Xanthogranulomatous cholecystitis vs Gallbladder...

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Xanthogranulomatous Cholecystitis: Ultrasound, CT, and MRI findings Julia T. Chu, HMS IV Laura Avery, M.D. Gillian Lieberman, M.D. Ultrasound MRI CT

Transcript of Xanthogranulomatous cholecystitis vs Gallbladder...

Xanthogranulomatous Cholecystitis:

Ultrasound, CT, and MRI findings

Julia T. Chu, HMS IVLaura Avery, M.D.

Gillian Lieberman, M.D.

Ultrasound MRICT

Agenda

Patient:

53yo M with RUQ abd

pain•

DDx: RUQ abd

pain

Imaging Modalities: available to image our patient•

Radiologic Findings:

US, CT, MRI

gangrenous cholecystitis, adenomyomatosisreview of anatomy and pathophysiology

Pathology Dx: Xanthogranulomatous

cholecystitis•

Management: Depends on radiologic Dx!

Take-Home Points

Our Patient: History & Physical Exam

Hx:

53yo M with intermittent RUQ abd

pain for 2 years; no fever/chills, nausea/vomiting, weight loss, or food association

PMH:

DM 2, hyperlipidemia, HTN•

Meds:

Metformin, ASA, lisinopril, atorvastatin

SH:

Plumber married w/ children; (+) smoking, (-) EtOH

Exam:

(+) Murphy sign, (+) guaiac•

Labs:

Leukocytosis, ↑

Alk

Phos, ↑

LFTs, ↑

GGT

VascularInfarct

Pyelophlebitis

Mesenteric thrombosis

Adrenal infarct

Occlusion

Embolism

Renal vein thrombosis

Clinical DDx:

RUQ abd

pain (by mnemonic)

“V I N D I C A T E”

Inflammation/InfectionCellulitis, Osteomyelitis

Diaphragmatic abscess

Trichinosis, TB, Herpes zoster

Hepatitis, Hepatic abscess

Cholecystitis, Cholangitis

Duodenitis, Diverticulitis, Colitis

Pancreatitis, Pyelonephritis

Ulcer, Mesenteric adenitis

Waterhouse-Friderichsen

syndrome

NeoplasmCarcinoma

Cholangioma

Pancreatic carcinoma

Hodgkin disease

Lymphosarcoma

Neuroblastoma

Adrenal carcinoma

Multiple myeloma

Intoxication/ IdiopathicAlcoholic hepatitis

Ulcer

Gout

DegenerativeOsteoarthritis

Allergic/ AutoimmuneRheumatoid spondylitis

Congenital/

Acquired AnomalyVentral hernia

Incisional

hernia

Diverticulum

Obstruction

Cyst

Hydronephrosis

TraumaContusion

Cough

Hemorrhage

Laceration

Rupture

Herniated disc

Spine fracture

EndocrineHyperparathyroidism

www.wrongdiagnosis.com

Gallbladder carcinoma

Cholecystitis

and cholelithiasis

Hepatic flexure syndrome

Carcinoma of the colon with obstruction

ColitisDiverticulitis

Pyelonephritis

Embolic nephritis

Renal calculus

Carcinoma of the pancreas

Pancreatic calculus

Pancreatitis

Duodenal ulcer

Common duct stone

Cholangitis

LacerationBudd-Chiari

syndromeCarcinoma

Subphrenic

abscess

Hepatitis

Liver abscess

DDx:

RUQ abd

pain (by anatomy)

Legend:

Liver Pancreas

Bile duct Small bowel

Gallbladder Large Bowel

Renal System Others

Budd-Chiari

syndrome

Liver abscess

Laceration

Hepatitis

Carcinoma

Common duct stone

Cholangitis

Pancreatitis

Pancreatic calculus

Carcinoma of the pancreas

Duodenal ulcer

ColitisDiverticulitis

Carcinoma of the colon with obstruction

Hepatic flexure syndrome

Cholecystitis

and cholelithiasis

Pyelonephritis

Embolic nephritis

Renal calculus

Gallbladder carcinoma

Subphrenic

abscess

Pneumonia/empyema

pleurisy

www.wrongdiagnosis.com

Our patient’s main DDx, based on:

RUQ pain

(+) Murphy Sign

leukocytosis,

would be most likely centered on which organ?

Thus, would involve which conditions?

Imaging Modalities: Available/Applicable to Our Patient

with RUQ pain, ↑

WBC, (+)Murphy•

Ultrasound (US): abdomen/gallbladder

to look for gallstones, aneurysm•

Nuclear Medicine: cholescintigraphy

(or

HIDA scan)

with or w/out cholecystokinin

to evaluate the function of the gallbladder and the bile ducts

X-ray: Upper GI series

to rule out stomach/duodenum conditions; abdomen; colon barium enema; chest x-ray to rule out pneumonia

Computed Tomography (CT): abdomen to further evaluate the gallbladder for mass/carcinoma as well as other abd

organs such as the nearby pancreas

Magnetic Resonance Imaging (MRI): T1 with fat saturation, T2 to assess soft tissue changes such as fluid, inflammation, edema; MR cholangiopancreatography

(MRCP) to visualize the biliary

tract and pancreatic ducts

Invasive: cholangiography, percutaneous

cholecystostomy, endoscopic retrograde cholangiopancreatography

(ERCP)

American College of Radiology, www.acr.org

Arrive at Our Dx, Step by Step …

Clinical DDx:•

Cholecystitis•

Cholelithiasis•

Choledocholithiasis•

Cholangitis•

Hepatitis•

Pancreatitis

Imaging: Ultrasound

H&P:•

Hx

RUQ abd

pain•

Exam –

(+) Murphy sign•

Labs –

Leukocytosis

Our Patient: Findings on Ultrasound

Patient

Marked irregular GB wall thickening √

Cholelithiasis

with (+) US Murphy sign

Abd

aorta

Impression:

Gangrenous cholecystitis

vs GB carcinoma

Partners CAS

Normal Liver

Gallbladder

Courtesy of Dr. MaryEllen

Sun (BIDMC PACS)

Hyperechoic

fatty liver with abnormality in the region contiguous to gallbladder

Film Findings: hyperechoic

fatty liver, markedly thickened gallbladder wall, cholelithiasis

with (+) US Murphy sign

SagittalSagittal

Arrive at Our Dx, Step by Step …

Clinical DDx:•

Cholecystitis•

Choledocholithiasis•

Cholangitis•

Hepatitis•

Pancreatitis

Imaging: CT to evaluate gallbladder wall thickening vs “mass”; why?

gallbladder carcinoma has a poor prognosis of 85% mortality within 1 year of diagnosis

need to further evaluate the US findings with more imaging studies before embarking on any treatment

H&P:•

Hx

RUQ abd

pain•

Labs –

Leukocytosis•

Exam –

(+) Murphy sign

US DDx:•

Gangrenous cholecystitis•

Gallbladder carcinoma

US Findings:•

Irregular gallbladder wall thickening

Our Patient: Findings on CT scan

Partners CAS

Axial, oral C+

Cystic structure

Cystic duct

Common hepatic duct

Common bile duct

Gallbladder

Neck

BodyFundus

www.wiltshiresurgery.com

Heterogeneous low density in the adjacent liver

Irregular wall thickening involving the gallbladder fundus

Film Findings:

Irregularly thickened wall at the gallbladder fundus, low attenuation in liver adjacent to the gallbladder, cyst at the fundus.

Partners CAS

Impression:

CT findings suspicious for malignancy. Infection much less likely given no pericholecystic

fluid

or inflammation.

Our Patient: Pertinent negative findings on CT scan

Coronal, oral and IV C+

No wall thickening in the inferior and medial aspect of the gallbladder

No pericholecystic

fluid or inflammation

No intra or extrahepatic

biliary

ductal

dilatation

Cystic structure

Irregular wall thickening involving the gallbladder fundus

Arrive at Our Dx, Step by Step …

Clinical DDx:•

Cholecystitis•

Choledocholithiasis•

Cholangitis•

Hepatitis•

Pancreatitis

CT Findings: •

Irregular wall thickening at the gallbladder fundus•

Cystic structure at the gallbladder fundus•

No pericholecystic

fluid or inflammation•

No biliary

ductal

dilatation

H&P:•

Hx

RUQ abd

pain•

Labs –

Leukocytosis•

Exam –

(+) Murphy sign

US DDx:•

Gangrenous cholecystitis•

Gallbladder carcinoma

US Findings:•

Irregular gallbladder wall thickening

CT DDx: gallbladder malignancy

Imaging: MR to further evaluate soft tissue changes in the gallbladder and the adjacent liver to assess inflammatory changes and confirm or rule out malignancy

Our Patient: Findings on MR imaging

Axial T1-weighted Gradient Echo with Fat Sat; Post-Gadolinium Arterial Phase

Partners CAS

Axial T1-weighted Hi-Resolution with Fat Sat; Post-Gadolinium

Partners CAS

Wall thickening along the fundus

measuring up to 15mm in maximum thickness

Slight enhancement of GB wall mucosa, most prominently involving the fundal

portion

Film Findings: thickened gallbladder wall with hyper-intensity of the mucosa mostly involving the fundus

Our Patient: Findings on MR imaging

Axial T1-weighted Gradient Echo with Fat Sat; Post-Gadolinium, Arterial Phase

Partners CAS

Axial T1-weighted Hi-Resolution with Fat Sat; Post-Gadolinium

Partners CAS

Small cystic area adjacent to the fundus

measuring up to 2.0 cm, (+) rim enhancement

No clear communication between the fundus

and this cystic collection could be demonstrated

Film Findings: small cyst at the fundus

with ? communication to the gallbladder that cannot be clearly identified on MR

Axial T2-weighted with Fat Saturation

Partners CAS

Our Patient: Findings on MR imaging

Gallbladder sludge and stones

Coronal T2-weighted Single-Shot Fast Spin Echo (SSFSE)

Partners CAS

Irregular wall thickening involving the gallbladder fundus

Film Findings: Gallstones and, again, irregularly thickened gallbladder wall involving the fundus

Our Patient: Findings on MR imaging

Partners CAS

Coronal 2D Thick-Slab Abdomen (MR Cholangiopancreatography, or MRCP)

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The McGraw-Hill Companies, Inc.

Gallbladder

Duodenum

Cystic duct

Right hepatic duct Left hepatic duct

Common hepatic duct

Common bile duct

Gallbladde

r

carcinoma

Common hepatic duct

CommonCommon bile duct

Common

CommonR and L

hepatic ducts

Cystic duct

CoGallbladder

ComMain pancreatic duct

ComHepatopancreatic

ampulla

ComMajor duodenal papilla

ComDuodenum

(1)

(2)

(3)(4)

Pancreatic duct

Hepatopancreatic

ampullaMajor duodenal papilla

http://academic.kellogg.cc.mi.us/herbrandsonc/bio201

McKinley/Digestive%20System.htm

Film Findings: No biliary/pancreatic duct obstruction/dilatation Impression:

Normal biliary/pancreatic ductal

system.

(1) R and L hepatic ducts merge to form a common hepatic duct

Quick Review: The Biliary

and Pancreatic Ducts

Copyright ®

The McGraw-Hill Companies, Inc.

Gallbladder carcinoma

Common hepatic duct

CommonCommon bile duct

Common

CommonR and L

hepatic ducts

Cystic duct

CoGallbladder

ComMain pancreatic duct

ComHepatopancreatic

ampulla

ComMajor duodenal papilla

ComDuodenum

(2)

(3)(4)

(1)

(4)

Bile and pancreatic juices enter duodenum at the major duodenal papilla

(2)

Common hepatic and cystic ducts merge to form a common bile duct

(3)

Pancreatic duct merges with common bile duct at the hepatopancreatic

ampulla

http://academic.kellogg.cc.mi.us/herbrandsonc/bio201_McKinley/Digestive%20System.htm

Our Patient: Findings on MR imaging

Axial T2-weighted with Fat Saturation

Partners CAS

T2 signal abnormality (hyper-intensity) surrounding the gallbladder and adjacent liver parenchyma

No enlarged lymph nodes. Patent hepatic vasculature. No ascites.

Film Findings: ↑

T2 signal surrounding the fundus, patent hepatic vasculature, no lymphadenopathy

or ascities

Impression:

Overall MRI findings suggestive of fatty infiltration, adenomyomatosis

likely complicated by chronic cholecystitis;

gallbladder adenocarcinoma

cannot be entirely excluded.

MRI Dx:

What is Adenomyomatosis?

Definition:

benign, abnormal though non-premalignant gallbladder mucosal hyperplasia, muscular wall thickening, and formation of intramural diverticula

or sinus

tracts called Rokitansky- Aschoff

sinuses

Radiologic Finding:

Pearl Necklace Sign

uodenumuodenum

Haradome, H. et al. Radiology 2003. 227(1): 80-8.

Very small cystic

structures

Very small cystic

structures

(Pearl Necklace Sign)

(Pearl Necklace Sign)

Multiple Multiple gallbladder stonesgallbladder stones

Arrive at Our Dx, Step by Step …

Clinical DDx:•

Cholecystitis•

Choledocholithiasis•

Cholangitis•

Hepatitis•

Pancreatitis

H&P:•

Hx

RUQ abd

pain•

Labs –

Leukocytosis•

Exam –

(+) Murphy sign

US DDx:•

Gangrenous cholecystitis•

Gallbladder carcinoma CT DDx: gallbladder malignancy

Pathology/Management: Open cholecystectomy to make the definitive, final Dx by histology and determine future management of our patient

MR DDx:•

Adenomyomatosis•

Gallbladder adenocarcinoma

MR Findings:•

Thickened gallbladder wall•

Fundus

cyst with ?communication•

Gallbladder stones•

No biliary

obstruction/dilatation•

T2 signal surrounding the fundus

Our Companion Patient: Findings on Gross Pathology

Diffuse wall thickening

Serosa

covered with dense fibrous adhesions

Ulcerated mucosal surface

Yellow nodules/plaques, or Yellow nodules/plaques, or xanthogranulomatousxanthogranulomatous

foci, extend into foci, extend into adjacent liver through the walladjacent liver through the wall

Levy, A. et al. Radiographics. 2002. 22(2): 387-413.

Cross section of the Cross section of the resectedresected

gallbladdergallbladder

Disruption of the gallbladder wall

Gross Pathology Findings:

(1) fibrosis and wall thickening (2) disruption of gallbladder wall (3) xanthogranulomatous

foci

Our Companion Patients: Findings on Histology

Varadarajulu

S, et al. Up-to-Date

Fibroblasts, Fibroblasts, inflammatory cellsinflammatory cells

SpindleSpindle--shaped cells shaped cells with more granular with more granular cytoplasm and cytoplasm and elongated nucleielongated nuclei

Lipid-laden mø: 2 morphological types

Levy, A. et al. Radiographics. 2002. 22(2): 387-413.

XanthogranulomatousXanthogranulomatous

cholecystitischolecystitis

focus (focus (blackarrowsblackarrows

above)above)

H&E stainH&E stain

Thickened, fibrotic wall

Contains: (1) bile pigment (2) chronic inflammatory cells (3) foamy pigment-laden macrophages (mø)

No dysplasia or malignancy!

Rounded foamy Rounded foamy macrophagesmacrophages

(1)(1)

(2)(2)

Arrive at Our Dx, Step by Step …

Clinical DDx:•

Cholecystitis•

Choledocholithiasis•

Cholangitis•

Hepatitis•

Pancreatitis

H&P:•

Hx

RUQ abd

pain•

Labs –

Leukocytosis•

Exam –

(+) Murphy sign

US DDx:•

Gangrenous cholecystitis•

Gallbladder carcinoma CT DDx: gallbladder malignancy

MR DDx:•

Adenomyomatosis•

Gallbladder adenocarcinoma

Pathology (Final) Dx: Xanthogranulomatous

cholecystitis

Gross/Histologic

Findings:•

Wall thickening with fibrotic serosa•

Xanthogranulomatous

foci•

Bile extravasation

through disrupted wall•

Lipid-laden macrophages•

Chronic inflammatory cells

Dx:

What is Xanthogranulomatous

Cholecystitis?

Definition:

unusual form of benign, chronic cholecystitis

with focal or diffuse destructive

inflammatory process•

Signs and symptoms: RUQ abd

pain, fever,

leukocytosis, vomiting, (+) Murphy sign•

Hallmarks:(1) thickened, fibrotic, disrupted gallbladder wall(2) foamy histiocytes(3) bile extravasation

Dx:

What is Xanthogranulomatous

Cholecystitis?

Pathophysiology: gallbladder or cystic duct obstruction ↑ gallbladder intraluminal pressure rupture of Rokitansky-Aschoff sinuses or mucosal ulceration extravasation of bile into the gallbladder wall

s63.jpgs63.4x1.jpg

bile

biles63.jpg

http://anatomy.iupui.edu/courses/histo_D502/D502f04/Labs.f04/digestive%20III%20lab/Lab13index.htm

Management:

Significance of Xanthogranulomatous

Cholecystitis

Significance: may simulate malignancy clinically, radiologically, and pathologically

Management of XG cholecystitis: open cholecystectomy

with complete resection of the

gallbladder due to dense fibrosis, extensive inflammation, ?coexistent malignancy

Management of GB carcinoma: (1) aggressive surgery

partial/segmental hepatic

resection or Whipple procedure(2) no resection

at all with chemo/radiation instead

XG cholecystitis: benign yet focally/diffusely destructive inflammatory gallbladder disease with (1) fibrosis and wall thickening, (2) bile extravasation, (3) lipid-laden mø, (4) acute/chronic inflammatory cells

XG cholecystitis

vs GB carcinoma:

Patients with carcinoma are more likely to present with anorexia, weight loss, palpable mass, and jaundice

Preoperative Dx

by radiographs:

may significantly alter therapy and patient prognosis –

be careful!

Take Home Points:

What happened to Our Patient?

Our patient underwent an exploratory laparoscopy that was converted to open cholecystectomy, which went successfully without any complications

His gallbladder was diagnosed with xanthogranulomatous

cholecystitis

without

any associated malignancy by pathology and histology

Our patient is alive and well as of today in June, 2008

Acknowledgements

Gillian Lieberman, M.D.•

Laura Avery, M.D.

James Kang, M.D. (resident)•

Karen Lee, M.D. (fellow)

Maryellen Sun, M.D. (fellow)

ReferencesChun KA, Ha HK, Yu ES, Shinn KS, Kim KW, Lee DH, Kang SW, Auh

YH. Xanthogranulomatous

cholecystitis: CT features with emphasis on differentiation from gallbladder carcinoma. Radiology. 1997 Apr; 203(1): 93-7.

Guermazi

A. Are there other imaging features to differentiate xanthogranulomatous

cholecystitis

from gallbladder carcinoma? Eur

Radiol. 2005 Jun; 15(6): 1271-2.Haradome

H, Ichikawa T, Sou

H, Yoshikawa T, Nakamura A, Araki T, Hachiya

J. The pearl necklace sign: an imaging sign of adenomyomatosis

of the gallbladder at MR cholangiopancreatography. Radiology. 2003 Apr; 227(1): 80-8.

Levy AD, Murakata

LA, Rohrmann

CA Jr. Gallbladder carcinoma: radiologic-pathologic correlation. Radiographics. 2001 Mar-Apr; 21(2): 295-314.

Levy AD, Murakata

LA, Abbott RM, Rohrmann

CA Jr. From the archives of the AFIP. Benign tumors and tumorlike

lesions of the gallbladder and extrahepatic

bile ducts: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 2002 Mar-Apr; 22(2): 387-413. Review.

Shuto

R, Kiyosue

H, Komatsu E, Matsumoto S, Kawano K, Kondo Y, Yokoyama S, Mori H. CT and MR imaging findings of xanthogranulomatous

cholecystitis: correlation with pathologic findings. Eur

Radiol. 2004 Mar; 14(3): 440-6.

Srivastava

M, Sharma A, Kapoor

VK, Nagana

Gowda

GA. Stones from cancerous and benign gallbladders are different: A proton nuclear magnetic resonance spectroscopy study. Hepatol

Res. 2008 May 27.Varadarajulu

S, Zakko

SF. Xanthogranulomatous

cholecystitis. Up-to-date. 2007.Slides 16 and 17 –

http://academic.kellogg.cc.mi.us/herbrandsonc/bio201_McKinley/Digestive%20System.htm

Slide 25 –

http://anatomy.iupui.edu/courses/histo_D502/D502f04/Labs.f04/digestive%20III%20lab/Lab13index.htm