1. Cystic Neoplasms of the Pancreas: A Surgical Perspective
Great Debates & Updates in GI Malignancies
Cystic Neoplasms of the Pancreas: A Surgical Perspective
Michael A. Choti, MD
Department of Surgery
UT Southwestern Medical Center
Dallas, Texas

2. Disclosures
none

3. An Increasing Clinical Dilemma
Pancreatic Cysts:
An Increasing Clinical Dilemma
Pancreatic cysts are being diagnosed with increasing frequency
2.3% of CT routine CT scans identify cysts1
2.4% - 13% found on MRI scans2
The spectrum of malignant risk is variable and the management is unclear
What is the risk of malignancy?
How morbid is the invasive management?
Observe vs Operate?
1Laffan et al (AJR 2008)
2DeJong et al (Scan J Gastro 2012), Lee et al (AJG 2010)

5. Classification of Pancreatic Cysts
No Malignant Potential
Pseudocyst
Lymphoepithelial cyst
Retention cyst
Congenital cyst
Endometrial cyst
Cystic lymphangioma
Cavernous hemangioma
Serous cystic adenoma*
Malignant Potential
Intraductal papillary mucinous neoplasm
Mucinous cystic neoplasm
Intraductal tubular carcinoma
Malignant
Cystic ductal adenocarcinoma
Cystic neuroendocrine tumor
Solid pseudopapillary neoplasm
Cystic pancreatoblastoma
Cystic acinar cystadenocarcinoma
Mature cystic teratoma
*Low malignant potential
Law et al. (2013)

6. Classification of Pancreatic Cysts
Cystic
Pancreatic Lesions
Non-neoplastic
Pseudocyst
Retention Cyst
Foregut Duplication Cyst
Neoplastic
Cystic Degeneration of Solid Neoplasm
Serous
Mucinous
Solid Pseudopapillary Neoplasm (SPEN/Franz/Hamoudi)
Cystic
pNET
Serous Cystadenoma
Mucinous Cystic Neoplasm (MCN)
Intraductal Papillary Mucinous Neoplasm (IPMN)

7. Classification of Pancreatic Cysts
Patient History and Demographics
Age, gender, history of pancreatitis
Imaging Characteristics
CT, MRI, EUS
Fluid Characteristics
Cyst Aspiration – amylase, CEA (mucin), cytology

8. Clinical Classification
Cystic Degeneration of Solid Neoplasm
Solid Pseudopapillary Neoplasm (SPEN/Franz/Hamoudi)
Cystic
pNET

9. Solid Pseudopapillary Neoplasm (Franz or Hamoudi Tumor)
Very uncommon
~800 total cases in all case series up to 2008
Most common in young females
Imaging characteristics
Solid and cystic, large
Central calcification
No communication with the pancreatic duct
Cyst fluid features
Serous fluid (low CEA)
Low amylase
Loosely cohesive epithelial cells with focal hemorrhage
Positive staining for nuclear beta-catenin
Malignant potential
All should be resected
Reddy et al., JACS 2008

10. Cystic Neuroendocrine Tumors
10-15% pNETs are cystic
Behavior similar to typical pNET
Imaging features
Solid rim of arterial enhancement on CT
No communication with duct
Focal
Cyst fluid features
Serous (low CEA)
Low Amylase
Clear malignant potential
Management same as typical pNET
Bordeianou et al.

11. Clinical Classification
Serous Cystadenoma
Serous

12. Serous Cystic Neoplasms (Serous Cystadenoma)
10-15% of all cystic neoplasms
Predilection for females (60s)
80% in HOP, 80% asymptomatic
Imaging characteristics
Microcystic/honeycomb and loculated
Unifocal, Central calcification
No communication with the pancreatic duct
Cyst fluid features
Lined with simple cuboidal or flattened epithelium
Thin serous fluid
Very low malignant potential
Resect only when symptomatic
Galanas et al., JOGS 2007

13. Clinical Classification
Mucinous
Mucinous Cystic Neoplasm (MCN)
Intraductal Papillary Mucinous Neoplasm (IPMN)

14. Aram F. Hezel et al. Genes Dev. 2006; 20: 1218-1249

15. Mucinous Cystic Neoplasm (MCN)
Relatively uncommon (1/5th of resected cysts)
Predilection for middle-aged women
95% female, median age 45
Imaging characteristics
“Macrocystic”, unifocal
Well demarcated
Body/Tail
No communication with Pancreatic Duct
Cyst fluid features
Mucinous (high CEA)
Low amylase
Ovarian-type stroma
Malignant association (6-36%)
All should be resected
Crippa et al., Annals of Surgery, 2007

16. IPMN Relatively common
Occur in both males and females with equal frequency
Tend to occur in the elderly
Imaging characteristics
Ductal dilatation often present
Often multifocal, macrocystic
Communicate with duct
Cyst fluid features
Mucin (high CEA)
High Amylase
Malignant potential
Dependent on further sub-classification

17. IPMN Classified by Relationship to Pancreatic Duct
Main Duct IPMN
Branch Duct IPMN

18. Cross-Sectional Imaging
Pancreas Protocol CT
Non-invasive
Fast
Relatively inexpensive
Extremely accurate in demonstrating defining characteristics
Limitations
Involves radiation
Solid Component
MRI
Non-invasive
Extremely accurate in demonstrating defining characteristics
Sensitive in determining solid component
Limitations
Less readily available than CT
Relatively Expensive

19. Endoscopic Ultrasound
Extremely accurate in demonstrating defining characteristics
Gold standard for identifying a solid component
Able to obtain fluid and tissue (Cyst aspiration, FNA or Core biopsy)
Limitations
More invasive
Expensive for routine screening

20. IPMN with Mural Nodule on EUS
Marchegiani & Fernandez-del Castillo. Adv Surg 48 (2014)

21. Cyst Aspiration & Biopsy
FNA or Core Biopsy
Level of dysplasia
Able to perform IHC
Limitations
Often indeterminate
Loss of cytoarchitecture
False negatives(dysplasia/cancer)
Cyst Aspiration
Helpful in differentiating serous from mucinous
Solid component
CEA level
Genetic analysis
“The Future”
Limitations
Highly operator dependent
Often cannot obtain fluid
GI tract contamination

22. Mutations Associated with Cyst Fluid
Law/Hruban et al. (2013)

23. International Consensus Guidelines for Management of IMPN and MCN of the Pancreas
Tanaka, Pancreatology, 2012

24. International Consensus Guidelines for the Management of MCN and IPMN (“Sendai Criteria”) Indications for Surgical Resection
Main Duct IPMN
Branch Duct IPMN
Defined as diffuse ductal dilatation > 3 mm at the neck of the pancreas
All Main Duct IPMN are resected in a patient who can tolerate an operation
Symptoms
Pain, jaundice weight loss, pancreatitis
Rapid Growth
0.5 cm/6 months
Size > 3 cm
Solid Component (mural nodule)
Tanaka, Pancreatology, 2006

25. 62 year old female No history of pancreatitis CT Scan Cyst fluid
Unifocal
Tail lesion
Non-dilated pancreatic duct
Cyst fluid
Mucinous (high CEA)
Low amylase
Diagnosis: MCN

26. 66 year old male Non-specific abd pain CT Scan Cyst fluid
Multi-focal
HOP lesion
Mildly dilated pancreatic duct
Cyst fluid
Mucinous (high CEA)
high amylase
Diagnosis: IPMN

27. 45 year old female History of breast cancer CT Scan Cyst fluid
Unifocal-focal
TOP lesion
Non-dilated pancreatic duct
Cyst fluid
serous (low CEA)
low amylase
Diagnosis: IPMN

28. Summary
Pancreatic cysts are being identified with increasing frequency.
Significant variability exists in the malignant potential and management recommendations of pancreas cysts.
While advances have been made in imaging and molecular assessment, current tools for differentiating high risk patients and guiding us in management and surveillance are limited.
Currently, mucinous neoplasms meeting the guideline criteria (Senai) should be resected.
Presumed IPMN must be followed closely.
Patients with pancreatic cysts benefit from a multidisciplinary team approach, which includes gastroenterologists, pancreatic surgeons, radiologists, cytopathologists and pathologists.

29. Thank You