CASE PRESENTATION:

Dr.Amra Farrukh
PG.T SU.I
Pt,s Profile

NAME maqsooda
AGE 65 years
MARITAL STATUS married
ADDRESS hazroo tehsil Attock
MOA ER
DATE 06/10/10
PRESENTING COMPLAINTS

Yellowish discoloration of the eyes and body for 1.5

months

Pain RHC and epigastrium for last 3 days

HOPI

-Yellowish discoloration of eyes and body

-Progresive worsening of discoloration

-Clay color stools and dark colored urine

-Itching on body and pain RHC

-Significant weight loss

PAST HISTORY

No significant surgical or medical history

DRUG HISTORY

Unremarkable
PERSONAL HISTOR Married
No children
Non smoker non addict
FAMILY HISTORY Insignificant
SYSTEMIC REVIEW

CVS
No c\o SOB,chest pain,pedal edema.
CNS
No complaints.
RESP.
No c/o chest pain SOB,or cough.
GIT.
H/o clay coloured stools,anorexia,jaundice
No c/o melena or haemetemesis.
GPE

-Pulse 85/min
-B.P 110/70 mmHg
-TEMP afebrile
-R/R 14/min
-yellowish discoloration of body
-Patient was jaundiced
Systemic Examination:

CVS
Unremarkable.
CNS
Intact.
RESP.
Normal vesicular breathing.
GIT.
No visible pulsations,scar marks.
Soft abdomen,minimally tender in RHC
No mass or swelling palpable
Shifting dullness,fluid thrill absent
Provisional Diagnosis:

Obstructive Jaundice

Malignancy
Gall stones
Investigations:

BCP
Hb 10g\dl
TLC 10,000
Plt 346

PT/APTT
Normal
Conti..............

RFT,s
WNL
RBS
WNL
LFT,s
Total billi 23mg/dl
Alt 113 IU/l
Alk Po4 1140 U/l
ULTRASOUND ABDOMEN

Grossly distended G.B

Wall thickness 3 mm

CBD 9 mm
C.T Scan

Ill defined hypodense lesion with differential post

contrast enhancement in periampullary region
SIZE : 2x2x1 cm
Laterally indenting the 2nd part of duoidenum
Postero medially compressing the IVC.
Anteriorly it is interfaced with gut loops
C.T Scan

CONCLUSION: Enhancing peri ampullary lesion

with grossly distended CBD,gall bladder and
intrahepatic cholestasis is suggestive of peri
ampullay carcinoma.
Final Diagnosis:

PERI AMPULLARY CARCINOMA

Pancreatoduodenectomy (Whipple
procedure)

19/10/2010
Peri ampullary mass not involving blood vessels and
adjacent structures.
Grossly distended CBD and G.B.
Lymph node around hepatic artery just proximal to
gastroduodenal artery.
Lymph node enlarged in the mesentry of jejunum.
Anastamosis

Pancreatico jejunal
Choledochojejunal
Gasstro jejunal
Feeding jejunostomy
Post op course:

Pt remained vitally stable

No blood transfusion needed
Abdomen remained soft
Feeding started on 2nd day
Post op labs:

DATE Hb TLC Total Billi. Alk.Po4

19.10.2010 9.1 11.2 17.0 -

22.10.2010 10.0 13.6 12.1 -

24.10.2010 10.8 10.4 8.6 -

25.10.2010 12.1 12.4 10.3 -

27.10.2010 11.3 10.4 8.8 -

28.10.2010 11.4 9.9 8.4 -

29.10.2010 10.7 9.8 9 -

30.10.2010 10.5 9.4 9 -

Histopathology

Ampulla of Vater
3.5x3.5x1 cm
Adenocarcinoma
Poorly differentiated
PT3 invading underlying pancreas
Conti.............

Lymph nodes recovered 7

L.N involved Nil
Circumferential margin involved
Immunohistochemistry:

CK7 +ve
CEA +ve
CA19-9 -ve
Discharge and Followup
 LITERATURE REVIEW
AMPULLARY CARCINOMA
Ampuula of Vater:

The ampulla of Vater, also known as the hepatopancreatic ampulla,

is formed by the union of the pancreatic duct and the
common bile duct.
The ampulla is specifically located at the major duodenal papilla.
The Ampulla of Vater is an important landmark, halfway along the
second part of the duodenum.
It marks the anatomical transition from foregut to midgut (and hence
the point where the celiac trunk stops supplying the gut and the
superior mesenteric artery takes over).
Periampullary Region:
The periampullary region is anatomically complex,



Represents the junction of 3 different epithelia.
-pancreatic ducts
- bile ducts
- duodenal mucosa.

Grossly, carcinomas originating in the ampulla of Vater can
arise from 1 of 4 epithelial types:

(1) terminal common bile duct,

(2) duodenal mucosa, (3) pancreatic duct, or (4) ampulla of
Vater
AMPULLARY CARCINOMA
Carcinoma of the ampulla of Vater is a rare malignant tumor
arising within 2 cm of the distal end of the common bile duct,
where it passes through the wall of the duodenum and
ampullary papilla.
EPIDEMIOLOGY:

Carcinoma of the ampulla of Vater is an uncommon tumor.

Ampullary cancer now accounts for approximately 0.5% of all
gastrointestinal tract malignancies.
The incidence has been increasing since 1973 at an annual percentage
rate of 9%.

Ampullary cancer accounts for some 7% of peripancreatic

tumors
ETIOLOGY:

The etiology of the disease is poorly understood.

Patients with ··familial adenomatous polyposis (FAP) have an
increased risk of both benign and malignant ampullary tumors.
As many as 50-90% of patients with FAP develop duodenal
adenomas, predominantly concentrated on or around the major papilla.
K-ras mutations may be a factor.
Microsatellite instability is associated with a better prognosis.
Chromosome 17p and 18q loss of heterozygosity are associated
with ampullary carcinoma
PATHOPHYSIOLOGY
Distinguishing between true ampullary cancers and periampullary tumors is
critical to understanding the biology of these lesions.

Each type of mucosa produces a different pattern of mucus secretions.

sulphomucins and

Sialomucins;

In general, ampullary cancers produce sialomucins, whereas periampullary

tumors secrete sulfated mucins. These researchers demonstrated that
ampullary tumors secreting sialomucins had a better prognosis (100% vs 27%
5-y survival rate.

Other investigators have confirmed the prognostic power of the pattern of

mucin secretion.
TUMOR MARKERS:
Immunohistochemical stains for expressions of carcinoembryonic
antigen (CEA), carbohydrate antigen (CA) 19-9, Ki-67, and p53
have been studied for prognostic power.
In a series of 45 patients, expression of CA 19-9 labeling intensity
and apical localization both were statistically significant predictors
of poor prognosis.
The 5-year survival rates were markedly different between tumors
that expressed CA 19-9 and those that did not (36% vs 100%)
CEA expression also might be a marker for prognosis, but it is
much weaker.
Ki-67 and p53 were not demonstrated to have an effect on
outcome.
Imaging Investigations:

C.T SCAN:
Increased targetlike enhancement of the papilla is likely to represent a
benign condition such as papillitis.
whereas an enhancing polypoid mass or focal asymmetric or irregular
thickening with prolonged enhancement in the ampulla of Vater
indicates a malignant condition such as ampullary or periampullary
carcinoma
Laparoscopy/Laparoscopic USG
Previously been shown to be an effective tool in the staging of pancreatic and
ampullary carcinomas.
Being more predictive of resectability than abdominal computed tomography.
With the combined use of CT scanning and LUS, unresectable disease is found
in 35–54% of patients

British Journal of Cancer (20200606) 94, 213–217. doi:10.1038/sj.bjc.6602919

Published online 24 January 2006
Evaluation of EUS versus CT scan for staging
of ampullary cancer.

EUS is an accurate diagnostic test and exhibits a high level of

agreement with surgical pathology.

(Gastrointest
Endosc 2009;
Gastroenterology. 1992 Jan;102(1):188-99.
Staging of pancreatic and ampullary carcinoma by endoscopic
ultrasonography. Comparison with conventional sonography,
computed tomography, and angiography

In a prospective study, endoscopic ultrasonography was compared with

transabdominal ultrasonography, computed tomography, and angiography
in 60 consecutive patients with pancreatic and ampullary cancer .
The diagnostic value of these imaging procedures in determining local
resectability was assessed
endoscopic ultrasonography was significantly superior to abdominal
ultrasonography and computed tomography in determining tumor size and
extent and lymph node metastases of pancreatic and ampullary cancer.
Furthermore, involvement of the portal venous system as judged by
histopathology or surgical exploration was correctly assessed by
endoscopic ultrasonography in 95%, whereas angiography (85%),
computed tomography (75%) and abdominal ultrasonography (55%) were
less sensitive
Staging of Ampullary cancer

Martin proposed a 4-stage system, as follows:

Stage I - Vegetating tumor limited to the epithelium with no
involvement of the sphincter of Oddi
Stage II - Tumor localized in the duodenal submucosa without
involvement of the duodenal muscularis propria but possible
involvement of the sphincter of Oddi
Stage III - Tumor of the duodenal muscularis propria
Stage IV - Tumor of the periduodenal area or pancreas, with
proximal or distal lymph node involvement
The currently accepted American Joint Committee on Cancer
staging system for ampullary carcinoma emphasizes the importance
of pancreatic invasion and lymph node metastases.
Size has little impact on tumor stage
The currently accepted American Joint Committee on Cancer
staging system for ampullary carcinoma emphasizes the importance
of pancreatic invasion and lymph node metastases.
Size has little impact on tumor stage
TNM Staging
Primary tumor
TX – Primary tumor cannot be assessed
T0 – No evidence of primary tumor
Tis – Carcinoma in situ
T1 – Tumor limited to ampulla of Vater
T2 – Tumor invades duodenal wall
T3 – Tumor invades less than 2 cm into pancreas
T4 – Tumor invades more than 2 cm into pancreas or other organs
Conti.............

Regional lymph nodes

NX – Regional lymph nodes cannot be assessed
N0 – No regional lymph node metastases
N1 – Lymph node metastases
Distant metastases
MX – Presence of distant metastases cannot be assessed
M0 – No distant metastases
M1 – Distant metastases
STAGING:

Stage T N M

Stage 0 Tis N0 M0

Stage I T1 N0 M0

Stage II T2-3 N0 M0

Stage III T1-3 N1 M0

Stage IV T4 N0-1 M0

… T1-4 N0-1 M1
Defination of vascular involvement
assesed by C.T Scanning.
Grade of Defination
Resect.
D Hypodense tumor is inseperable from
adjacent vessels,point of contact forms a
concavity against the vessel or partially
encircles the vessels.
E Hypodense tumor encircles adjacent
vessels, no fat plane is identifiable b/w
tumor and vessels.
F Tumor occludes the vessels.
Defination of vascular involvement
assessed by C.T scanning
Grade of Defination
Resectability
A Fat plane seperates the tumor from
adjacent vessels.
B Normal parenchyma seperates the
hypodense tumor from adjacent vessels
C Hypodense tumor is inseperable from
adjacent vessels and point of contact
forms a convexity against the vessel.
British Journal of Cancer (20200606) 94, 213–217. doi:10.1038/sj.bjc.6602919
Published online 24 January 2006
C.T grading for vascular involvement
This grading system examines the relationship between the tumour and the
major vessels
-superior mesenteric vein
-portal vein
-superior mesenteric artery
-hepatic artery
Patients with grade A to D tumours are considered potentially resectable.
while those with grade E or F tumours are invariably not resectable.
In addition a grade O was added to this grading system for those patients with
pancreatic and biliary duct dilatation without the presence of a pancreatic mass.
Options for Ampullary cancer.

Pancreaticodudenectomy
Local excision
Palliative

The standard surgical approach is pancreaticoduodenal resection

(Whipple procedure).
The procedure involves en bloc resection of the gastric antrum and
duodenum; a segment of the first portion of the jejunum, gallbladder,
and distal common bile duct; the head and often the neck of the
pancreas; and adjacent regional lymph nodes
Prognosis of Whipple in Peri-
Ampullary C\A Study Annals of surg
The tumor-specific 10-year actuarial survival rates were
- Pancreatic 5%
-Ampullary 25%
- Distal bile duct 21%
-Duodenal 59%

Among patients with periampullary adenocarcinoma treated by

pancreaticoduodenectomy, those with duodenal adenocarcinoma are most likely
to survive long term.
Five-year survival is less likely for patients with ampullary, distal bile duct, and
pancreatic primaries, in declining order
Prognosis of Whipple for Ampullary
Cancer
Results after radical resection of ampullary of Vater carcinoma
have been improving.
During the past decade, 5-year survival rates have ranged from 20-
61%, averaging higher than 35%.
The reported mortality rates from this operation are decreasing
Local Excision for Ampullary cancer
Because of the mortality and morbidity associated with
pancreaticoduodenectomy, physicians have been interested in performing local
excisions of cancers of the ampulla of Vater to avoid a major resection.
Transduodenal excision of ampullary tumors has been proposed as an
intermediate option between radical resection and palliative bypass for high-risk
patients.
Some have argued that this approach is simpler, is better tolerated, and might
provide a comparable cure rate (mortality rate 8-13%, 5-y survival rate 0-43%).
This approach generally has been reserved for poor operative candidates (eg,
elderly patients, those with other comorbid conditions) with favorable tumors
(generally <2 cm, polypoid). Unfortunately, this approach compromises local
control
Whipple and Local resection
The 5-year survival rate reported after Whipple's resection for ampullary
cancer varies from 22% to 55%..
On the other hand, pancreaticoduodenectomy has been reported to result
in morbidity of 43% and mortality of 11%.
This fact has led to interest in local resection of ampullary tumors.
After local resection, it has been reported that the mortality rate reaches
7.1% and the 5-year survival rate 35%
Although there are many case reports and a few series on the treatment
of ampullary neoplasms by local ampullary resection, the criteria used to
decide when local excision is suitable for certain patients are
controversial, and not well addressed.

Int Semin Surg Oncol. 2005; 2: 16.

Published online 2005 August 30

Indications of Local excision
Bottger et al stated that the indications for local excision should be that
-The tumor is completely removed (R0),
-Limited to the ampulla of Vater (pT1),
-Not poorly differentiated and
-With no venous/lymphatic infiltration in patients with
ASAgrade IV, regardless of their age

Int Semin Surg Oncol. 2005; 2: 16.

Published online 2005 August 30

Study: comparison of whipple and
local excision

From 1990 to 1999, 205 pts diagnosed with periampullary C/A.

32 of these patients that proved to have carcinoma of the ampulla of
Vater, and underwent surgical treatment.
Pancreaticoduodenectomy was the first choice as the type of surgical
treatment.
Local resection was the preferable treatment when the ampullary lesion
was less than 2 cm in diameter, the pre-operative biopsy showed a pT1
cancer or adenoma of the ampulla of Vater and/or the patient's
concomitant medical illness or age contraindicated a major operation
such as Whipple's procedure.
Int Semin Surg Oncol. 2005; 2: 16.

Published online 2005 August 30

Study: Comparison of whipple and
local excision
Survival after local resection has been reported to be 40% to 50% at 5
years.
This figure is comparable to 37.5% to 62.7% 5-year survival rate
reported in pancreaticoduodenectomy series.

Int Semin Surg Oncol. 2005; 2: 16.

Published online 2005 August 30.

Technique of local Excision:

-The abdomen is explored through a subcostal or midline incision.

-After a Kocher maneuver for the mobilization of the second part of
the duodenum, the latter is opened by a 4–5 cm "antimesenteric"
longitudinal incision.
- Stay sutures are placed in the duodenal wall circumferentially.
-Bile and pancreatic duct are canulated with a Fogarty catheter.
-Then, the normal duodenal mucosa surrounding the ampullary tumor
is injected with saline containing 1 to 100,000 epinephrine
Conti..............

Once the identification of the ducts had been accomplished, a circumferential

resection of duodenal mucosa to a depth necessary to excise the tumor is
undertaken.
Margins of 1 cm are obtained in all directions beyond the gross border of the
lesion, in order to obtain free margins resection.
Because bile and pancreatic ducts were transected a reconstruction procedure
is essential to ensure adequate billiary and pancreatic drainage and to
repair the transduodenal defect.
Reconstruction is accomplished by approximating the common walls of the
pancreatic and bile ducts that eventually are sutured together on the
duodenal wall
Conti...............

-The ducts are probed with billiary dilators to ensure appropriate size.
-A diameter of 6 to 8 mm for the bile duct and 4 to 5 mm for the
pancreatic duct are obtained, assuming that scarring will reduce
these diameters by 50%.
- After the establishment of an adequate duct patency the duodenotomy
is closed transversely.
OPERATIVE FINDINGS:sch
Findings contraindicating resection

Liver metastases (any size)

Celiac lymph node involvement

Peritoneal implants

Invasion of transverse mesocolon

Hepatic hilar lymph node involvement

Findings not contraindicating resection

Invasion at duodenum or distal stomach

Unresectable Disease.
If metastatic disease or unresectable local vascular invasion is detected
during staging then jaundice can be palliated by endoscopic or
radiological biliary stenting.
survival is similar following surgical bypass or biliary stenting for the
relief of jaundice and this is comparable with the findings of a meta-
analysis of three randomised control trials.
For patients with unresectable disease, the presence of distant metastases
or advanced local disease may alter the palliative options.

British Journal of Cancer (20200606) 94, 213–217. doi:10.1038/sj.bjc.6602919

Published online 24 January 2006

.
Unresectable Disease
For patients with unresectable disease, endoscopic stenting to
achieve biliary decompression is an appropriate palliative
procedure.
No established answer exists to the question of further therapy.
Very little has been published on adjuvant treatment for locally
advanced and advanced ampullary carcinoma.
Confining one's approach to relief of symptoms is reasonable.
Biliary-enteric bypass to palliate unresectable pancreatic cancer. (Reproduced with permission from Bell, Rikkers, Mulholland (eds): Atlas of
Pancreatic Surgery, 1st ed. Philadelphia: Lippincott, Williams & Wilkins, 1996.)
Radiotherapy
Pancreaticoduodenectomy is the procedure of choice for patients
with resectable disease
but local recurrence plagues all surgical series, particularly when
the pancreas has been invaded or lymph node metastases are
discovered.
In fact, whether major resection impacts survival in the setting of
disease spread to the lymph nodes remains unclear.
Postoperative irradiation of at least 45 Gy with 5-FU as a
radiosensitizer is a reasonable treatment and reduces local
recurrence.
Cisplatin Plus Gemcitabine Improves Survival of Patients
with Advanced Ampullary cancer.

Compared to gemcitabine alone, the chemotherapy combination of cisplatin

and gemcitabine improved overall survival of patients with
locally advanced or metastatic ampullary cancer.
The drugs were given by intravenous infusion for 12 weeks and patients’
tumors were then evaluated by imaging.
The combination chemotherapy also improved progression-free survival.
CONCLUSION: While there has been no single standard of care for advanced
ampullary cancer.
This is the closest we have to a gold standard.
Physicians can now feel confident they are practicing evidence-based
medicine in recommending this combination to their patients

National cancer institute-clinical trial results-posted 5/11/2010

Prognostic Factors for Survival
Survival after surgical resection is related to
-The extent of local invasion of the primary lesion
-Lymph node involvement
-Vascular invasion
-Perineural invasion
-Cellular differentiation
-Uninvolved surgical margins.
Even a single lymph node with evidence of metastatic carcinoma
portends a poor outcome with surgery alone.
Factors influencing long term
survival
Long-term survival was independently influenced by
-The depth of tumor infiltration and
-Lymph node metastasis

World J Surg. 2007 Jan;31(1):137-43; discussion 144-6

Pancreatobiliary versus intestinal histologic
type of differentiation
By definition "periampullary", originate from ampullary, duodenal, biliary, or
ductal pancreatic epithelium.
Typically, periampullary adenocarcinomas have either intestinal or
pancreatobiliary type of differentiation.
The aim of the study was to determine whether the histologic type of
differentiation is an independent prognostic factor in periampullary
adenocarcinoma.

CONCLUSION: Pancreatobiliary versus intestinal type of differentiation

independently predicts poor prognosis after pancreaticoduodenectomy
for periampullary adenocarcinoma.

BMC Cancer. 2008 Jun 11;8:170

Lymph Node Ratio as prognostic
factor
CONCLUSIONS: Not the lymph node involvement per se but
especially the LN ratio is an independent prognostic factor after
resection of pancreatic cancers.
In our series, the LN ratio was even the strongest predictor of
survival.
The routine estimation of the LN ratio may be helpful not only for
the individual prediction of prognosis but also for the indication of
adjuvant therapy

J Gastrointest Surg. 2009 Jul;13(7):1337-44. Epub 2009 May 6

Portal vein Resection.
In view of the close anatomical proximity between the head of the pancreas and
the portal/superior mesenteric vein confluence, it is logical for surgeons to seek
to expand the pool of patients who may benefit from pancreaticoduodenectomy
by undertaking en bloc resection of the vein
However portal vein resection is controversial.

Due tothe small proportion of patients who undergo portal vein resection, it is
difficult to construct a suitably designed randomised trial to address this
question.
Under these circumstances, surgeons will continue to base their practice on
summative evidence from case series.
Pancreaticoduodenectomy for Peri-Ampullary Malignancy:
The Case for Portal Vein Resection
2009 by the Annals of The Royal College of Surgeons
of England

The current literature suggests the addition of a venous resection does not cause
an increase in morbidity and mortality over a standar pancreaticoduodenectomy.

Importantly, the data indicate that portal vein resection can lead to similar
survival outcomes in same-stage tumours.
Currently, there is no randomised control trial or metaanalysis assessing the
potential benefit of the addition of venous resection to
pancreaticoduodenectomy.
However, there are numerous large case series and a systematic review
supporting venous resection
Conti...............

Yekebas et al recently reported on 585 patients undergoing

pancreaticoduodenectomy between 1994 and 2005 and compared patients
undergoing venous resection with those undergoing a standard
pancreaticoduodenectomy on an intentionto- treat analysis.

They found comparable median survival rates (15 months versus 16 months; P =
0.086) with no difference in peri-operative morbidity and mortality.
Concluded that venous resection at the time of pancreaticoduodenectomy can be
offered with similar morbidity and mortality.
Options for portal vein
reconstruction
Various methods are currently used for portal vein reconstruction.
-Adequate mobilisation of the liver and small bowel .
-Various conduits for reconstruction.
-Bovine pericardium
-Autologous saphenous
-Internal jugular vein or
-Left renal vein.
when the venous involvement is over 3 cm, hepatic mobilisation and use a
left renal vein graft, preferring this conduit since it is autologous and
located in the same operating field.
Conclusion

All surgeons undertaking pancreaticoduodenectomy should be able to

undertake venous resection and reconstruction since, occasionally, the
requirement for thismay be unexpected.

Despite the lack of randomised control trials, large series from major
pancreatic centres demonstrate that, when venous resection is
performed, it can be done safely and appears to give similar survival.
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