Chapter 088. Hepatocellular Carcinoma (Part 10)

Fibrolamellar HCC (FL-HCC) This rarer variant of HCC has a different biology than adult-type HCC. None of the known HCC causative factors seem important here. It is typically a disease of younger adults, often teenagers and predominantly females. It is AFP negative, but patients typically have elevated blood neurotensin levels, normal liver function tests, and no cirrhosis. Radiology is similar for HCC, except that characteristic adult-type portal vein invasion is less common. Although it is often multifocal in the liver, and therefore not resectable, metastases are common, especially to lungs and locoregional lymph nodes, but survival is often much...
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Chapter 088. Hepatocellular Carcinoma (Part 10) Chapter 088. Hepatocellular Carcinoma (Part 10) Fibrolamellar HCC (FL-HCC) This rarer variant of HCC has a different biology than adult-type HCC.None of the known HCC causative factors seem important here. It is typically adisease of younger adults, often teenagers and predominantly females. It is AFPnegative, but patients typically have elevated blood neurotensin levels, normalliver function tests, and no cirrhosis. Radiology is similar for HCC, except thatcharacteristic adult-type portal vein invasion is less common. Although it is oftenmultifocal in the liver, and therefore not resectable, metastases are common,especially to lungs and locoregional lymph nodes, but survival is often muchbetter than with adult-type HCC. Resectable tumors are associated with 5-yearsurvival of ≥50%. Patients often present with a huge liver or unexplained weightloss, fever, or elevated liver function tests on routine evaluations. These hugemasses suggest slow growth. Surgical resection is the best management option,even for metastases, since these tumors respond much less well to chemotherapythan adult-type HCC. Although several series of OLTX for FL-HCC have beenreported, the patients usually to die from tumor recurrences, with a 2- to 5-year lagcompared with OLTX for adult-type HCC. Anecdotal responses to gemcitabineplus cisplatin-TACE are reported. Epithelioid Hemangioendothelioma (EHE) This rare vascular tumor of adults is also usually multifocal and can also beassociated with prolonged survival, even in the presence of metastases, which arecommonly in the lung. There is usually no underlying cirrhosis. Histologically,these tumors are usually of borderline malignancy and express factor VIII antigen,confirming their endothelial origin. OLTX may be associated with prolongedsurvival. Cholangiocarcinoma (CCC) CCC typically refers to mucin-producing adenocarcinomas (different fromHCC) that arise from the bile ducts. They are grouped by their anatomic site oforigin as intrahepatic, hilar (central, ~65% of CCCs), and peripheral (or distal,~30% of CCCs). They arise on the basis of cirrhosis less frequently than HCC,excepting primary biliary cirrhosis. Nodular tumors arising at the bifurcation ofthe common bile duct are called Klatskin tumors and are often associated with acollapsed gallbladder, a finding that mandates visualization of the entire biliarytree. The approach to management of central and peripheral CCC is quitedifferent. The incidence seems to be increasing in the United States. Althoughmost CCCs have no obvious cause, several predisposing factors have beenidentified, including primary sclerosing cholangitis, an autoimmune disease (10–20% of PSC patients), and liver fluke in Asians, especially Opisthorchis viverriniand Clonorchis sinensis. CCC seems also to be associated with any cause ofchronic biliary inflammation and injury, with alcoholic liver disease,choledocholithiasis, choledochal cysts (10%), and Carolis disease. CCC mosttypically presents as painless jaundice, often with pruritus or weight loss, andacholic stools. Diagnosis is made by biopsy, percutaneously for peripheral liverlesions or, more commonly, via endoscopic retrograde cholangiopancreatography(ERCP) under direct vision for central lesions. The tumors often stain positivelyfor cytokeratins 7, 8, and 19 and negatively for cytokeratin 20. However, histologyalone cannot usually distinguish CCC from metastases from primary tumors of thecolon or pancreas. Serologic tumor markers appear to be nonspecific, but CEA,CA 19-9, and CA-125 are often elevated in CCC patients and are useful forfollowing response to therapy. Radiologic evaluation typically starts withultrasound, which is useful in visualizing dilated bile ducts, and then proceedswith either MRI or magnetic resonance cholangiopancreatography (MRCP) orhelical CT scans. Invasive ERCP is then needed to define the biliary tree andobtain a biopsy or is needed therapeutically to decompress an obstructed biliarytree with internal stent placement. If that fails, then percutaneous biliary drainagewill be needed, with the biliary drainage flowing into an external bag. Centraltumors often invade the porta hepatis, and locoregional lymph node involvementby tumor is frequent. Cholangiocarcinoma: Treatment Hilar CCC is resectable in ~30% of patients and usually involves bile ductresection and lymphadenectomy. Typical survival is around 24 months, withrecurrences being mainly in the operative bed but with ~30% in the lungs andliver. Distal CCC, which involves the main ducts, is normally treated by resectionof the extrahepatic bile ducts, often with pancreaticoduodenectomy. Survival ...