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Pancreatic Cystic Neoplasms
Bible Class4th Sept.2013
Universitätsklinik für Viszerale Chirurgie und Medizin
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
What type of pancreatic cysts exist ?
Acquired Cysts:
Congenital Cysts:
Cystic Neoplasms:
Post-inflammatory fluid collectionPseudo-,-PseudocystPostnecrotic sequestrumParasitic, Ecchinococcal etc.
True cystsEnterogenous cysts/ duplication cysts(Epi)dermoid cysts, EndometriosePolycystic diseases; Cystic Fibrosis
Cystic Neoplasms:
- IPMN: Intraductal papillary mucinous neoplasm
- MCN: Mucinous cystic neoplasm
- SCN: Serous cystic adenoma/ neoplasm
- SPN: Solid pseudopapillary neoplasm
- CPEN: Cystic pancreatic endocrine neoplasm
Why is this differentiation important ?
Risk Malignancy
Benign
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:
Location:
Demographics, rate:
Morphology: micro-, oligo-, macrocystic
typically: multicystic cluster (each < 2 cm) = honeycumbed
No communication with pancreatic duct
Stroma: (central fibrous and) calcified (stellate scar)
NO
throughout the pancreas
(older) women (80%), 15-20% of PCNs
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
Key features: IPMN
Types:
Malignant potential:
Location:
Demographics, rate:
Morphology:
Yes (esp. main/combined duct IPMN)
M: head BD: multifocal !!
Equal m/w, middle-age/old; >25% of PCNs
Main-, branch-duct, mixed type
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:
Location:
Demographics, rate:
Morphology:
Yes (but lower than IPMN)
Body/tail (95%), always single lesion!
Middle-aged women (95%), 25% of PCNs
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 ?
IPMN: BD-:
MD-:
MCN:
SCN:
SPN:
CPEN:
1: Sakorafas GH et al. Surg Oncol. 2011; 2 Sakorafas GH et al. Surg Oncol 2012
++ ̴ 40% (6-46%) Risk of HGD/ malignancy 1
++++ ̴ 65% (57-92%) Risk of HGD/ malignancy in 5 y 1
++ 6-36% Prevalence malignancy 1
(+) VERY low (malignant = serous cystadenocarcinoma)
+ Low malignant potential 2
Variable 2
What factors determine malignant risk in IPMN/MCN?
Size
Histopathological type
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: PancreatitisImaging: Cyst > 3 cm
Thickened/enhancing cyst wallsMain duct size 5-9 mmNon-enhancing mural noduleAbrupt change in caliber of pancreatic ductwith 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
Operator-Dependent Investigation
Sampling Error
Contamination (gastric wall)
Low cellularity -> Low senstivity
e.g. SCN only 30-40% enough cells
diagnostic accuracy: 10-60%
often NON-diagnostic
What are drawbacks of EUS ?
Including high-grade
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
Mucin
Amylase
CytologyCytology negative or
Glyogen-con-taining cuboid
cells
mucin-
containing column cells
papillary clusters of
mucin-column cells,
atypia
Branching papillae
cuboid or cylindric cells, high cellularity, myxoid stroma
«dirty material»
Macrophages,Inflammatory cell
Viscosity Low High High NA Low
Mucin Low High High NA Low
Amylase < 250 U/L < 250 U/L < 250 U/La Low High
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
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:
1-2 cm:
2-3 cm:
> 3 cm:
CT/MRI in 2-3 years
Close surveillancealternating MRI with EUS every 3-6 monthsStrongly consider surgery (in young, fit patients)
EUS in 3-6 monthsLengthen interval, alternating EUS and MRIConsider surgery in young, fit patients (long surveillance)
CT/MRI yearly (for 2 years) lengthen interval if no change
Universitätsklinik für Viszerale Chirurgie und Medizin / www.chirurgiebern.ch
Which syndrome associates with multiple/oligocystic SCN ?
Hippel-Lindau-Syndrome