Case of the Month

Submitted by: Amit "Sunny" Mittal

History

• CC: Abdominal pain and rash
• HPI: 46 y/o AAM with hx of PUD and Hep C who c/o abdominal pain x three weeks, greatest in the RUQ, that radiates to the back. Pt also states that he developed a diffuse pruritic rash two weeks ago.
• ROS: + dark brown urine x three-four days, nausea, vomiting, and decreased appetite.
• PMH: PUD, Hep C
• PSH: Partial gastrectomy
• SH: + Tob, EtOH

Physical Exam

• VS: BP 155/99 P 66 R 20 T 100.3
• Gen: Jaundiced
• HEENT: Icteric sclera
• Resp: CTA B/L
• CV: RRR
• GI: RUQ tenderness, + Murphy's sign
• Skin: + excoriations B/L upper extremities

Labs

• Hgb: 13.6 L
• Hct: 37.2 L
• Plt: 101 L
• UA: 2+ Bili
• ALT: 118 H
• AST: 214 H
• Alk Phos: 171 H
• TBili: 7.4 H
• DBili: 5.6 H

Differential Diagnosis

RUQ Ultrasound

Normal

• Transverse and longitudinal images were obtained
• Enlarged liver at 17.8 cm
• Inhomogeneous mass in posterior right lobe of liver measuring 5.6 x 6.4 x 5.6 cm with some blood flow on doppler, suspicious of neoplasm
• Gall bladder showed no thickening or fluid collection
• No evidence of intrahepatic biliary ductal dilatation
• Common hepatic duct with normal caliber at 4.1 mm
• Common bile duct mildly enlarged at 7.0 mm
• CT of abdomen with liver mass protocol recommended

CT Abdomen

Normal

• CT of abdomen with liver mass protocol
• Within central portion of segment 7 of right liver lobe there is a 6 cm heterogeneous mass concerning for malignancy
• Partial thrombosis of right portal vein
• Right liver lobe with biliary duct dilatation
• Abnormal soft tissue density in porta hepatis likely represents LAD
• 9 mm simple cyst in right kidney

Differential Diagnosis

Special Procedures

• U/S - guided liver biopsy was attempted but could not be completed due to poor visualization of the mass
• CT - guided biopsy was performed successfully with a 20-gauge 9 cm core biopsy gun
• 3 passes were made into the mass in the right lobe of the liver and specimens taken and sent to pathology for evaluation

Pathology

• Sections show moderately to poorly differentiated adenocarcinoma composed of small round, mildly pleomorphic cells with hyperchromatic nuclei and little cytoplasm.
• Immunohistochemistry showed positive expression for CK7, MOC-31, and negative for Hep Par-1. Based on this, it is highly unlikely that the tumor is a primary HCC, but the studies alone cannot distinguish between metastatic adenocarcinoma and cholangiocarcinoma.

EGD and Colonoscopy

• GI performed EGD and Colonoscopy in order to evaluate for extra-hepatic source of primary malignancy
• EGD showed partial gastrectomy with Billroth I anastomoses and esophageal varices
• Colonoscopy revealed hemorrhoids and some friability with vascular ectasias near the cecum likely secondary to portal hypertension
• No evidence of primary malignancy

MR Cholangiopancreatography

• MR cholangiopancreatography performed with and without contrast
• Dilatation of right intrahepatic biliary ducts not involving the main hepatic duct or main biliary duct
• Ill-defined mass within liver segments 7 and 8 at the confluence of the right intrahepatic biliary ducts measuring 7.3 x 5.5 x 5.0 cm
• No retroperitoneal LAD or ascites
• Small right renal cyst

Chest X-ray

Normal

• No acute cardiopulmonary disease
• Hilar calcified lymph nodes from previous granulomatous disease

MRI Abdomen

• MRI of abdomen with and without contrast was performed
• A mass measuring 6.3 x 5.0 x 8.0 cm is seen in segments 7 and 8 of the right lobe of the liver occluding intrahepatic biliary ducts with resultant obstructive dilatation
• There is also invasion of the right portal vein by the mass
• No retroperitoneal LAD
• The findings are consistent with a cholangiocarcinoma

Treatment and Follow-up

• Patient was discharged home on 4/16/05 in fair condition with a diagnosis of metastatic adenocarcinoma vs. cholangiocarcinoma
• Surgery will continue to follow the patient and will discuss possible surgical resection of the liver mass
• Patient also to follow-up with oncology and dermatology clinic

Cholangiocarcinoma

• An adenocarcinoma that arises from bile duct epithelium
• The second most common primary hepatic tumor after Hepatocellular carcinoma
• All cholangiocarcinomas grow slowly, infiltrate locally, and metastasize late in the course of the disease
• Usually become symptomatic when the tumor obstructs the biliary drainage system
• Classification:
  • Extrahepatic
  • Intrahepatic
    • Hilar: arises from one of the hepatic ducts or the bifurcation of the common hepatic duct (Klatskin tumor)
    • Peripheral: arises peripheral to the secondary bifurcation of the left or right hepatic duct

Clinical Findings

Radiographic evaluation

1. Transabdominal U/S
   • Allows visualization of bile ducts for dilatation
   • Can evaluate for vascular involvement with color Doppler (portal vein or hepatic artery involvement indicates unresectability)
   • Not good for exact localization of tumor
2. Abdominal CT
   • Useful for detecting intrahepatic tumors and the level of biliary obstruction
   • Ductal dilatation within an atrophied hepatic lobe along with a hypertrophied contralateral lobe suggests invasion of the portal vein
   • Provides better anatomic detail and more accurate staging than MR
   • Multiphasic CT is still limited in its ability to determine local extent of tumor and resectability
3. MR Cholangiopancreatography
   • Noninvasive technique that uses MR technology to create 3-dimensional images of the biliary tree, liver parenchyma and vascular structures
   • Can evaluate bile ducts above and below a stricture and identify intrahepatic masses
   • Allows better detection and evaluation of hepatic parenchymal changes peripheral to the tumor than CT
   • Cholangiocarcinomas appear hypointense on T1- and isointense to hyperintense on T2-weighted images
   • Dynamic images show peripheral enhancement followed by concentric filling in of the tumor with contrast nPooling of contrast on delayed images is suggestive of peripheral cholangiocarcinoma

Imaging Summary

• Day 2: RUQ Ultrasound
• Day 2: CT abdomen w/ liver mass protocol
• Day 7: CT-guided liver biopsy
• Day 16: MR Cholangiopancreatography
• Day 17: Chest X-ray
• Day 29: MRI Abdomen

References

1. Anand, M.K. and David Nicholson. Cholangiocarcinoma. http://www.emedicine.com/.
2. Baylor Radiology: http://erc.bcm.edu/rad_elect/index.htm
3. Chari, R.S., et al. Clinical Manifestations and Diagnosis of Cholangiocarcinoma. http://www.uptodate.com/
4. Han, J.K., et al. Cholangiocarcinoma: Pictorial essay of CT and Cholangiographic findings. Radiographics, 2002;22:173-187.
5. Jackson Gastroenterology; www.gicare.com/pated/ei00001.htm
6. Lee, W.J., et al. Radiologic Spectrum of Cholangiocarcinoma: Emphasis on Unusual Manifestations and Differential Diagnoses. Radiographics. 2001;21:S97-S116.
7. Virtual Hospital: www.vh.org/index.html

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