Ampullary Tumors

Edward Chin, MD
February 3, 2005

Case
87M with scc of mouth
S/p R radical neck, partial glossectomy, mandibulectomy,
alveoloplasty, flap coverage 94
2001: elevated LFTs
U/S: intra & extrahepatic biliary dilatation, dilated CBD
CT: same
2003: CBD stones on U/S, CT
EGD ampullary mass, bxd
tubulovillous adenoma w/ foci of high-grade dysplasia

Case

MRCP not feasible (oral hardware)

ERCP, EUS declined by patient
2/1/05: N/V, RUQ pain
Afebrile, 60/40, 100
RUQ tenderness
WBC 19, ALT 87, AST 209, GGT 232, alk
phos169, bilirubin 4.6/2.7, amylase/lipase nl
CT

Case

CT: dilated CBD (2.6 cm) with stones

Dilated intra & extrahepatic ducts
Dilated pancreatic duct
IVF, antibiotics, ICU
GI consult ERCP today

Ampullary Tumors
Periampullary tumors:

pancreatic
duodenal
CBD
ampulla

1o ampullary tumors:
Rare: 1 per 6 million
Benign & malignant
w/ FAP, HNPCC

Ampulla of Vater

Consists of 3 epithelia (CBD, PD, duodenal mucosa)

Type I: (70%): formed by union of PD, CBD
Type II: (20%) biliopancreatic junction forms close to papilla; short
ampulla
Type III: CBD & PD enter separately
Type IV: BPJ separate from papilla

Ampullary Tumors
Benign:
adenomas, lipoma, hamartoma, lymphangioma,
hemangioma, neurogenic tumors

Adenomas

Tubulovillous (40%)
Villous (30%)
Tubular (10%)
Non-epithelial (20%)

Malignant transformation:
adenoma dysplasia carcinoma?

Ampullary Tumors
Favorable (vs. periampullary)
Resectability > 90%
5-year survival 30-50%

K-ras mutation (37%)

p53 mutation
CA 19-9, CEA elevated

Presentation
Ampullary cancers:
80% with jaundice
Adenomas (sporadic)
Typically older patients (70s)
Non-specific symptoms (mild pain, fullness, N/V, anemia, GI bleeding)
Jaundice 50-75%
Cholangitis, pancreatitis rare
CBD stones seen in 25%

Diagnosis
U/S, CT: indirect evidence (ductal dil, CBD stone)
EUS: good for staging (T > N)

Cholangiography
ERCP, MRCP, PTC for diagnosis
Intraductal extension
Indurated, rigid papilla,
ulceration seen w/ ca
Biopsies lack sensitivity
False negative < 50%
~15% of biopsies w/ adenoca
OR pathology: 30-60%

Staging
5-year survival:
Stage I: 84%
Stage II: 70%
Stage III: 27%

LN Spread

J Hepatobil Pancreat Surg 2004. 11: 232-38

Surgical Options
Whipple procedure (pancreaticoduodenectomy)
Low mortality in large centers (< 5%)
Best survival (> 50% 5-year)
High morbidity (25-65%)

Results for Whipple

J Hepatobil Pancreat Surg 2004. 11: 232-38

Survival

J Hepatobil Pancreat Surg 2004. 11: 232-38

Prognostic Factors

Lymph node involvement

Local spread adjacent organs (e.g. pancreas)
Tumor grade/differentiation
Positive margins

Transduodenal Excision (TDE)

1st described by William Halsted, 1900
Less morbid (5-20%)
Less successful (recurrence, survival )

Ulster Med J 2002. 71(2): 121-27

TDE Results
Lack of high-quality studies
Largest: German study (n = 41)

All were adenomas (2/3 with dysplasia)

Routine frozen section
5/41 whipple for cancer
No recurrences at 42 months
Ann Oncol 1999. 10 suppl 4: 212-4

TDE
26 patients underwent TDE (Duke)
Adenoca (n= 8)

5 died of recurrent/metastatic disease

1 died, unrelated
1 recurrence, survivor
1 disease-free survivor (f/u of 21 months)

Ann Surg 1996. 224(5): 621-27

Duke, TDE
Adenomas (n=18)
5 complications
5 recurrences
Mean time = 34 months
2 with Gardners syndrome
4/5 treated endoscopically, 1 whipple

No deaths
Ann Surg 1996. 224(5): 621-27

Radical vs. Local Surgery

Considerations:
Tumor stage (T1, T2)
Patient condition

TDE indications:
Adenomas with HGD (ca in < 30%)
Large villous, TV adenomas (> 2 cm)
Low-grade, stage tumors w/ comorbidities
J Hepatobil Pancreat Surg 2004. 11: 239-44

More Data
88 patients with ampullary tumors
94% underwent surgery
92% curative resection performed
89% whipple, 3% local excision

Br J Surgery 2004. 91(12): 1600-07

Results
0 deaths
39% morbidity
88% malignant on
pathology
12% benign adenomas
Med survival 42 months
vs. 8 months unresectable
Br J Surgery 2004. 91(12): 1600-07

Prognostic Factors
LN spread
Resectability
Elevated bilirubin

Endoscopic Therapies
Snare resection
Laser ablation
Photodynamic irradiation

Endoscopic Snare Excision

Criteria:

Adenomas < 4 cm
Absence of strictures or extension duct
No ulcers
Benign results of > 6 biopsies

No mortality, but morbidity (10-27%)

bleeding
pancreatitis

Recurrence variable (5-25%)

Adjuvant Therapy
Investigational
Chemotherapy (5-FU)
+/- XRT (45 Gy)