Cystic neoplasm of pancreas

1. Primary Pancreatic Cystic
Neoplasms
Anup Shrestha
CMC

2. History
• Becourt in 1830.

3. Introduction
• Cystic neoplasms of the pancreas are the
heterogenus group of cystic lesions and
remain a diagnostic challenge.
• While the incidence of these lesions increases,
so does the differential diagnosis.
• Management decisions are extremely difficult
due to the uncertain biologic behavior of
these lesions.

4. Klöppel’s Classification of Cystic Neoplasms
of the Pancreas

5. • The three most common types of PCNs are
serous cystic neoplasms (SCNs), mucinous
cystic neoplasms (MCNs), and IPMNs,
representing approximately 90% of all PCNs.
• MCNs and IPMNs are the most common and
more importantly have the highest potential
for malignant transformation.
• SCNs occur much less frequently and are
almost always benign.

6. SEROUS CYSTIC NEOPLASMS(SCN)
• described as multilobulated multiloculated cystic
masses with central stellate scars and calcifications
• SCNs are most commonly observed in women (3 : 1
female to male ratio)
There are four subtypes of SCNs:
• serous microcytic adenoma
• serous macrocytic (oligocystic) adenoma
• von Hippel-Lindau (VHL)-associated pancreatic cysts
• serous cystadenocarcinoma.

7. Pathology
• SCNs are characterized by
serous fluid–filled cysts lined
by a single layer of cuboidal
epithelial cells with uniform,
round, darkly stained nuclei
and a glycogen-rich cytoplasm.
• Notably, there is a lack of
atypia, necrosis, and mitotic
features in SCNs.
• On gross examination,
microcystic SCNs have a
characteristic honeycomb
appearance with multiple thin-
walled cysts around a central
scar

8. Von Hippel-Lindau–Associated
Pancreatic Cysts
• VHL disease is an autosomal dominant mutation
of chromosome 3p25 causing multiorgan
pathology. VHLassociated pancreatic cysts show a
loss of heterozygosity on chromosome 3p25 and
mutations in the VHL gene.
• These lesions are indistinguishable from sporadic
serous cystadenomas. In contrast, VHL patients
tend to have multifocal disease rather than a
single lesion. These lesions behave similarly to
non-VHL serous cystadenomas with minimal
malignant potential.

9. Serous Cystadenocarcinoma
• Malignant SCNs are
exceedingly rare
• Serous cystadenocarcinomas
are misdiagnosed as a
malignancy due to the
presence of vascular
impingement on imaging.
• These malignant cysts are
nearly identical to benign
serous cystadenomas and are
distinguished only by the
presence of metastases

10. Imaging
• Three different imaging patterns exist for
SCNs: microcystic, honeycomb, and oligocystic
1.Microcystic

11. 2.Honeycomb Pattern

12. 3.Oligocystic pattern

13. MRI
• SCNs on MRI appear as a
“cluster of grapes” on T2-
weighted images with
multiple small cysts and
enhanced septations.
• The presence of bright T1-
weighted cystic fluid suggests
hemorrhagic fluid content.
• The main disadvantage of
MRI is the inability to detect
central calcifications
commonly seen with SCNs

14. EUS-FNA and Cytology
EUS
• SCNs typically appear as
microcystic compartments
with the absence of fluid.
• Some SCNs have
macrocystic components
that can resemble MCNs.
Cytology
• For cytological assessment
and cyst fluid drainage, in
order to distinguish
between serous and
mucinous lesions
• The cytology of SCNs
includes bland cuboidal
cells containing glycogen,
clear cytoplasm without
cellular atypia or necrosis

15. Treatment and Survillence
• Asymptomatic patients with radiological evidence of an
SCN should be followed up for 1 year.
• When the diagnosis of SCN is clear, surgery is
recommended only in patients with symptoms related to
the compression of adjacent organs (ie, bile duct, stomach,
duodenum, portal vein)
• Other indications include cyst size greater than 4 cm and
uncertainty of diagnosis despite appropriate radiologic
assessment
• enucleation of the cyst.
• Avoid Enucleation : is associated with high morbidity
(approximately 40%) due to the development of a
pancreatic fistula

16. MUCINOUS CYSTIC NEOPLASMS
• The average age range of presentation is 40
to 50 years old
• they are found predominantly in
perimenopausal women (20 : 1 female-to-
male ratio).
• The vast majority of MCNs are found in the
body and tail of the pancreas.
• Most MCNs are approximately 6 to 10 cm
at the time of diagnosis but range from 1.5
to 35 cm in diameter.
• On gross examination, they are spherical
and infrequently encapsulated by a calcified
fibrous wall.
• The cyst can be filled with mucin, blood, or
a watery fluid. This fluid tends to be thicker
and more viscous than in SCN, due to the
presence of mucus. MCNs do not
communicate with the pancreatic ductal
system

17. MCN- Histology
• MCNs are lined by tall mucin-
producing columnar cells.
• These epithelial cells that line
the cyst may be papillary or
flat and can show a tendency
toward gastric or intestinal
differentiation.
• Ovarian-like stroma is a
histologic feature often seen
• in MCN.

18. MCN-Malignant Potential
• three distinct categories based on the degree of
cellular atypia: low-, intermediate-, and high-grade.
• The classification is based on the greatest degree, and
not the average, of the epithelial dysplasia.
• Less than 20% of MCNs are associated with invasive
carcinoma and thus should be considered a potential
precursor to pancreatic cancer.
• Extensive sampling of the cyst is recommended, given
the relatively small volume of the invasive component.
It appears that the incidence of malignant
transformation is directly correlated to the overall size
of the cyst and the complexity of the cyst.

19. Imaging-CT
(A) Macrocystic form: note
septum and lack of
surrounding inflammatory
reaction.
(B) Several macrocystic areas
(>2 cm) in midbody of
pancreas.

20. MRI
• well-defined, uniloculated or multiloculated cystic lesion(s)
with enhanced septations and occasionally solid components..
• High signal intensity on T1- and T2-weighted imaging can result
from mucin within the cyst.
• The proximity of the cyst to the ductal system is better
assessed with MRI and can help to differentiate MCNs from
pseudocysts and IPMNs.

21. Cystic Fluid Analysis
• The presence of mucin is highly specific for MCNs
• Most MCNs contain higher concentrations of CEA
compared with their SCN counterparts
(>192ng/mL)

22. Treatment and Survilence
• MCN ≥40 mm should undergo surgical resection.
• Resection is also recommended for MCN which
are symptomatic or have risk factors (ie, mural
nodule) irrespective of their size
• MCN measuring <40 mm without a mural nodule
or symptoms may undergo surveillance with MRI,
EUS, or a combination of both. Surveillance is
recommended every 6 months for the first year,
then annually if no changes are observed

23. INTRADUCTAL PAPILLARY MUCINOUS
NEOPLASMS
IPMNs are defined in the WHO Classification of Tumors of the Digestive
System as an intraductal, grossly visible epithelial neoplasm of mucin-
producing cells. Using imaging and histology, IPMNs can be classified into
three types based on duct involvement:
1. Main-duct IPMN (approximately 25% of IPMNs): Segmental or diffuse
dilation of the main pancreatic duct (>5 mm) in the absence of other
causes of ductal obstruction.
2. Branch-duct IPMN (approximately 57% of IPMNs): Pancreatic cysts (>5
mm) that communicate with the main pancreatic duct.
3. Mixed type IPMN (approximately 18% of IPMNs): Meets criteria for both
main and branch duct.

24. Pathology
• IPMN lesions are
characterized by papillary
projections of columnar-
lined epithelium with
varying degrees of
dysplasia.
• Mucin is typically
abundant both within the
cytoplasm of the lining
epithelial cells as well as
within the acellular fluid
matrix

25. Intraductal Papillary Mucinous Neoplasm–Associated
Malignancy
• Invasive cancer is found in 20% to 50% of
resected IPMN specimens
Factors shown to be associated with the presence
of invasive cancer
• jaundice at presentation
• larger lesions (≥ 30mm)
• mural nodules (≥5 mm)
• main-duct dilation ≥10 mm
• serum cancer antigen (CA) 19-9 levels

26. Risk of high-grade dysplasia or
malignancy according to dilatation of
the main pancreatic duct in IPMN
The European Study Group on Cystic Tumours of the Pancreas. Gut 2018;67:789–804.

27. Genetics of Intraductal Papillary
Mucinous Neoplasm
• Similar to the genetic landscape of pancreatic
cancer, KRAS mutations, loss of p16, and TP53
mutations are frequently observed in IPMN
• SMAD4/ DPC4 expression, which is inactivated
in more than half of patients with pancreatic
adenocarcinoma, is preserved in virtually all
noninvasive IPMNs.
• Another prominent difference is the
mutations of the GNAS gene in IPMN.

28. Clinical presentation
Presentation:
• Abdominal pain.
• Pancreatitis.
• Weight loss.
• Jaundice.
• New onset diabetes.
Investigation
• variable amylase level
• a high CEA level (>200 ng/mL)
• CT Scan characteristics:
• Main pancreatic or duct dilation.
• Involvement of any part of the pancreas
or the whole pancreas.
• Continuity of cyst with ductal system.
• Irregular and poorly demarcated

29. MRCP and EUS
• MRCP may actually be
better than CT for
determining a
communication with the
pancreatic duct and thus
in diagnosing branch-duct
IPMN
• Careful evaluation with
EUS can provide detailed
images of the cyst wall
and internal cyst
architecture. Fine-needle
aspiration and biopsy can
be performed with EUS.

30. EUS

31. Treatment of MD-IPMN
• Indications for Surgery

32. Extent of Resection
• Limited to a part of the pancreas and there is no overt
radiographic evidence of malignancy resection of the
IPMN-containing pancreas, with pancreaticoduodenectomy
for the head of the pancreas
• distal pancreatectomy for the body and tail of the
pancreas.
• Patients with MD-IPMN diffusely involving the whole gland
pose a difficult management dilemma.
• In cases in which there is a mural nodule within the MPD
further along the duct, or in patients with an increased risk
for malignancy (ie, patients with familial pancreatic cancer),
a total pancreatectomy can be considered
• MT-IPMN carries a risk of malignant transformation that is
comparable to MD-IPMN, and resection is therefore
advised in patients who are fit for surgery

33. Resection of BD-IPMN
Tanaka M, Fernández-del Castillo C, Adsay V, Chari S, Falconi M, Jang J-Y, et al.
International consensus guidelines 2012 for the management of IPMN and MCN of the
pancreas. Pancreatology. 2012 Jun;12(3):183–97
High-risk stigmata of malignancy
• Obstructive jaundice in a patient with a cystic lesion in the
head of the pancreas
• Enhancing mural nodule ≥ 5 mm
• Main pancreatic duct ≥ 10 mm in size
Worrisome clinical features
• Pancreatitis
Worrisome imaging features
• Cyst ≥ 3 cm
• Enhancing mural nodule < 5 mm
• Thickened/enhancing cyst walls
• Main duct size 5–9 mm
• Nonenhancing mural nodule
• Abrupt change in caliber of pancreatic duct with distal
pancreatic atrophy
• Lymphadenopathy
• Increased serum level of CA-19-9
• Cyst growth rate ≥ 5 mm / 2 years
Resection
without
further
investigation
Should undergo
further
evaluation with
EUS

34. Survillence

35. Types of Surgery
• Branch-duct lesions in the head of
the pancreas are resected with
pancreatoduodenectomy
• In the tail, distal pancreatectomy
with or without splenectomy.
• Recently, pancreas-sparing
procedures, such as enucleation
and central pancreatectomy, have
been reported in an effort to
maintain the exocrine and
endocrine pancreatic function
• Total pancreatectomy is reserved
for fit patients with diffuse MD-
IPMN

36. Reference
• European evidence-based guidelines on
pancreatic cystic neoplasms (The European Study
Group on Cystic Tumours of the Pancreas)
• BLUMGART’S Surgery of the Liver, Biliary Tract,
and Pancreas (6th edition)
• Shackelford’s SURGERY Of The ALIMENTARY
TRACT( 8th Edition)
• The many faces of pancreatic serous
cystadenoma: Radiologic and pathologic
Correlation L.C. Chu et al. Diagnostic and
Interventional Imaging (2017) 98, 191—202

37. Thank You