Radiological Analysis of Cystic Lesions of the Pancreas

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

Radiological Analysis of Cystic lesions of the Pancreas

Shruthi Mahalingaiah, Harvard Medical SchoolYear III, Gillian Lieberman, MD

September 2002

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Agenda

• Background • Anatomy and histology• Radiological workup of a cyst in the

pancreas• Patient presentations • Summary

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Background

2.2-2.4% of all cancers are pancreatic

4.7-5.5% deaths from pancreatic cancer

5-10% of all pancreatic neoplasms are of the cystic variety

Adapted from Landis, SH, et al: Cancer Studies. CA 48:6, 1998

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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The Pancreas: Anatomy

“Here lies the fickle romance of the

abdomen; the pancreas lies with her head in the

arms of the duodenum while her feet tickle the

spleen.”

–NJ Mizeres

Plate255 Netter’s Atlas of Human Anatomy

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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The Pancreas: Anatomy

•Retroperitoneal

• “Hidden”

•Virtually impossible to palpate

•Radiographic studies allow greater visualization and understanding of disease of the pancreas

Plate279 Netter’s Atlas of Human Anatomy

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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The Pancreas: Ductal Anatomy

• Pancreatic ductal system forms from dorsal and ventral buds

•Wirsung and Santorini named after fusion

•Failure of fusion results in Pancreas divisum, which increases susceptibility to pancreatitis

Plate279 Netter’s Atlas of Human Anatomy

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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The Pancreas: Ductal Anatomy

Plate279 Netter’s Atlas of Human Anatomy

•2.5- 3L of alkaline fluid daily

•Ave 15 cm in length

•Apposed to duodenum, stomach, spleen, great vessels

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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The Pancreas: Histology

Large reservoir of both endocrine and exocrine function.

Disease becomes symptomatic only when severe impairment occurs

85% exocrine

Gartner, LP, Hiat, JL, Color Atlas of Histology

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Cystic lesions of the Pancreas

• Retention Cysts

• Pseudocysts• Cystic

Neoplasms

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Cystic lesions of the Pancreas

• Retention Cysts: No clinical significanceSmall, developmental,

fluid filled, lined by normal duct or acinar cells.

• Pseudocysts• Cystic Neoplasms

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Cystic lesions of the Pancreas

This fifteen minute presentation focuses on cystic pancreatic neoplasms with a brief discussion of pancreatic pseudocysts. For further discussion

of general cystic lesions and a more encompassing differential, please refer to the

reference section.

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Pseudocysts• secondary to

inflammation and necrosis

• located within or outside the pancreas

• Lining contains fibrous and granulation tissue

• Lack of epithelial lining• Present >6wks

Cystic lesions of the Pancreas: Pseudocyst

from ACR teaching files

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Pseudocysts• Pyloric Antrum• Remarkably

distended C-loop of duodenum

• Large Mass CT for further evaluation

Cystic lesions of the Pancreas: Pseudocyst

from ACR teaching files

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Pseudocysts• Secondary to

pancreatitis • Variable size• located within or

outside the pancreas

• Present >6 wks

Cystic lesions of the Pancreas: Pseudocyst

John Kruskal, M.D, Ph.D, uptodate.com

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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• Serous: Microcystic• Cystadenoma/ cystadenocarcinoma

• Mucinous: Macrocystic• Cystadenoma/ cystadenocarcinoma• Intraductal papillary mucinous tumor (IPMT)

• Zebras

Cystic Neoplasms of the Pancreas

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Survey of 9 Patients with Cystic Disease

• 7/9 cystic lesions were incidental findings

•4/9 MRI

•2/9 US

•1/9 CT

•

2/9 lesions in pancreas were suspected from patients’

history

CT is often initial modality in which neoplastic

cyst is found.

Silas, AM, Morrin, MM, Ratatopoulos, V, Keogan, MT, Intraductal

Papillary Mucinous Tumor, AJR. 176(1):179-85, 2001, Jan

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Work-up of an incidental cyst

Cyst found incidentally• CT

• US

• ERCP

• MRI

Focused Radiological Study

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Work-up of an incidental cystCyst found incidentally

• CT provides density of differentiation of intraductal and cystic spaces

•Septa may be appreciated with contrast enhancement

•Sensitive to Ca+ can detect pathognomonic stellate calcific lesion of serous cystadenoma

•Allows for non-invasive CT follow up

Focused Radiological Studies

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Work-up of an incidental cystCyst found incidentally Ultra Sound

•Non-invasive

•More sensitive for finding septa than CT

•Often used for follow up of pseudocyst

•US guided biopsy

•Fast, cheap

•Operator dependent

Focused Radiological Study

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Work-up of an incidental cystCyst found incidentally ERCP

•clarifies non-specific findings

•Visualize filling defects

•Observe mucinous extrusions from ampulla of Vater in cases of IPMT

•Allows sampling of ductal lining cells

•Risk of pancreatitis

Focused Radiological Study

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Work-up of an incidental cystCyst found incidentally •MRI

•Non-invasive

•Better display of subtle abnormalities- of complex cystic masses, cavities, septae, and nodules

•MRCP may be able to replace ERCP in the future

Focused Radiological Study

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Work-up of an incidental cyst

Enzyme richHx-recent pancreatits

fluid thin and wateryMultiple small or microcysts

•Mucinous aspirate

•One or macrocystic spaces

Cyst found incidentally

Focused Radiological Study

Fluid Aspiration/Tissue Biopsy

DDX

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Serous Cystadenoma/ Cystadenocarcinoma

• Second most common cystic tumor of the pancreas•Middle aged women•Equal distribution throughout the pancreas•Mass may be quite large and produce symptoms from organ displacement.•Microcystic: Many small cysts lined by glycogen-rich cells •Lining is often denuded•Low malignant potential

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Patient KS

65 y/o female with epigastric pain, nausea, and vomiting.

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Abdominal Plain Film Patient KS

Courtesy: Dr. H. Gramm

• Stellate

calcification• Possibly renal• Possibly pancreatic• CT for further eval.

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Computational Tomography (CT) of the Abdomen Patient KS

Courtesy: Dr. H. Gramm

•

Stellate

calcification in pancreas

• tiny cysts in a large

multilobulated

mass lesion

•Some cysts are confluent

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Reconstruction CT of the Abdomen Patient KS

Courtesy: Dr. H. Gramm

• Symptoms from mass effect

impingement on stomach and

duodenum

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Correlation of KUB to CT Patient KS

Courtesy: Dr. H. Gramm

Serous CystadenomaMicrocystic

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Radiological Parameters of Serous Cystadenoma

Courtesy: Dr. H. Gramm

Serous CystadenomaMicrocystic

At least 4-6 cm

If multiloculated contains no more than 6 loculations each less than 2cm

Highly vascular on angio

Lined with evenly spaced, flat, cuboidal to polygonal cells

Cyst contains abundant glycogen

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Correlation of KUB to CT Patient KS

Courtesy: Dr. H. Gramm

Serous CystadenomaMicrocystic

www.uptodate.com

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Mucinous

Cystadenoma/ Cystadenocarcinoma

•Most common cystic neoplasm of the pancreas•Typically affects middle aged women•Occurs in in body or tail of pancreas•Presents with a mass lesion composed of one or more macrocytic

spaces

•Lined by mucous secreting cells•Lining is often denuded (hence difficult to make diagnosis even with pathology)•High malignant potential•Most are malignant at time of diagnosis

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Patient MG

45 y/o woman with abdominal pain

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Single Contrast Barium Study Patient MG

Courtesy: Dr. H. Gramm

•Impressive displacement of Small Bowel to RLQ

*

*

* Body of Stomach

* Antrum of stomach

CT for follow up Eval.

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Abdominal CT Patient MG

Courtesy: Dr. H. Gramm

* unilocular

cystNo septa!

•Thin rim

•No fat strandingin surroundings

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Correlation of Single-contrast Ba and CT Patient MG

Courtesy: Dr. H. Gramm

*

Mucinous

cystadenomaMacrocystic

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Gross Pathology Patient MG

Courtesy: Dr. H. Gramm

*

Mucinous

cystadenomaMacrocysticFrom: www.uptodate.com

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Intraductal

Papillary Mucinous

Tumor (IPMT)

• “mucinous duct ectasia”•Dilated ductal segments usually within the head of the pancreas•Hyperplasia and dysplasia of mucin producing columnar epithelium. •Formation of papillary projections may either protrude into pancreatic duct or remain in branch ducts•Duct obstruction from mucin plug or compression from cystic mass•High malignant potential•More common in elderly men •Can have thick mucous extruding from ampulla of Vater•Patients can present with repeat episodes of pancreatitis

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Patient JT

45 y/o male with abdominal pain

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Computational Tomography (CT) of the Abdomen Patient JT

Pancreas

Cyst

Courtesy: Dr. H. Gramm

Dilated ductof Wirsung

communicates with cystLumen of duodenum

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Endoscopic Retrograde Cholangiopancreatography Patient JT

Reveals Cyst

Filling defects

Courtesy: Dr. H. Gramm

Typically can obs.Mucin extruding fromPapilla of Vater

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Correlation of (ERCP) with CT Patient JT

Pancreas

Cyst

Courtesy: Dr. H. Gramm

IPMT-Main Type

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Radiological Parameters for IPMT

Pancreas

Cyst

Courtesy: Dr. H. Gramm

IPMT-Main Type

Malignancy Highly Suspected:

Filling defects Diffuse main duct dil > 15mm

Side branch > 3 cm

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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IPMT Variants

Main Type

Main pancreatic duct +/- side branch involvement consider resection

Side Brianch Type

Side branch involvement onlyif lesion is less than 2.5 cm follow up with MRI

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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T1 W MRI Side Branch Variant

Courtesy: Dr. M. Morrin

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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T1 W MRI Side Branch Variant

* Liver

*IVC

*Aorta

*SMAPancreas

Courtesy: Dr. M. Morrin

*

* **

(fat bright; fluid dark)

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Ultrasound Side Branch Variant

Cysts on side branches

Not communicating with main pancreatic duct

Courtesy: Dr. M. Morrin

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Mucin

Producing Zebras of the Pancreas

Rare cystic neoplasms

also included in differential:• Papillary Cystic Tumor of the pancreas• Cystic Islet Cell tumor• Pancreatic Sarcoma• Pancreatic Cysts associated with

•Von Hippel-LindauVery Rare•Cystic teratoma (very rare)• Enteric cyst of the pancreas (very rare)•Lymphoepithelial cysts•Cystic lymphangioma

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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Pseudocysts

Cystic Neoplasms: • Serous cystadenoma• Mucinous Cystadenoma/ cystadenocarcinoma• Intraductal papillary mucinous tumor

Imaging Modalities

Cystic lesions of the Pancreas: Summary

Shruthi Mahalingaiah, HMS IIIGillian Lieberman, MD

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References

•Feldman: Sleisenger & Fordtran’s Gastrointestinal and Liver Disease, 6th

ed., 1998: WB. Saunders, p.896-899.•Grogan JR, Saeian K, Taylor AJ, Quiroz F, Demeure MJ, Komorowski R. Making Sense of Mucin Producing Pancreatic Tumors. AJR 2001; 174(4): 921-9.•Gartner JP, Hiatt JL. Color Atlas of Histology, 2nd ed., 1994: Williams and Wilkins, p.280.•Silas AM, Morrin MM, Raptopoulos V, Keogan MT. Intraductal Papillary Mucinous Tumors of the Pancreas. AJR 2000; 176: 179-185.•Netter, FH. Atlas of Human Anatomy, 2nd ed., 1997:Novartis, plates: 255, 279.•Steer, MJ. Cystic Lesions of the Pancreas, June 11,2002 (last updated)•www.uptodate.com•Van Houten T, et al. Anatomy Dissector, 2002: Unpublished. Chapter 12: Biliary System, Pancreas, and Spleen.

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Acknowledgements

Dr. Herbert Gramm, MD

Dr. Robert Ronan, MD

Dr. Martina Morrin, MD

Dr. Chad Brecher, MD

Dr. Gillian Lieberman, MD

Early morning gang-James, Eduardo, Jon

Pamela Lepkowski

Michael Larson

Larry Barbaras and Cara Lyn D’amour