall accuracy of diagnosis with MRCP was 76 %. Intraductal USS -Can be passed through standard endoscopes directly into the bile or pancreatic duct. -IDUS can be useful for diagnosing and assessing the size and extent of papillary tumors as distinguishes SOD from remainder of papilla -IDUS was more accurate than EUS for T-staging and evaluating ductal invasion. ERCP Serum tumor markers Can get increased CA 19-9 and/or carcinoembryogenic antigen (CEA) . EUS Useful to clarify biopsy negative uncharacteristic lesions at ampulla EUS+ FNA at ampulla- sensitivity 82 %, specificity of 100 %. Very accurate for T staging EUS is the most accurate modality available to assess the T-stage of ampullary tumors, which is critical for planning surgical interven - tion. Primary tumor (T) staging accuracies of 80 %. Diagnosis and Staging Treatment Of Ampullary Adenomas
Written by Dr Sebastian Zeki
Posttreatment Surveillance Surveillance endoscopy is indicated every 6m for 2yrs, then annually for 3-5 years.
