This document provides an overview of benign and malignant tumors of the pancreas. It begins with the anatomy and physiology of the pancreas, describing its location, duct system, vasculature, and endocrine and exocrine functions. It then discusses different types of cystic tumors (serous cystadenoma, mucinous cystadenoma, intraductal papillary mucinous adenoma), adenocarcinoma, tumors of the ampulla of Vater, and endocrine tumors. Adenocarcinoma makes up about 85% of pancreatic cancers and has a very poor prognosis. Surgical resection remains the only potentially curative treatment option for pancreatic tumors.

1. Benign and Malignant Tumors of
The Pancreas
MEDİCAL UNIVERSITY OF SOFIA
Nur UYANIK

2. ANATOMY
The pancreas is an abdominal glandular organ,
with a digestive (exocrine) and hormonal
(endocrine) function.
The normal pancreas is about 15 cm in length,
approximately 120 g, and is located in the
retroperitoneum.
It is a retroperitoneal structure (lies behind the
peritoneal cavity), located deep within the upper
abdomen in the epigastrium and left
hypochodrium regions.

3. ● Stomach – lies anteriorly and superiorly.
● Duodenum – situated anteriorly and medially,
curving around the head of the pancreas.
● Spleen – located posteriorly and laterally. It is
connected by ligaments to the tail of the
pancreas.
● Vasculature – the aorta and inferior vena cava
pass posteriorly to the head of the pancreas.

4. ANATOMIC RELATIONSHIPS OF THE
PANCREAS

5. The pancreas is typically divided into five parts;
● Head: This is the widest part of the pancreas. It
lies within the C-shaped curve created by the
duodenum, and is connected to it by connective
tissue.
● Uncinate process: This is a projection arising
from the lower part of the head and extending
medially to lie beneath the body of the
pancreas. It lies posterior to the superior
mesenteric vessels.
● Neck: Located between the head and the body
of the pancreas. It overlies the superior
mesenteric vessels which form a groove in its
posterior aspect.

6. ● Body: The body is centrally located, crossing
the midline of the human body to lie behind the
stomach and to the left of the superior
mesenteric vessels.
● Tail: The left end of the pancreas that lies within
close proximity to the hilum of the spleen. It is
contained within the splenorenal ligament with
the splenic vessels. This is the only part of the
pancreas that is intraperitoneal.

8. Duct System
The exocrine compartment is classified as a
serous gland. It is composed of approximately a
million ‘berry-like’ clusters of cells called acini,
connected by short intercalated ducts.
Intercalated duct cells beginning within acini are
called centroacinar cells. The intercalated ducts
drain into a network of intralobular collecting
ducts, which in turn drain into the main pancreatic
duct.

9. The pancreatic duct runs the length of the
pancreas and unites with the common bile duct,
forming the hepatopancreatic ampulla of Vater.
This structure opens into the duodenum.
Secretions into the duodenum are controlled by
a muscular valve – the sphincter of Oddi. It
surrounds the ampulla of Vater, acting as a valve.

10. Vasculature
● The pancreas is supplied by the pancreatic
branches of the splenic artery. The head is
additionally supplied by the superior and inferior
pancreaticoduodenal arteries which are
branches of the gastroduodenal and superior
mesenteric arteries, respectively.
● Venous drainage of the head of the pancreas is
into the superior mesenteric branches of the
hepatic portal vein. The pancreatic veins
draining the rest of the pancreas do so into the
splenic vein.

13. This classification is used to denote the location of
metastatic spread of pancreatic neoplasms or for
other detailed studies.

14. PHSYOLOGY OF THE PANCREAS
● The endocrine islets of Langerhans which
secrete insulin, glucagone and somatostatin.
● Acinar gland tissues which produce pancreatic
juice ( the main source of digestive enzymes).
The cells lining the acini are serous cells
containing zymogen granules.

15. ● Pancreatic Secretion
● Pancreatic juice is secreted in response to the
presence of chyme in the upper portions of the
small intestine.
● The major functions of pancreatic secretion:
- To neutralize the acids in the chyme .
-To produce enzymes involved in the digestion
of dietary carbohydrate, fat, and protein.

16. ● Pancreatic Secretion
-The volume:- 1.2-1.5 L/day.
-The osmolarity of pancreatic fluid is equal to
that of plasma (isotonic).
- pH= 8 alkaline.
-The electrolytes They are produced from the
epithelial cells of the ductules and ducts and
include cations Na +, K +, Ca ++ and anions
HCO3 - and Cl

20. ENDOCRİNE PANCREAS
● β (B) cells:
–Constitute 70% of islet cells.
–Concentrated in islet center.
–Function: secrete insulin which ↓ blood sugar.
● α (A) cells:
–Constitute 15-20%.
–Concentrated in islet periphery.
–Granules are much more numerous, more tightly
packed, smaller, and denser than those of β cells.
–Function: secrete glucagon which ↑ blood sugar.

21. ● δ (D) cells: δ (D) cells:
–Constitute 5-10% of islet cells.
–Function: secrete somatostatin which ↓ release of
hormones from endocrine pancreas and enzymes
from exocrine pancreas.
● G cells:
–Constitute 1% of islet cells.
–Function: secrete gastrin which ↑ production of HCl
by parietal cells of stomach.
● PP cells:
–Constitute 1% of islet cells.
–Function: secrete pancreatic polypeptide which ↓
exocrine secretions of pancreas.

22. CANCERS OF EXOCRİNE PANCREAS
BENIGN
● Serous cystadenoma
● Mucinous cystadenoma
● Intraductal papillary
mucinous adenoma
● Mature cystic teratoma
MALIGN
● Ductal adenocarsinoma
● Serous-mucinous
cystadenocarcinoma
● Intraductal mucinous
papillary tumor
BORDERLINE
● Mucinous cystic tumor
● Intraductal papillary mucinous tumor
● Solid pseudopapillary tumor

23. EPIDEMIOLOGY
● Age
● Obesity
● Family history
● Smoking
● Chronic pancreatic disease

24. Cystic tumors of the pancreas
● Cystic tumors of the pancreas derive their name from
the presence of fluid in the tumor.
1.Serous cystadenoma
2.Mucinous cystadenoma
3.Intraductal papıllary mucinous adenoma

25. 1.Serous cystadenoma :
● Generally benıgn , no malıgnant potential.
● Can be found anywhere in the pancreas but
frequently in the HEAD of the pancreas.
● Also known as a microcystic adenoma and is a
second most common cystic tumor of the
pancreas.
● These tumors have a honeycombed
appearance.
● Diagnosis is made based on a characteristic CT
scan for this tumor.

26. ● Mean age 66, 70% women, associated with von
Hippel Lindau syndrome
● Symptoms: local discomfort/pain, obstruction if
in pancreatic head; may cause diabetes if
tumor destroys enough islets
● Excision is almost always curative

28. 2.Mucinous cystadenoma.. Patient: 9:1, F:M
● Mucinous cystadenomas are the most frequent cystic
tumors of the pancreas . While these tumors are
usually benign, if left untreated will probably evolve to
a malignant tumor.
● 80% of mucinous cystic tumors occur in females and
the majority of the tumors occur in the younger female.
● Abdominal pain or mass
● < 20% associated with invasive carcinoma
● Metastases usually restricted to abdominal cavity;
metastases to ovary may simulate primary ovarian
tumors
● Can also occur in the liver

29. ● The surgical procedure depends on the location
of the tumor. The vast majority of these tumors
are precancerous. Because it is a precancerous
tumor ,pancreatic head resection preserving the
duodenum and the bile duct is offered.
● Generally located in the body and the tail of the
pancreas.

31. Intraductal papıllary mucinous adenoma (IPMN)
Patient: M=W
● Epithelial neoplasm of mucin-producing cells, arising
in the main pancreatic duct or its branches.
● has replaced such terms as “mucin-producing tumor”
and “mucinous ductal ectasia.”
● Mostly seen in HEAD of the pancreas.
● High risk for malıgn transformation.
● More common in men age 60+ at head of pancreas
● Signs and symptoms include epigastric pain, weight
loss, jaundice, diabetes, pancreatitis

33. Pancreatic pseudocysts
● Pancreatic pseudocysts most often develop
after an episode of acute pancreatitis.
● The cyst happens when the ducts (tubes) in the
pancreas are damaged and fluid with enzymes
cannot drain.
● Bloating of the abdomen
● Constant pain or deep ache in the abdomen,
which may also be felt in the back
● Difficulty eating and digesting food
● Does not include epithelial cell in aspirated fluid
!!!!

34. Possible treatments include:
● Drainage through the skin using a needle, most
often guided by a CT scan
● Endoscopic-assisted drainage using an
endoscope (a tube containing a camera and a
light that is passed down into the stomach)
● Surgical drainage of the pseudocyst, which
involves making a connection between the cyst
and the stomach or small intestine. This may be
done using a laparoscope.

35. ADENOCARCINOMA
● The most common ( 85% ) type of all
cancerous tumors of the pancreas are
adenocarcinomas.
● HEAD ---> 60-70 %
● BODY ---> 15-20 %
● TAIL ---> 5-10 %

36. 1955-2011

38. >Pancreatic Adenocarcinoma Prognosis
● 20 % survival at 1 yr
● <4 % survival at 5 yrs
>Pancreatic Adenocarcinoma Risk Factors
● Smoking (biggest)
● > 50 yrs old
● Diets high in fat
● Obesity
● Hereditary Pancreatitis
● MEN
●
Dıabet

39. ...
● K-ras oncogen
● HER-2 / neu oncogen activation
and ALSO :
● P16
● P53
● BRCA2
● DPC4 tumor supressor gene mutations are
involved.

40. Pancreas head Adenocarcinoma
Clinical Presentation (70 % of lesions)
-Jaundice
-Steatorrhea
-Constant pain: radiates to the back
-Courvoisier's sign
pancreas tail Adenocarcinoma
Clinical Presentation
-Diagnosed later than head lesions
-Weight loss
-Constant pain: radiates to the back

41. Clinical findings
Generally ;;;;
● Late onset of clinical presentation
● Epigastric pain
● Head --> jaundice
● Body and tail --> x
● Weight loss is most common symptom.
● Trousseau sign (thrombophlebitis migrans)
● Courvoiser – terrier

42. ●Trousseau sign of malignancy
● Due to release of PAF
(platelet aggregating
factor ) from the
tumor or necrotic
tissue around the
tumor .

43. jaundice

44. ● Courvoisier’s sign (or
law) describes an
enlarged, palpable
gallbladder in patients
with obstructive jaundice
caused by tumors of the
biliary tree or by
pancreatic head tumors.
The gall bladder will be
dilated, with a thin wall; it
is not tender to the
touch.

45. DIAGNOSTIC
● Laboratory: higher bilirubin, tumormarker CA 19–9 (but
is not specific, can be found in patients with colon
cancer or biliary obstruction);
● USG: in clasical ultrasonography need not to be found
small tumors of pancreas (or even pancreas);
● EUSG: endoscopic sonography is better methode for
finding of pathologies in pancreas than clasical
abdominal USG;
● ERCP (endoscopic retrograde cholangio-
pancreatography) is the best methode for therapeutic
intervention of biliary obstruction (stent)
● CT: computer tomography is always neccesary in
staging of pancreatic cancer, results of CT will decide
about the therapy.

46. TREATMENT
● Curative therapy
1..Surgery
● Whipple’s operation (partial pancreatico-
duodenectomy) – pancreatic head tumors
● total pancreatico-duodenectomy (with gastro-
jejunoanastomosis), then is neccesary
pancreatic enzymes and hormones substitution
● resection of the tail of pancreas (just only in
pancreatic tail tumors).

47. ● Chemotherapy
● There is no chemotherapy bringing better
resultes than 6–8 months survival time. At this
time is used:
● 5FU – 5 fluoruracil;
● gemcitabine.

48. Palliative Therapy
● Palliative therapy is based on patients
symptoms:
● therapy of pain – analgetics, epidural analgesia
or coeliac ganglion destruction;
● therapy of biliary obstruction – metalic stents
via ERCP or hepatico-jejuno anastomosis
(surgery);
● therapy of gastrointestinal obstruction
(especially duodenal obstuction by pancreatic
head tumor) – gastro-jejuno anastomosis.

52. Tumor of ampulla of vater

53. Production of local carcinogens through the
combined interactions of the components of bile,
pancreatic juice, and duodenal contents. Both
benign and malignant tumors of the ampulla of
Vater occur. The benign tumors include
adenomas, gastrointestinal stromal tumors
(GISTs), lipomas, and neuroendocrine tumors.
ADENOMA AND ADENOCARCINOMA Most
common malignant tumor of the ampulla of Vater .

54. ● Weight loss occurs in 75% of patients
Abdominal pain in 50% Occult gastrointestinal
bleeding is common, in one third of patients
Nonspecific symptoms such as anorexia,
dyspepsia, and malaise Rarely, with
pancreatitis secondary to pancreatic duct
obstruction With features of sphincter of Oddi
dysfunction .

55. ● Physical examination include conjunctival or
cutaneous icterus and, less commonly,
hepatomegaly, a distended gall-bladder.
● Diagnosis The earliest and most common
laboratory abnormality is an increase in the
serum alkaline phosphatase level Followed by
hyperbilirubinemia as the tumor obstructs the
bile duct. No tumor markers have been
identified that are either sensitive or specific
The first imaging modality should be
ultrasonography or CT to determine the level of
biliary obstruction. Dual-contrast helical CT is
the most informative . ERCP is often the next
procedure for patients with a suspected
ampullary malignancy .

56. Staging Primary Tumor (T Stage)
● T1 Tumor limited to ampulla of Vater
● T2 Tumor invades duodenal wall
● T3 Tumor invades 2 cm into pancreas
● T4 Tumor invades >2 cm into pancreas and/or
adjacent organs
Regional Lymph Nodes (N Stage)
● N0 No regional lymph node metastasis
● N1 Regional lymph node metastasis

57. Distant Metastasis (M Stage)
● M0 No distant metastasis
● M1 Distant metastasis
TNM Stage Grouping
● Stage I T1 N0 M0
● Stage II T2-3 N0 M0
● Stage III T1-3 N1 M0
● Stage IVA T4 N0-1 M0
● Stage IVB T1-4 N0-1 M1

58. ● local resection of ampullary tumors is reserved
for patients with a benign adenoma or
ampullary neuroendocrine tumor and for highly
selected patients with ampullary
adenocarcinoma The options for local treatment
are endoscopic snare removal, endoscopic
ablation, and surgical ampullectomy.

59. Endescopic ampullectomy

61. Endocrine tumors of pancreas
● Neuroendocrine tumors (NETs) are rare tumors
(incidence rate, 5 cases per 1 million person-
years) that arise from endocrine cells within or
near the pancreas and account for less than
5% of all pancreatic tumors.
● sporadically or as part of inherited genetic
syndromes such as multiple endocrine
neoplasia type 1, neurofibromatosis...
● Most primary NETs arise within the gastrinoma
triangle, composed of the joining of the cystic
and common hepatic ducts, second and third
portions of the duodenum, and border of the
body and tail of the pancreas.

62. A special epidemiology.. MEN
● The term multiple endocrine neoplasia is used
when two or more endocrine tumor types,
known to occur as a part of one of the defined
MEN syndromes, occurs in a single patient and
there is evidence for either a causative
mutation or hereditary transmission. The
presence of two or more tumor types in a single
patient does not automatically designate that
individual as having MEN because there is a
small statistical chance that development of two
"sporadic" tumors that occur in one of the MEN
syndromes could occur by chance.

63. ● APUD cells : unrelated endocrine cells.
common function of secreting a low molecular
weight polypeptide hormone.
● APUD cells in pancreas :insulin , gastrin,
somatostatin, pancreatic polypeptide.
● gastric APUD cells :gastrin, glucagon ,
substance P .
● Intestinal APUD cells : motilin , substance C ,
enteroglucagon, secretin , somatostatin

64. INSULINOMA
● Most common
● 90% -- benıgn
● 10% -- malıgnant
● 10% --part of MEN 1
● Increased insulin and C peptide levels
● Cerebral glucose dercrease leads to symptoms
such as : convulsion , loss of memory ,
behavioral changes...
● Ct – endoscopic ultrasonography
● TREATMENT : Distal pancreatectomy ,
enucleation (refers to the surgical removal of a

66. Blood glucose:
< 50mg /dl

68. GASTRİNOMA
● Gastrin is a peptide hormone that stimulates
secretion of gastric acid (HCl) by the parietal
cells of the stomach and aids in gastric motility.
It is released by G cells in the pyloric antrum of
the stomach, duodenum, and the pancreas.
● A gastrinoma is a gastrin-secreting tumor .More
than 80% of gastrinomas arise within the
triangle ( passaro triangle ).
● 25% -- MEN 1.
● OCTEREOTİDE SCINTİGRAPHY ! Octreotide
is a synthetic analogue of somatostatin .Tumors
with high expression of somatostatin receptors
ARE DETECTED.

73. PATOPHYSİOLOGY

74. Prognosis
Patients with hepatic metastases may have a
remaining life span of less than 1 year; the 5-year
survival rate is 20-30%.
In patients with localized disease or metastasis to
local lymph nodes without liver metastasis, the 5-
year survival rate may be 90%.
Surgical resection of localized disease leads to a
complete cure without any recurrence in 20-25%
of patients with gastrinomas.

75. Treatment
Symptomatic treatment
● Proton pump inhibitor; octreotide
● Enucleation: Many small gastrinomas in the
pancreas may be treated by enucleation alone.
This is a procedure of choice for patients that
have small tumors (less than 1cm) where the
tumor is located on the surface of the pancreas.
● Resection of the pancreas: in patients with
large tumors a distal pancreatectomy or a
Whipple operation may be indicated depending
on where the tumor is located in the pancreas.

76. ● Duodenal exploration: Gastrinomas often occur
in the wall of the duodenum (first part of the
intestine) and therefore opening duodenum and
carefully feeling it to remove any tumors in this
area is important.
● Lymph nodes: In some patients the tumor may
be located in the lymph glands outside the
pancreas therefore careful palpation and
removal of these glands is important at the time
of surgery

77. ● Other neuroendocrine tumors such as
glucagonoma, VIPoma and somastatinoma are
extremely uncommon tumors. These tumors
are malignant in the vast majority of patients
and may present as large tumors at the time of
diagnosis. Up to 70% of patients have evidence
of spread of the tumor at the time of the
diagnosis. Aggressive surgical removal to
relieve some of the severe symptoms .

78. ● Glucagonoma : Glucose intolerance; migratory
necrolytic erythema; weight loss; anemia;
● 90% malignant
● Somatostatinoma :Diabetes; gallstones;
secretory diarrhea
● VIPoma ( verner morrison syndrome ) :
Cholera-like, secretory diarrhea; hypokalemia;
hypochlorhydria

79. ● When a NET is suspected, imaging tests are
used to locate the primary tumor and determine
the presence of metastases . NETs may be
difficult to localize. Contrast-enhanced CT and
MRI may be used as initial tests; however, they
have a low yield for small tumors. Endoscopic
US is a more sensitive test for detecting small
pancreatic neuroendocrine tumors. Insulinomas
are not well visualized with octreotide scans
because they do not possess high
concentrations of somatostatin receptors...