Intrahepatic Cholangiocarcinoma (ICC) Detected by Sonography

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1 661245JDMXXX / Journal of Diagnostic Medical SonographyHamer research-article2016 Case Study Intrahepatic Cholangiocarcinoma (ICC) Detected by Sonography Journal of Diagnostic Medical Sonography 2016, Vol. 32(5) The Author(s) 2016 Reprints and permissions: sagepub.com/journalspermissions.nav DOI: / jdms.sagepub.com Breianon A. Hamer, BS, RDMS, RVT Abstract Intrahepatic cholangiocarcinoma (ICC) is a malignant disease process that develops within the small branches of the biliary system within the liver. It is one of two types of cholangiocarcinoma (CCA). The patient in this case study experienced general abdominal and pelvic discomfort when further sonographic evidence proved there to be a moderate amount of fluid within the pelvic and abdominal cavities, suggesting a malignant process. Three types of growth processes related to ICC require different methods of treatment, thus making it imperative for the sonographer to identify the origin of the disease process. Appropriate knowledge of sonographic landmarks such as the biliary tree, liver, hepatic veins, and portal system is an important factor when evaluating normal and abnormal right upper quadrant structures. This case study focuses on the importance of sonography when detecting the size and location of ICC to determine an appropriate treatment method for this disease process. Keywords cholangiocarcinoma, intrahepatic, carcinoma, hepatic, biliary system, ascites Intrahepatic cholangiocarcinoma (ICC) is an unusual, mass-forming carcinoma that originates from the biliary system. 1 It arises from the epithelial lining of the bile ducts. 1 ICC is defined as a disease that originates within the small ducts and cells in the liver. 1 The tiny intrahepatic ducts transport bile, which is produced from the liver cells. 2 If the ducts become obstructed or interrupted, the flow of bile will cease. 2 Bile ducts play an active role in the absorption and secretion of biliary components and in the regulation of the extracellular matrix composition. 2 Dilatation of the biliary ducts can help provide an area of interest to further examine (such as the pancreatic head). With sonography, an extrahepatic mass can be differentiated from other pathologies inside the liver. After investigating the area for dilated extrahepatic ducts, the sonographer may further examine the liver. This case study highlights the utility of sonography to identify ICC, which can help provide a confident decision for treatment. Case Report A 67-year-old woman presented to the sonography department with diffuse abdominal and pelvic pain. The sonography examination was performed with a Philips iu22 system (Philips Ultrasound, Bothell, WA). Multiple images were obtained using a curved linear 5-MHz transducer and a Philips C10-3V broadband curved array transducer. Sagittal and transverse scanning planes were used, as well as gray-scale imaging, color Doppler imaging, and pulse-wave Doppler analysis. The patient was in three different positions during the examination to ensure adequate visualization of anatomical structures. The supine position was used to image the great vessels and pancreas, a right decubitus position to visualize the spleen and left kidney, and a left decubitus position to image the liver, portal system, gallbladder, right kidney, and all of the biliary structures. The pelvic sonogram was performed first, both transabdominally and transvaginally, and exhibited a mild to moderate amount of fluid localized within the endometrial canal (Figures 1 and 2), as well as free fluid surrounding the pelvic organs (Figure 3). The uterus measured cm in length, anteroposterior (AP), and transverse dimensions. The endometrial stripe measured 2.5 mm. Both ovaries were obscured by overlying bowel gas. Further endovaginal imaging showed additional fluid in the left adnexa (Figure 4) and within the posterior cul-de-sac (Figure 5). Immediately after the pelvic sonogram, a complete abdominal scan was performed. Upon review of the images from the abdominal sonogram, a large heterogeneous mass was discovered. The mass was centrally located within the liver at the junction of the right and Received March 22, 2014, and accepted for publication June 30, Corresponding Author: Breianon A. Hamer, 5979 Devecchi Ave., Citrus Heights, CA 95621, USA. breianonhamer@gmail.com

270 Journal of Diagnostic Medical Sonography 32(5) Figure 1.

anterior portion of the pelvis (left side of the image). Figure 4. Transverse endovaginal sonographic image that demonstrates fluid visualized in the left adnexa.

Axial (transverse) sonographic view of fluid within the endometrial canal and surrounding the uterus. Figure 3.

Figure 5. Sagittal endovaginal sonographic image that demonstrates fluid in the posterior cul-de-sac is appreciated.

8 cm (Figures 6 and 7). There was normal hepatopetal (toward the liver) flow demonstrated within the main portal vein at the porta hepatis (Figure 8).

2 270 Journal of Diagnostic Medical Sonography 32(5) Figure 1. Sagittal endovaginal sonographic image displays the endometrium with a mild to moderate amount of fluid seen within the endometrial canal as well as surrounding fluid in the anterior portion of the pelvis (left side of the image). Figure 4. Transverse endovaginal sonographic image that demonstrates fluid visualized in the left adnexa. The uterus is displayed in a cross-sectional view on the left side of the image. Figure 2. Axial (transverse) sonographic view of fluid within the endometrial canal and surrounding the uterus. Figure 3. Transabdominal sagittal sonogram that displays fluid surrounding the superior and anterior portion of the uterus, as well as bowel being displaced by the ascites (free fluid). Figure 5. Sagittal endovaginal sonographic image that demonstrates fluid in the posterior cul-de-sac is appreciated. left lobes, involving the caudate lobe and inferior vena cava (IVC). The liver demonstrated a heterogeneous mass of mixed echogenicities that measured cm (Figures 6 and 7). There was normal hepatopetal (toward the liver) flow demonstrated within the main portal vein at the porta hepatis (Figure 8). Furthermore, the liver measured 16.4 cm in length, and there was no intrahepatic or extrahepatic biliary ductal dilatation. The common bile duct measured 4.0 mm at the porta hepatis, which was within normal limits. The gallbladder was normally distended but had a thickened wall measuring up to 6 mm from surrounding ascites in the abdomen (Figure 9). The sonographic Murphy s sign was negative. In Figure 10, the mass is defined with color Doppler showing the mass effect on the surrounding vessels. Furthermore, in Figure 11, the invasion of the mass into the caudate lobe is appreciated.

dimensions. Figure 9. Transverse image of the gallbladder wall.

Transverse measurement and location of the mass in relation to the inferior vena cava.

Transverse image of the liver using color Doppler to display the compression of the vessels at

Doppler analysis of the main portal vein displaying hepatopetal flow.

3 Hamer 271 Figure 6. Longitudinal image displaying the mass and its measurements in both length and anteroposterior dimensions. Figure 9. Transverse image of the gallbladder wall. It measured approximately 6 mm, which exceeds the normal thickness of 3 mm. Figure 7. Transverse measurement and location of the mass in relation to the inferior vena cava. Figure 10. Transverse image of the liver using color Doppler to display the compression of the vessels at the porta hepatis due to the location of the mass. Figure 8. Doppler analysis of the main portal vein displaying hepatopetal flow. Lastly, there was a small to moderate amount of fluid visualized around the liver and gallbladder (Figure 12). Due to the visualization of a thickened gallbladder wall, Figure 11. This sagittal view of the left lobe and caudate lobe of the liver displaying the mass invading both lobes. hepatocellular disease was suspected, and further testing was recommended.

272 Journal of Diagnostic Medical Sonography 32(5) Figure 12. Transverse image of the liver/kidney/gallbladder interface displaying free fluid surrounding the right upper quadrant (RUQ) organs.

The findings were similar to the findings on the sonogram. Within the abdomen, a large heterogeneous enhancing intrahepatic mass that measured 8.0 11.2 9.

There was mild to moderate fluid in the abdomen and pelvis, which was previously seen on sonography. Focal infiltrates were visualized in the right lower lobe and left upper lobe of the liver.

4 272 Journal of Diagnostic Medical Sonography 32(5) Figure 12. Transverse image of the liver/kidney/gallbladder interface displaying free fluid surrounding the right upper quadrant (RUQ) organs. The patient returned to the outpatient center a day later to obtain a computed tomography (CT) scan of the abdomen and pelvis with and without contrast. The findings were similar to the findings on the sonogram. Within the abdomen, a large heterogeneous enhancing intrahepatic mass that measured cm was visualized at the junction of the right and left hepatic lobes. Also noted were slightly prominent gastrohepatic, celiac, periportal, precaval, aortocaval, and periaortic lymph nodes. There was mild to moderate fluid in the abdomen and pelvis, which was previously seen on sonography. Focal infiltrates were visualized in the right lower lobe and left upper lobe of the liver. The appearance was suggestive of an infectious or inflammatory etiology, although metastases were not excluded due to the patient s history. Further findings of the CT examination included the appearance of IVC compression by the mass without direct intravascular extension. Also compressed was the left portal vein; however, no direct invasion of the vessel was visualized. In the following CT images (Figures 13 and 14), the mass is represented centrally within the liver. As a result of the large hepatic mass that was rediscovered through CT, the patient underwent a CT-guided biopsy. The pathology results presented as ICC. The laboratory values were unobtainable for this patient. After the biopsy results were obtained, a CT of the chest with contrast was performed for the purpose of staging the ICC. This malignancy was reported to be stage III to IV. The treatment options were limited for this patient due to the late findings. After the staging and diagnosis, the patient went to the local hospital facility for weekly paracentesis procedures as a therapeutic treatment and palliative care. Discussion ICC is a rather rare malignancy and second most common primary liver cancer, although far less prevalent Figure 13. Computed tomography is of the liver (on the left side of the image) showing the large intrahepatic mass of mixed echogenicity. Figure 14. A sagittal plane was used with computed tomography imaging to view the chest and abdomen. The liver mass is appreciated in the bottom center of the image. than the leading hepatic carcinoma. 3 Hepatobiliary malignancies account for 13% of the 7.6 million annual cancer-related deaths worldwide. 4 The specific etiology for this type of malignancy remains unknown in most cases, but multiple risk factors contribute to this disease process. 3 Risk factors for ICC may include hepatitis B virus and hepatitis C virus infections, as well as liver cirrhosis, regardless of etiology. 4 Other common risk factors may include a history of obesity, diabetes, smoking, toxins, parasitic infections, primary sclerosing cholangitis, and hepatolithiasis. 5 Clinical symptoms that may occur with this disease process include jaundice, weakness, fever, weight loss, fatigue, hepatomegaly, ascites, and a palpable abdominal mass. 6 There is also patient

5 Hamer 273 history that may be related to this disease such as hypertension, diabetes mellitus, and a history of surgery to a part of the biliary system. 6 For this pathology, no laboratory tests will identify that the malignancy is specific to a type of cholangiocarcinoma (CCA), but many will help identify the location of the pathology to narrow down the diagnosis. Examples would include liver function tests and bilirubin within the blood. When these levels are elevated, it means that there are abnormalities within the liver and/or biliary system. Further testing for CCA diagnosis may include a complete blood count, hematocrit, and carcinoembriogenic antigen and carbohydrate antigen 19-9 (CA 19-9) evaluation. 4 Among these markers, CA 19-9 remains the most commonly used tumor marker for CCA. 4 The other blood test that shows abnormalities is alpha-fetoprotein (AFP). When the levels of this test are elevated, it may indicate a malignant process. 4 The use of sonography allows the capability to image possible portal vein or IVC invasion, along with ductal interruption and dilatation. Under sonographic evaluation, it was evident that the lesion was primarily intrahepatic due to the central location. In addition, due to the increased amount of pelvic and abdominal ascites, there was an increased probability for malignancy, although not diagnostic with ultrasound alone. The CT with contrast was performed to further detect the level of involvement resulting in similar findings to the sonogram. ICC can be divided into three types of growth patterns: massforming growth type, periductal infiltrating type, and intraductal growth type. 1 The one of interest is the massforming growth type, which occurs from a chronic inflammation of the walls of the intrahepatic ducts, which then leads to autonomous cellular proliferation. 7 Although not well understood, there is likely a progression from hyperplasia to dysplasia, in situ carcinoma, and finally invasive carcinoma. 7 Due to the delay in diagnoses and aggressive nature of the disease, the prognosis for ICC is poor. There are only a few treatment options available for a patient dealing with CCA. There is the option of performing a surgical hepatic resection of the specific area of interest where the mass resides. Surgical resection is the only treatment that offers a chance for a cure. 8 Total hepatectomy and subsequent liver transplantation may be considered one option for the treatment of patients with greatly advanced ICC. 9 Unnecessary surgery can be avoided if the prognosis is extremely poor for the patient, and pre- or postoperative chemotherapy or radiation may be effectively added, even though the prognosis is still poor but not hopeless. 4 Lastly, there is the option of image-guided radiofrequency ablation (RFA). The procedure may be guided with CT imaging, ultrasound, or the use of both modalities. Image-guided percutaneous RFA may have a role in achieving local tumor control for patients with ICC. 8 If other treatment options (such as surgical resection or chemotherapy) are unavailable to the patient, RFA may be a therapeutic option for the remaining months of life. 8 How well a patient handles the treatment of bile duct cancer depends on many factors, including where the tumor is located, metastatic staging, and the patient s underlying general health. Specific factors influencing postsurgery survival are still unclear. 4 An alternative imaging modality that may help diagnose CCA is magnetic resonance imaging, which is useful for determining the specific location of the liver pathology and its relation to the vascular and biliary system. It also helps the doctor to analyze the overall health of the liver. Sonography was the initial imaging modality of choice due to the clinical presentation. Furthermore, the American College of Radiology (ACR) Appropriateness Criteria rank sonography as the imaging choice for a patient presenting with jaundice, evaluating fluid in the most dependent areas of the body, as well as visualizing solid versus cystic masses. 10,11 A benefit of continuing with the CT with contrast was the ability to determine if the mass was invading the surrounding vessels or simply causing a mass effect on the IVC and left portal vein. Conclusion ICC is a disease process that may go unnoticed in the early stages but may be fatal if identified too late, thus confirming the reason sonographers should be familiar with the RUQ structures and their origins. With the expected background knowledge of the sonographer, ICC can be documented and treated appropriately. Sonography provides real-time imaging of anatomy structures and is known to be a primary imaging modality for determining solid from cystic lesions. Sonography remains a primary imaging modality to help diagnose specific pathology related to symptoms such as diffuse abdominal pain. Specific treatment options are invaluable after the diagnosis of ICC. Treatment options such as surgical resection, chemotherapy, radiation, transplant, and RFA should be closely researched and decided upon after the level of involvement and overall health of the patient are taken into consideration. Declaration of Conflicting Interests The author declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. Funding The author received no financial support for the research, authorship, and/or publication of this article.

6 274 Journal of Diagnostic Medical Sonography 32(5) References 1. Nakanuma Y, Sasaki M, Ikeda H, et al: Pathology of peripheral intrahepatic cholangiocarcinoma with reference to tumorigenesis. Hepatol Res 2008;38: Wang DQ-H, Neuschwander-Tetri BA, Portincasa PAM: The Biliary System. San Rafael, CA, Morgan & Claypool, Kokudo N, Arita J: Staging for intrahepatic cholangiocarcinoma. Liver Int 2010;30: Blechacz B, Gores GJ: Cholangiocarcinoma: advances in pathogenesis, diagnosis, and treatment. Hepatology 2009;48: Tyson GL, El-Serag HB: Risk factors for cholangiocarcinoma. Hepatology 2011;54: Lee K-F, Cheung Y-S, Tsang YY-Y, Ng WW-C, Wong J, Lai PB-S: Intrahepatic cholangiocarcinoma: a diagnostic and therapeutic challenge. Surg Pract 2008;12: Bartlett DL: Intrahepatic cholangiocarcinoma. Cancer J 2009;15: Butros SR, Shenoy-Bhangle A, Mueller PR, Arellano RS: Radiofrequency ablation of intrahepatic cholangiocarcinoma: feasibility, local tumor control, and long-term outcome. Clin Imaging 2014;38:4. 9. Ohtsuka MM, Ito HH, Kimura FF, et al: Results of surgical treatment for intrahepatic cholangiocarcinoma and clinicopathological factors influencing survival. Br J Surg 2002;89(12): ACR Appropriateness Criteria jaundice. acr.org/docs/69497/narrative/. Accessed June 30, ACR Appropriateness Criteria radiologic management of benign and malignant biliary obstruction. acr.org/docs/69344/narrative/. Accessed June 30, 2016.

Abdomen and Retroperitoneum Ultrasound Protocols

Abdomen and Retroperitoneum Ultrasound Protocols Reviewed By: Anna Ellermeier, MD Last Reviewed: March 2018 Contact: (866) 761-4200, Option 1 **NOTE for all examinations: 1. If documenting possible flow