Pancreatic Cystic Neoplasms
Bible Class
4th Sept.2013
Universitätsklinik für Viszerale Chirurgie und Medizin
What type of pancreatic cysts exist ?
Why is this differentiation important ?
Acquired Cysts:
Post-inflammatory fluid collection
Pseudo-,-Pseudocyst
Postnecrotic
sequestrum
Benign
Parasitic, Ecchinococcal etc.
Congenital
Cysts:
Cystic
Neoplasms:
-
IPMN:
-
MCN:
True cysts
Enterogenous
cysts/ duplication
Intraductal
papillary mucinous
neoplasmcysts
(Epi)dermoid cysts, Endometriose
Mucinous
cystic neoplasm
Polycystic
diseases; Cystic Fibrosis
- SCN:
Serous
cystic
adenoma/ neoplasm
Risk
Malignancy
Cystic Neoplasms:
- SPN:
Solid pseudopapillary neoplasm
-
CPEN:
Cystic pancreatic endocrine neoplasm
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
How frequent are neoplastic
pancreatic cystic lesions ?
Average:
2.5%
Age > 70 years: 10-20%*
*: MRI in non-pancreatic disease: 20% of 1444 patients; Zhang XM et al. Radiology 2002
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
Key features: Serous Cystic Neoplasm
 Malignant potential:
NO
 Location:
throughout the pancreas
 Demographics, rate: (older) women (80%), 15-20% of PCNs
 Morphology:
micro-, oligo-, macrocystic
typically: multicystic cluster (each < 2 cm) = honeycumbed
No communication with pancreatic duct
Stroma: (central fibrous and) calcified (stellate scar)
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
Key features: IPMN
 Types:
Main-, branch-duct, mixed type
 Malignant potential:
Yes (esp. main/combined duct IPMN)
 Location:
M: head
BD: multifocal !!
 Demographics, rate: Equal m/w, middle-age/old; >25% of PCNs
 Morphology:
Cystic dilatation main (> 6 mm) or side
branches; M: Fish-mouth, globules of mucin (= masses)
Stroma: Lack of ovarian stroma (vs. MCN)
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
Key features: MCN
 Malignant potential:
Yes (but lower than IPMN)
 Location:
Body/tail (95%), always single lesion!
 Demographics, rate: Middle-aged women (95%), 25% of PCNs
 Morphology:
thick-walled single cyst, often septations
Epithelial layer with mucin-producing cells, ovarian-like stroma
No communication with pancreatic duct
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
Risk of malignancy in pancreatic neoplastic cysts ?
What factors determine malignant risk in IPMN/MCN?
IPMN: BD-:
 Size̴ 40% (6-46%)
++
Risk of HGD/ malignancy 1
++++
̴ 65% (57-92%) Risk of HGD/ malignancy in 5 y
MCN:
++
6-36% Prevalence malignancy 1
SCN:
(+)
VERY low (malignant = serous cystadenocarcinoma)
SPN:
+
Low malignant potential 2
CPEN:
Variable 2
MD-:
1
 Histopathological type
1: Sakorafas GH et al. Surg Oncol. 2011; 2 Sakorafas GH et al. Surg Oncol 2012
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
What are high-risk stigmata for
malignancy in IPMN/MCN?
 Obstructive jaundice (and cystic lesion of the pa-head)
 Enhancing solid component within cyst
 Main pancreatic duct > 10 mm in size
Consequence?
Consider surgery, if clinically appropriate
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
If no high-risk stigmata in IPMN/MCN:
What are worrisome features ?
Clinical:
Imaging:
Pancreatitis
Cyst > 3 cm
Thickened/enhancing cyst walls
Main duct size 5-9 mm
Non-enhancing mural nodule
Abrupt change in caliber of pancreatic duct
with distal pancreatic atrophy
Consequence?
Endo-Sonography
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
What are the advantages of EUS in
diagnostic workup of pancreatic cysts ?
 Superior, higher-resolution imaging of the pancreas
(ductal communication, additional (smaller) cysts, nodules etc.)
 Fine-needle-aspiration (FNA): sampling fluid for
Cytology and tumor markers
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
What are drawbacks of EUS ?
 Operator-Dependent Investigation
 Sampling Error
 Contamination (gastric wall)
 Low cellularity -> Low senstivity
e.g. SCN only 30-40% enough cells
diagnostic accuracy: 10-60%
Including high-grade
often NON-diagnostic
atypical epithelial cells:
diagnostic in mucinous cysts
diagnostic accuracy: 80%
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
What are EUS features leading to consider
surgery ?
 Define mural nodule(s): 3-9 fold risk malignancy
 Main duct features suspicious for involvement
 Cytology: suspicious or positive for malignancy
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
EUS-FNA: Fluid Analysis in Cysts
Typ
SCN
MCN
IPMN
SPN
Pseudocyst
Viscosity
Low
High
High
NA
Low
Mucin
Low
High
High
NA
Low
Amylase
< 250 U/L
< 250 U/L
< 250 U/La
Low
High
Cytology
negative
mucin-
papillary
Branching
papillae
cuboid or
cylindric cells,
high cellularity,
myxoid stroma
«dirty
material»
clusters of
or
Glyogen-containing cuboid
cells
containing
column cells
mucincolumn cells,
atypia
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
Macrophages,
Inflammatory cell
CEA in Cyst-Fluid: What for ? Useful ?
 Mucinous vs. Non-mucinous (serous)
 Cut-off unclear: e.g. > 800 ng/mL
 No correlation with risk of malignancy
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
How to perform surveillance for
BD-IPMN and MCN?
< 1 cm:
CT/MRI in 2-3 years
1-2 cm:
CT/MRI yearly (for 2 years)
lengthen interval if no change
2-3 cm:
EUS in 3-6 months
Lengthen interval, alternating EUS and MRI
Consider surgery in young, fit patients (long surveillance)
> 3 cm:
Close surveillance
alternating MRI with EUS every 3-6 months
Strongly consider surgery (in young, fit patients)
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
Which syndrome associates with multiple/
oligocystic SCN ?
Hippel-Lindau-Syndrome
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch