This document provides guidelines for the diagnosis and management of cystic pancreatic lesions. It discusses various types of cystic masses that can occur in the pancreas such as pseudocysts, serous cystadenomas, mucinous cystic neoplasms, intraductal papillary mucinous neoplasms (IPMN), and solid pseudo-papillary tumors. For each type, it provides information on characteristics, malignant potential, imaging appearance, and treatment approach. Initial evaluation of pancreatic cysts should aim to exclude pseudocysts based on history of pancreatitis. Morphological evaluation and cyst fluid analysis via EUS and FNA are important diagnostic tools to characterize cyst type and guide management.
3. True simple cyst in pancreas
Probably congenital cystic lesion
• Thin walled lesion, no intra-luminal excrescence or solid
compound, large amounts of clear fluid, no connection with PD
• Mostly occur in infants & younger patient
• Polycystic disease, fibrocystic disease, or von Hippel-Lindau
• Essentially benign & mostly remain asymptomatic
• Final diagnosis made by histological study
Heindryckx E et al. Eur Radiol 1998 ; 8 : 1627 – 1629.
4. True simple cyst of pancreas
Heindryckx E et al. Eur Radiol 1998 ; 8 : 1627 – 1629.
Uniloculate cyst in pancreatic
head
CT scan
Pancreatic cyst lined by mono-layer
of cylindrical/cuboidal epithelium
Histological specimen
5. Screening of ADPKD
Renal US & DNA analysis in 319 patients at risk
Ravine D et all. Lancet 1994 ; 343 : 824 – 827.
Nicolau C et al. Radiology 1999 ; 213 : 273 – 276.
Person at risk & younger than 30 years
Two cysts in one kidney or one cyst in each kidney
Person at risk & aged 30 – 59 years
Two cysts in each kidney
Person at risk & aged 60 years or older
Four cysts in each kidney
Sen: 95% (ADPKD-1) – 65% (ADPKD-2)
(Sen: 100%)
(Sen: 100%)
6. Autosomal dominant polycystic kidney disease
Small cyst in body of pancreas
Enlarged polycystic kidneys & small hepatic cysts
41-year-old man with ADPKD
Demos TC et al Am J Roentgenol 2002 ; 179 : 1375 – 1388.
7. von Hippel-Lindau disease
Rare disease (prevalence 1/ 35.000 – 40.000)
• Autosomal dominant disease with high penetrance
• Development of variety of benign & malignant tumors
• Broad clinical manifestations: 40 lesions in 14 organs
• Diagnostic criteria
More than one CNS hemangioblastoma
One CNS hemangioblastoma & visceral manifestations
Any manifestation & familial history of VHL disease
13. Initial evaluation of a pancreatic cyst should be
directed toward exclusion of a pseudocyst
Patients with pseudocyst generally have a history
of acute or chronic pancreatitis, whereas those
with cystic tumors most often lack such a history
14. Pancreatic pseudocyst
EUS of pancreatic pseudocyst with dependent layering debris
Fasanella KE et al. Best Pract Res Clin Gastroenterol 2009 ; 23 : 35 – 48.
15. Calcified pseudocyst
Demos TC et al. Am J Roentgenol 2002 ; 179 : 1375 – 1388.
44-year-old man with chronic pancreatitis
Pseudocyst with calcified wall in head of pancreas
When pseudocysts are chronic, the wall can calcify
16. Pancreatic abscess
30-year-old man with a history of pancreatitis
Rim enhancing fluid collection with multiple foci of internal air
Axial CECT
Molvar C et al. Curr Probl Diagn Radiol 2011 ; 40 : 141 – 148.
In absence of gas, differentiation of abscess from necrosis
or simple fluid is not possible with imaging
17. Gas in pancreatic bed
• Pancreatic abscess
• Pancreatic-enteric fistula
• Previous internal pseudo-cyst drainage
• Previous drainage of pancreas by pancreatico-jejunostomy
Demos TC et al. Am J Roentgenol 2002 ; 179 : 1375 – 1388.
Pancreatic gas is not pathognomonic of an abscess
18. Pancreatic-enteric fistula
Thin-walled collection with
air-fluid level in pancreatic tail
Asymptomatic 58-year-old man
Several weeks after episode of acute pancreatitis
Fistula between colon &
pancreas as source of gas
Demos TC et al. Am J Roentgenol 2002 ; 179 : 1375 – 1388.
19. Pancreatic hydatid cyst
Rare: < 1% of cases
Acute pancreatitis due to pancreatic hydatid cyst: 9 reported cases
Pancreatic cystic mass of 10 cm
Calcified wall & daughter cysts
Left pancreatectomy with splenectomy
Wall cyst & daughter cysts
Makni et al. World J Emergency Surg 2012 ; 7 : 7 - 10.
38-year-old man presented with acute pancreatitis
20. Pancreatic tuberculosis
Complex solid/cystic mass
encasing PV & CBD
CT scan
Heterogeneous green
predominant pattern
Tissue elastographyEUS
Large hypoechoic LN
With central necrosis
EUS-FNA: Granuloma – Positive AFB culture
Chatterjee S et al. J Gastrointestin Liver Dis 2012 ; 21 : 105 – 107.
25. Serous cystadenoma
Microcytic lesion with central scar
Diagnostic of benign serous cystadenoma
T2-weighted MRI
Sahani DV et al. J Am Coll Radiol 2009 ; 6 : 376 – 380.
26. Serous cystadenocarcinoma
• 26 published cases of serous cystadenocarcinoma
• Mean age at diagnosis 68 2 years (range: 52 to 81)
• Women affected more commonly: 2:1
King JC et al. J Gastrointest Surg 2009 ; 13 : 1864 – 1868.
Small but finite risk of malignancy for serous cystic
neoplasms of pancreas
27. Mucinous cystic neoplasm
Sahani DV et al. J Am Coll Radiol 2009 ; 6 : 376 – 380.
Large septated macrocyst in pancreatic tail of middle-age women
Typical of mucinous cystic neoplasm
Contrast-enhanced multi-detector CT
28. Mucinous cystic neoplasm
Khalid A et al. Am J Gastroenterol 2007 ; 102 : 2339 – 2349.
Hypodense lesion in body of pancreas
Internal septations & wall calcifications suggesting MCN
Contrast-enhanced CT scan
29. Mucinous cystic neoplasm
Fasanella KE et al. Best Pract Res Clin Gastroenterol 2009 ; 23 : 35 – 48.
Unilocular cystic lesion
in pancreatic tail
CT scan EUS
Small septation/‘daughter cyst’
Posterior cyst enhancement
30. Morphologic classification of IPMN
Branch pancreatic duct
Sahani DV et al. Clin Gastroenterol Hepatol 2009; 7 : 259 – 269.
Diffuse main pancreatic duct
Segmental main pancreatic duct
Mixed (main & branch ducts)
31. Prevalence of cancer in IPMNs
• Main duct IPMN 60 – 90%
• Branch duct IPMN 5 – 45%
Sahani DV et al. Clin Gastroenterol Hepatol 2009; 7 : 259 – 269.
32. Intraductal papillary mucinous neoplasm
Endoscopic view
Fish mouth deformity
Secondary to mucin overproduction & extrusion
Pathognomonic for IPMN
Talley NJ , Kane SV, & Wallace MB. Practical Gastroenterology & Hepatology:
Small & Large Intestine & Pancreas. Blackwell Publishing, 1st edition, 2010.
34. Main duct IPMN
79-year-old man with prostate cancer & rising PSA underwent CECT
Total pancreatectomy → High risk of associated adenocarcinoma
Zaheer A et al. Abdom Imaging 2012 in press.
Markedly dilated main pancreatic duct
Dilation of main duct ≥ 1 cm strongly suggests IPMN
35. Main duct IPMN
Sahani DV et al. Clin Gastroenterol Hepatol 2009; 7 : 259 – 269.
EUS of 66-year-old man with non-specific symptoms
Dilated main pancreatic duct with mural nodule
36. Pre-surgical follow-up according to IPMN size
Sahani DV et al. Clin Gastroenterol Hepatol 2009; 7 : 259 – 269.
EUS
1 cm: 1 year
1 – 2 cm: 6 – 12 months
2 – 3 cm: 6 months
Malignant
Resection
Benign
Follow-up Resection
Suspicious features
Increased size > 3 cm
> 1 cm growth per year
Mural nodules
Main duct dilatation
Solid component
Thick wall/septations
37. Major features of cystic pancreatic neoplasms
Pseudocyst SCN MCN IPMN
Age Variable Middle age Middle age Elderly
Sex M > F F > M F M > F
Alcohol abuse Yes No No No
History of pancreatitis Yes No No Frequent
Malignant potential None Very rare Moderate to high Low to high
Barresi L et al. World J Gastrointest Endosc 2012 ; 4 : 247 – 259.
38. EUS/FNA of cystic pancreatic neoplasms
Brugge WR. Gastrointest Endosc 2009 ; 69 (suppl): 203 – 209.
Pseudocyst SCN MCN IPMN
Location Evenly Evenly Tail Head
Cytology Pigmented
histiocytes
Bland PAS + Mucinous Mucinous
Viscocity Low Low Increased High
Cystic amylase High Low Low High
Cystic CEA < 200 ng/mL < 0.5 ng/mL > 200 ng/mL > 200 ng/mL
K-RAS mutations Negative Negative Positive Positive
39. Traditional therapeutic approach to
management of cystic lesions
Pseudocyst Serous Mucinous Malignant
Head Drain Monitor Monitor Resect
Body Drain Monitor Resect Resect
Tail Resect Resect Resect Resect
Brugge WR. Gastrointest Endosc 2009 ; 69 (suppl): 203 – 209.
40. EUS evaluation of cystic lesions of pancreas
• Morphologic analysis Microcystic
Macrocystic
Associated mass
• FNA Complete evacuation if possible
Antibiotic prophylaxis
• Cyst fluid analysis Amylase
CEA
Cytology
Brugge WR. Gastrointest Endosc 2004 ; 59 : 698 – 707.
Interpretation in conjunction with history & CT scanning
41. Solid pseudo-papillary tumor
• Infrequently-encountered tumor
• Typically affects young women without significant symptoms
• Its behavior relatively indolent & largely benign
• Patients may survive long time after radial resection
• If possible, surgery justified for local invasion or metastasis
• Prognosis even with unresectable metastasis is good
• Role of chemo & radiotherapy remains to be studied
Yu PF et all. World J Gastroenterol 2010 ; 16(10): 1209 – 1214.
45. Pancreatic adenocarcinoma with cystic degeneration
Infiltrative lesion in head of pancreas involving SMA & SMV
Obstructed–dilated pancreatic duct
Axial CECT image
Khan A et al. Am J Roentgenol 2011; 196 : W668 – W677.
46. Cystic insulinoma
Small cystic masses in body & tail of pancreas
36-year-old woman with MEN-1
Patient presented with primary hyperparathyroidism
Demos TC et al. Am J Roentgenol 2002 ; 179 : 1375 – 1388.
47. Cystic glucagonoma
34-year-old woman presented with diabetes
& necrolytic migratory erythema
Large cystic mass in tail of pancreas
Demos TC et al. Am J Roentgenol 2002 ; 179 : 1375 – 1388.
48. Cystic nonfunctioning neuro-endocrine neoplasm
Demos TC et al. Am J Roentgenol 2002 ; 179 : 1375 – 1388.
65-year-old man
Cystic elements in neoplasm extending from tail of pancreas
49. Metastatic renal cell carcinoma
Demos TC et al. AJR 2002 ; 179 : 1375 – 1388.
Large cystic mass with irregularly thickened wall in body & tail
Note similarity to RCC of right kidney
70-year-old woman with RCC
50. Pancreatic schwannoma
Mummadi RR et al. Gastrointest Endoscopy 2009 ; 69 : 341.
7-cm multiloculated cystic pancreatic body mass
Thick septations with many solid areas
More consistent with a cystic neoplasm
EUS-FNA not performed & surgical resection performed
Linear EUS
51. Pancreatic arteriovenous malformation
Yamamoto T et al. J Clin Ultrasound 2000 ; 28 : 365 – 367.
Pulsatile color signals with low resistance
Color & pulsed Doppler US
Cystic pancretic lesions
Gray-scale US
Racemose hypervascular
network
Celiac arteriogram
Early filling of PV
52. Conclusion
Discriminate benign lesions from those that require surgery
• Clinical history is essential to suggest diagnosis
• CT gives critical information: size, septations, ductal
dilatation, calcifications, mural nodules, etc.
• MRI/MRCP recommended as next imaging study in:
Equivocal differentiation between cystic & solid lesions
Evaluation of subtle enhancement
Communication with pancreatic duct
• EUS morphology, fluid analysis & cytology aid in diagnosis
Zaheer A et al. Abdom Imaging 2012 in press.
Barresi L et al. World J Gastrointest Endosc 2012 ; 4 : 247 – 259.
53. Conclusion
• No single test accurate enough to make sure diagnosis
• Diagnosis of cystic pancreatic lesion is a puzzle
Bits of information deriving from demography, clinical
history, radiology, EUS & intracystic fluid analyses
Barresi L et al. World J Gastrointest Endosc 2012 ; 4 : 247 – 259.
Renal US & DNA analysis for ADPKD were performed in 319 patients who were at risk. PKD1: short arm of chromosome 16 – Account for 85 – 90 % of population with ADPKD.PKD2: Long arm of chromosome 4 – Account for 10 – 15% of population with ADPKD.In some other families, no linkage to either PKD1 or PKD2 has been reported.