Ampullary Carcinoma Flashcards
(14 cards)
What is periampullary tumors?
Neoplasms arising in the vicinity of ampulla of Vater (pancreas, duodenum, distal CBD), or the structures of the ampullary complex.
Classification of bile duct dilatation
What is ampullary carcinoma?
Neoplasms arising within the ampullary complex, distal to the confluence of the distal common bile duct and the pancreatic duct.
Distinction between ampullary and periampullary cancers is not essential preoperatively since the treatment is the same for both lesions
Epidemiology of Ampullary ca.
- rare disease with an incidence of < 1 per 100 000 per year
- 0.6%–0.8% of digestive cancers,
- male to female ratio of 1.5
- 50–70 yrs
- majority Sporadic.
Etiology of Ampullary ca.
- Associated with – FAP, HNPCC, Neurofibromatosis type I (not only for somatostatinomas but also for carcinoma), Muir–Torre syndrome
- Two main histological subsets of precursor lesions can arise from intestinal-type mucosa as well as from pancreatic duct-type ampullary mucosa.
Histological subtypes of Ampullary ca.
Two main subtypes:
- Intestinal type evolves through adenoma–carcinoma sequence (better outcome).
- Pancreaticobiliary type evolves from precursor pancreatic duct intraepithelial neoplasia → adenoma → intraepithelial neoplasia (dysplasia and carcinoma in situ) → adenocarcinoma (worse outcome).
Other variants
- Mixed-type (glandular and squamous cell components),
- Mucinous (colloid),
- Signet-ring cell carcinomas,
- Neuroendocrine,
- Undifferentiated carcinomas
Presentation of Ampullary ca.
- Obstructive Jaundice (80%),
- weight loss (61%),
- Abdominal pain, & back pain (46%)
- Pancreatitis (4.1 %)
- Cholangitis (1%)
- Steatorrhea
- UGIB
- Large lesions – intestinal obstruction
Investigation for Ampullary ca.
General Features
- Soft tissue mass involving ampulla
- “Double duct” sign with obstruction of both common bile duct (CBD) and pancreatic duct (PD)
- Location - Within ampulla of Vater or overlying periampullary duodenal mucosa
- Size - 1-4 cm in diameter; mean 2.7 cm
1) USG
- 1st line
- Accuracy 15%
- ampullary mass usually not visibile
2) CECT
- Accuracy 20%
- Pancreatic mass protocol type of CT
- Water as the oral “contrast agent” (to distend the duodenum and improve visualization of the duodenal lumen and adjacent pancreas), and intravenous contrast is injected as a bolus to permit both arterial- and venous-phase imaging.
3) ERCP
- Accuracy 88%
- Jaundiced patient with suspected malignant bile duct obstruction, ERCP is the preferred initial endoscopic study - biopsy, decompression.
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- Benign small ampullary adenomas can be indistinguishable from normal papilla.
a) Benign- Regular surface/margins, soft appearance, and mobility.
b) Local invasion features - exophytic mass, ulceration, firmness, spontaneous bleeding or friability, depressed component, non lifting of laterally spreading lesion.
- Obstruction of CBD and PD.
- Cannot determine the extent of local tumor invasion of an ampullary carcinoma into the adjacent duodenum or pancreatic parenchyma, information that is essential for preoperative staging and surgical planning.
4) Indigo carmine Chromoendoscopy and NBI
- To differentiate benign from neoplastic lesions, in context of irregular villous arrangement and abnormal microvasculature, diagnosed adenocarcinoma with sensitivity (69%), specificity (100 %), positive predictive value (100 %), negative predictive value (85 %), and accuracy of (89 %).
5) MRCP
- Up to 76% accuracy
- If contraindication to ERCP (e.g. Roux-en y)
- Dilated PD and CBD, appear as filling defects protruding into duodenal lumen.
6) EUS
- Most accurate modality for T staging sensitivity (77%) and specificity (78%) and guided fine-needle aspiration/ biopsy (EUS-FNA/B) - sensitivity (82.4%), specificity (100 %), and accuracy (88.8%).
- Detection of nodal metastases, sensitivity (70%) and specificity (74%).
7) Intraductal ultrasonography
- for T-staging in ampullary tumors has been reported to have overall accuracies between 78% and 90.2%
- IDUS could also be useful to guide direct tissue acquisition by biopsy or brush cytology
TNM staging for ampullary ca.
Management of Ampullary ca.
Preoperative biliary drainage reserved for patients with:
- cholangitis
- severe symptomatic jaundice (e.g., intense pruritus),
- delayed surgery
- for before neoadjuvant chemotherapy in jaundiced patients.
When required, ESGE recommends endoscopic biliary drainage with endoscopic SEMS insertion.
Role of Surgery in Ampullary ca.
- Complete tumor resection with negative margins (R0 resection) is a prerequisite for cure.
- ESGE recommends en bloc resection of ampullary adenomas up to 20–30mm in diameter to achieve R0 resection.
- Pancreaticoduodenectomy (Whipple operation) or PPPD for stage T1 or higher– curative 90%.
role of adjuvant therapy
Post OP chemo – Not shown to be of advantage. Not included in NCCN, ESMO.
Palliative. for Ampullary ca.
- Minimally invasive nonsurgical therapies for ampullary carcinoma include endoscopic snare resection, Nd:YAG laser ablation, and photodynamic therapy – for non operative candidates.
- ERCP with self-expandable metal stent (SEMS) insertion in patients with ampullary tumors and biliary obstruction in palliative settings.
- European physicians offer stage T2N0 or higher. Treated similar to pancreatic head CA – ESPAC-1 trial Fluorouracil chemo prolongs survival.
- American Physicians - chemoradiotherapy as well as adjuvant chemotherapy
Follow up for Ampullary ca.
- After endoscopic papillectomy or surgical ampullectomy, duodenoscopy with biopsies of the scar and of any abnormal area, within first 3 months, at 6 and 12 months, and thereafter yearly for at least 5 years.
- Recurrence after endoscopic papillectomy endoscopic examination and biopsies, and EUS and MRCP investigations before any treatment.
