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home - Pancreas - Pancreatic Cancers - Periampullary Cancer Written by Dr Sebastian Zeki

Knows the presentation investigation and staging of pancreatic

Recognises the importance of considering and being able to identify
uncommon pancreatic tumours (such as neuroendocrine or
intrapapillary mucinous tuours)

Knows the range of potential therapies and recognises the factors
that make such tumours potentially operable or inoperable

Knows the prevalence and natural history of benign cysts/serous
cystadenoma and potentially malignant cystic lesions

Knows the options for palliative treatment
Shows ability to sequence investigations appropriately
Understands value of multi-disciplinary team
Recognises the importance of considering possibility that the tumour
is unusual

Communicates effectively within the multi-disciplinary team and with
the patient and their family

Periampullary Cancer

Treatment 5yr survival = 50 %.Adjuvant therapyRT +/-combination chemoradiotherapy with a fluoropyrim-dine (eg.(5-FU)) or capecitabine if positive surgical margins.Guidance if negative margins is contraversial Prognosis70 % 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 lesionDefinition: Neoplasms that arise in the vicinity of the ampulla of Vater. Adenomas (villous and tubulovillous)Also...HemangiomasLeiomyomas 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 Pathology15% 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 featuresUsually present > age 40 if sporadic; earlier if have FAP.Patients usually present with obstructive jaundice. K-ras COX-2 Mucosal Origin: Minimally-invasive nonsurgical therapiesPhotodynamic therapy- less healthy tissue destructionArgon plasma coagulation (APC)Nd:YAG laser ablationOffers the potential for control of local tumor growth May result in prolonged survival if tumor is not extensive.Endoscopic snare resection-Reduces tumor bulkBenefit is short-lived because of incomplete tumor removalProcedure is complicated by hemorrhage. Local resectionLower morbidity than Whipple’s but higher recurrence and worse survival.Used for: a) Elderly with significant co-morbiditiesb)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 thisc)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-preservingCurative 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.Complicationsa) Pancreatic fistula (20 %) usually from the pancreaticodu-denal anastomosis- higher in ampullary cancer than pancreatic as normal pancreas in the formerb) Delayed gastric emptying,c) Haemorrhaged) Sepsis (pneumonia/ leaks/collections)e) Postoperative diabetes as a result of pancreatic resection. Duodenum Bile Duct Pancreas LeiomyofibromasLipomasLymphangiomasNeurogenic 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 surveillanceSnare ampullectomy Has lower mortality and morbidity than local surgical excision But...May need multiple procedures (mean 2.3) to effect complete excisionRecurrence rates approaching 30 %Requirement for continued endoscopic surveillance. Abdominal CTPancreatic protocol CT is more sensitive than US for evaluating the periampullary region.Magnification endoscopy with narrow band imagingVisualises abnormal vessels on the surface of adenomas/adenocarcinomasMRCP (= 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.ERCPSerum tumor markersCan get increased CA 19-9 and/or carcinoembryogenic antigen (CEA).EUS Useful to clarify biopsy negative uncharacteristic lesions at ampullaEUS+ FNA at ampulla- sensitivity 82 %, specificity of 100 %.Very accurate for T stagingEUS 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 SurveillanceSurveillance endoscopy is indicated every 6m for 2yrs, then annually for 3-5 years.

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