IMAGING AMPULLOMA OF VATER’S PAPILLE

SERIES OF FIFTEEN CASES

YAHDI VICHE, R SAOUAB, J EL FENNI, S. CHAOUIR, T. AMIL, A HANINE, B RADOUANE

Radiology Service Instruction Military Hospital Mohammed V

In collaboration with the visceral surgery service (Sair Pr)

GI8

INTRODUCTION

The ampullomas vatériens = often malignant or benign tumors derived from the intersection area bounded by biliopancreatic tract and the sphincter of Oddi (the last 2cm of the biliopancreatic junction)

Biliary obstruction is early clinical symptoms

The cross-sectional imaging is a great contribution to the diagnosis, staging and monitoring

Early diagnosed , the prognosis is better than pancreatic cancer

OBJECTIVES

To report the clinical and epidemiological aspects of ampullomas vatériens in the series.

Establish the role and limitations of each imaging system.

Describe aspects of imaging ampullomas vatériens.

Discuss the differential diagnosis.

MATERIALS AND METHODS

Retrospective review of records of ampullomas vatériens explored in the training.

During 6 years period [January 2005 - December 2010].

The image system used:- Ultrasound (n: 15)- CT (n: 11)- MRI (n: 4)- Endoscopic retrograde cholangiography (n = 4).

RESULTS 15 cases of ampulla vatériens were detected

Their representation are:- 0.2% of hospitalizations in the department of visceral surgery - 3.4% of digestive cancers (rank 8)

Average age: 68 years [ Between 52ans and 89ans]

9 Males and 6 Female

RESULTS

• Cholestatic Jaundice 100%

• Disorders of transit 46%

• AEG 66.60%

• Abdominal pain 40% • Fever 13%

• Melaena 26.60%

The clinical symptoms were mostly dominated by cholestatic jaundice:

Ultrasound Imaging

RESULTS

ampullary tumor 1 case 6.6%

Expansion of the CBD and IHBD (Fig 1 and 2) 11 cases 91%

Dilation of Wirsung (Fig 3) 2 cases 16.6%

Hydrocholecyste (Fig 4) 2 cases 16.6%

Gallstones 1 case 8.3%

RESULTS

The ampullary tumor was detected in 6 cases:

- Process hypodense bulging through the duodenal wall: 4 patients (36.3%)

- Barrier tissue density of the lower bile duct: 2 patients (16.6%).

Flooding pancreas: 2 cases

Lymph node metastasis: 2 cases

Visceral metastases: 1 patient

CT Imaging

RESULTS

Case 1:Abdominal CT in axial(a),Reconstruction with frontal (b), C +: Tissue process of duodenal papilla, enhanced homogeneously, causing a dilation of a EHBD.

RESULTS

Case 2:Abdominal CT in axial, C +: Dilatation of intrahepatic bile ducts and extrahepatic upstream of a process of lower bile duct tissue coming in contact with the posterior wall of D3

RESULTSCase 3: 40 years old man, obstructive jaundiceAbdominal CT in axial, C-(a, c) / C + (b, d): Tissue mass of the pancreatic duodena's block, is moderately enhancing after injection of Pc and invading the pancreatic head, and he joins in a slight bile duct dilatation (d).ADK poorly differentiated ampullary

RESULTSCase 3: one year laterIncreasing the size of the process with ampullary appearance of liver metastases

RESULTSCase 4: 62 year old man; obstructive jaundice + GI bleedingAbdominal CT in axial, C-(a, c) / C + : Large mass enhanced after injection, bulging into the duodenal lumen and invading the biliopancreatic junction (arrow) with dilatation of upstream bicanalaire (arrow heads)

RESULTS

The ampullary tumor was mentioned in 3 cases

MRI Imaging

Dilatation of the CBD and upstream IHBD a circumferential thickening with stenosis of the lower bile regularly.The main pancreatic duct is not dilated

RESULTS

endoscopy

histology

evolution

Normal appearance of the papilla 2 cases

Mark on D2 2 cases

Appearance ulcerative budding 4 cases

sprouting aspect 2 cases

ulcerated appearance 1 case

Adenocarcinoma in all cases

CPD: 9 cases (Cephalic pancreato-duodenectomy)

Surgery bypass: 5 cases

Endoscopic bypass: 1 case

• Death: 2 cases (5 to J and J 10)• Tumor recurrence: a case (15 months)

• Death: 3 cases (5 months, 6 months and 9 months)

• Death at 4 month

DISCUSSION

1. Choledocho-wirsungo-duodenal junction and sphincter of Oddi:

Headquarters: middle part of D2 at the junction of the posterior and inner surfaces

Variations: Low set, sometimes at D3

The lower part of channels is surrounded by the sphincter of Oddi.

This block sphincter is located at a true dehiscence of the duodenal wall: the "duodenal window." The posterior part is low

Frequency diverticulum at this level

Anatomy

1. Choledocho-wirsungo -duodenal junction and sphincter of Oddi

The system terminal pancreatic duct is less and ventral to the common bile duct

The type of anastomosis of the two channels is variable: common channel (60%); gunmetal at the top of the papilla (38%); separate duodenal anastomosis (2%)

ANATOMY

RadioGraphics 2002; Volume 22 ● Number 6

2.The pancreatic duodenal block :

The pancreas with its reports (after L. Testut, Human Anatomy).

• A, pancreas, has with his head, and B, duodenum, C, jejunum, D, gallbladder;• 1, pancreatic duct, 2, accessory pancreatic duct, the arrow indicates its opening in 2 ', on the posteromedial wall of the duodenum, 3, ampullary, 6, hepatic duct, 7, aorta; 8, mesenteric vessels higher; 9, celiac trunk with three branches.

Anatomy

The ampullary vatérien is a rare tumor: 0.02 to 5% of gastrointestinal tumors

Peak age between 50 and 70 years with slight male predominance

Predisposing factors:

Familial adenomatous polyposis (ampullary adenoma in 50% of cases)

Gardner's syndrome

Van Recklinghausen's disease

The association with cholelithiasis is found in 8-20% depending on the series

Epidemiology

The region tumors vatérienne can develop from the bulb itself or from the duodenal mucosa, pancreas and bladder.

Gross pathology: two types of developmentIntra-duodenal (2/3 of cases): the tumor may be polypoid or vegetative (30%), submucosal (26%) or ulcerated

Intra-papillary (1/3 of cases): strictly localized to the ampulla of Vater

Microscopy:ampullary tumors are malignant in 95% of cases dominated by adenocarcinomas

Pathology

Sprouting aspect of the papilla at endoscopy performed in one patient in our series

Pathology• TNM Classification: UICC 2002

T Primary tumorTx Tm primary can not be demonstrated

Tis Tm intraepithelial or lamina propria

T1 Tm limited to the ampulla of Vater or sphincter of Oddi

T2 Tm invading the duodenal wall

T3 Tm invading the pancreas 2 cm or less

T4 Tm invading the pancreas more than 2 cm and / or adjacent organs

N Extension node

Nx Regional lymph nodes unproven

N1 Absence of metastasis in regional lymph nodes

N2 regional lymph node metastases: peri-pancreatic, pyloric, proximal mesenteric, cystic, pericholedochal.

M Distant metastasis

M1 Absence of distant metastasis

M2 Liver metastases, peritoneal, lymph nodes of the tail of the pancreas and / or spleen.

T1 (a)Tm limited to the ampulla of Vater or sphincter of Oddi

T2 (b)Tm invading the duodenal wall

T3 (c)Tm invading the pancreas 2 cm or less

Q4 (d)Tm invading the pancreas more than 2 cm and / or adjacent organs

PATHOLOGICAL ANATOMY• TNM Classification: UICC 2002

The obstructive jaundice: it is the sign most frequently revealing and often

constant, found in 70-80% of cases

GI bleeding: Sx evocative but inconstant (6% of cases), melaena, anemia

Other: abdominal pain, transit disorders, IGC

Clinical

Review of first-line before a cholestatic jaundice

Interest:

Confirm the dilated bile ducts in 100% of cases with hydrocholecyste Specify the

level of obstruction in 90% of cases

View ampullary tumor in 25% of cases especially if the tumor size> 2 cm

To identify liver metastases

Limits:

Tumors <2 cm

The nodal

The interposition gas or obesity + + +

Imagery1.Échographie:

Technical:

Use of a transducer rotating scanning high frequency.

In recent years, development of mini probes of 2 mm diameter and high frequency (20MHz) Possibility of retrograde catheterization of the bile and pancreatic ducts and Possible distinction between the sphincter of Oddi and duodenal mucosa in

NB: the risk of nodal involvement is zero in case of tumors limited to the sphincter

Mini probe intra ductal (1: sphincter of Oddi).

Imagery2.Echoendoscopie:

NB: the risk of nodal involvement is zero in case of tumors limited to the sphincter

Mini probe intra ductal (1: sphincter of Oddi).

Interest:The visualization of the tumor vatérienne in 90 to 100% of casesSuperior sensitivity than other imaging techniques for evaluation of: - The extension of the tumor (T): if malignancy crossing the fourth hypoechoic layer of the duodenal wall (muscularis) - The nodal (N): diagnostic accuracy of 68 to 76% for stage N1 - The invasion of the vein axis mesocaval door with a sensitivity of 91% and a specificity of 97%

Indications:Suspicion of pathology with an ampullary OGDF a cross-sectional imaging and inconclusive Assessment of preoperative extension of ampullary tumors proven choice of TRT (surgical or endoscopic)

Imagery2.Echoendoscopie

Importance: Sensitivity of 85 to 90% in case of biliary dilatation and specificity of 90%.Technical: Acquisition helical thin sections

Ingestion of water + + +Study with and without injection of the PC in arterial and portal venous phase (with 100cc flow 3cc/sec)Results: Positive diagnosis:Turgid appearance of the papilla or hypodense heterogeneous process bulging into the duodenal lumenThe dilated bile ducts inside and outside the liver associated with dilatation of the pancreatic duct is highly suggestive of the diagnosis

Extension: The pancreas, lymph node, peritoneal or hepatic vein thrombosis

Imagery3.TDM:

Interest: better contrast resolution and multi planar study

Technical: morphological sequences: axial acquisitions SPT1 and FAT-SAT GADO T1, T2 Sp coronal acquisition, 4mm thick Sequence diffusion and Bili-sequence MRI

Results: MRI allows visualization of the ampullary tumor in 93% of cases: Small polypoid lesion, iso or hypo T1 and T2, weakly or moderately enhanced after injection protruding into the duodenal lumen Sometimes, a simple engorgement of the papilla Irregular thickening of the biliopancreatic junction

The bili-MRI appreciate the topography and the length of the obstacle. Frank said in a ruling "pellet shells" referred to the diagnosis.

ImageryMRI

Images evocative:

The gap tumor protruding into the duodenal lumen.The classic image epsilon (sign Frosberg or "reverse 3") ulceration within a tumor proliferation.

Images nonspecific:

The irregular stenosis of the duodenum by discussing the second duodenal cancer;Expansion of the duodenum;Printing bulbar post a bile duct dilatation

Imagery

a. UGI:

5. Other

It allows:

To objectify stricture or bile duct or ampullary gap in intra papillary forms that go

unnoticed at duodenoscopy.

To complete the review by a wirsungographie possible.

Imagery

5.Other:

b. Cholangiographie Retrograde Endoscopic (ERCP)

Neoplastic causes: Cancer of the pancreas head The lower bile duct Cholangiocarcinoma Cancer duodenal

Non-neoplastic causes: Lithiasis of the CBD; Barrier parasite: cyst, roundworms or flukes. Sclerosing cholangitis;Pancreatitis Inflammatory stenosis of the bile duct. Sphincter of Oddi dysfunction: about 5% of patients suspected of having a DSO have an ampullary; Diverticulum juxta-ampullary: lithiasis and thus promotes misdiagnosis. Benign papilla papilla 'forced' migration after gallstone.

Differential Diagnosis

a. Carcinome pancreatic:

pancreatic mass: hypovascular, often with an infiltrative lymph node status.

The expansion bicanalaire qq with special features:Sx of four segments: visualization of biliopancreatic channels upstream and downstream of the tumor

The pancreatic duct dilatation secondary

Differential Diagnosis1.Cancers peri-ampullary:

b. Cholangiocarcinome:

Irregular thickening of the bile duct wall or

intraluminal polypoid mass

The distal common bile duct is often visible sign

3 segments (2 segments of the bile duct +

pancreatic duct non-dilated)

Differential Diagnosis1.Cancers peri-ampullary:

c) duodenal Cancers:

Uncommon tumor

It can be polypoid, ulcerated or infiltrative

Lymph Nodes in 22-71% of cases

The duct dilatation biliopancreatic is

moderate or absent

Differential Diagnosis

1.Cancers peri-ampullary:

Papillary epithelial proliferation, benign or malignant mucin-producing ductal dilatation Peak age of 60 years with male predominance

Imaging: papilla large (> 10 mm) with multicystic dilatation of the pancreatic duct and mural nodules

Differential Diagnosis 2. Papillary inflammation:

Multiple causes: passage + + + gallstones, cholangitis, pancreatitis or acute infectious (parasitic)

Swollen appearance of the papilla with homogeneous enhancement

3. Tumeur intra ductal papillary mucinous pancreas (IPMT):

Differential Diagnosis4. Other: choledochal cyst

Intraoperative view after a duodenotomy cholédochocèle, the ampullary ≠ vatérien

Subsidence choledochal cyst after dilation of the papilla.

(Iconography of surgery visceral I)

The treatment of choice remains wide surgical excision CPD type (cephalic pancreatico-duodenectomy) unless otherwise-cons:

Vascular invasion.

Remote node metastases (<5%)

Métastases liver (5-10%)

Peritoneal carcinomatosis

Treatment

Duodenopancretactomie cephalic part. (Iconography of surgery visceral I)

The endoscopic ampullectomy Tm small ampullary benign or malignant is not invading the submucosa duodenal

Endoscopic sphincterotomy diagnostic, therapeutic preoperative or palliative therapy

Biliary drainage + / - stent grafts: Tm locally advanced

Radio-chemotherapy: adjuvant TRT after surgical resection or as palliative

TreatmentOther methods:

The prognosis is better compared to other peri-ampullary cancers.

It is mainly related to nodal involvement.

The prognosis is greatly improved by early treatment attitude and thoughtful.

The average survival to 5 years is directly related to tumor stage and nodal

involvement.

Evolution and prognosis

The ampulloma vatérien is a rare tumor, often malignant.

Always think before a cholestatic jaundice + GI bleeding.

Ultrasound is the first review confirm biliary obstruction and determine the

level of obstruction.

CT, MRI with MRI-Sq Bili are fundamental for the diagnosis and staging.

The echo-endoscopy is a great thing if the cross-sectional imaging is inconclusive

Conclusion