Chapter 088. Hepatocellular Carcinoma (Part 10)

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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) 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 better than with adult-type HCC. Resectable tumors are associated with 5-year
survival of ≥50%. Patients often present with a huge liver or unexplained weight loss, fever, or elevated liver function tests on routine evaluations. These huge masses suggest slow growth. Surgical resection is the best management option, even for metastases, since these tumors respond much less well to chemotherapy than adult-type HCC. Although several series of OLTX for FL-HCC have been reported, the patients usually to die from tumor recurrences, with a 2- to 5-year lag compared with OLTX for adult-type HCC. Anecdotal responses to gemcitabine plus cisplatin-TACE are reported. Epithelioid Hemangioendothelioma (EHE) This rare vascular tumor of adults is also usually multifocal and can also be associated with prolonged survival, even in the presence of metastases, which are commonly 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 prolonged survival. Cholangiocarcinoma (CCC) CCC typically refers to mucin-producing adenocarcinomas (different from HCC) that arise from the bile ducts. They are grouped by their anatomic site of origin 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 of the common bile duct are called Klatskin tumors and are often associated with a collapsed gallbladder, a finding that mandates visualization of the entire biliary tree. The approach to management of central and peripheral CCC is quite different. The incidence seems to be increasing in the United States. Although most CCCs have no obvious cause, several predisposing factors have been identified, including primary sclerosing cholangitis, an autoimmune disease (10– 20% of PSC patients), and liver fluke in Asians, especially Opisthorchis viverrini and Clonorchis sinensis. CCC seems also to be associated with any cause of chronic biliary inflammation and injury, with alcoholic liver disease, choledocholithiasis, choledochal cysts (10%), and Caroli's disease. CCC most typically presents as painless jaundice, often with pruritus or weight loss, and acholic stools. Diagnosis is made by biopsy, percutaneously for peripheral liver lesions or, more commonly, via endoscopic retrograde cholangiopancreatography (ERCP) under direct vision for central lesions. The tumors often stain positively for cytokeratins 7, 8, and 19 and negatively for cytokeratin 20. However, histology alone cannot usually distinguish CCC from metastases from primary tumors of the colon 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 for following response to therapy. Radiologic evaluation typically starts with ultrasound, which is useful in visualizing dilated bile ducts, and then proceeds with either MRI or magnetic resonance cholangiopancreatography (MRCP) or
helical CT scans. Invasive ERCP is then needed to define the biliary tree and obtain a biopsy or is needed therapeutically to decompress an obstructed biliary tree with internal stent placement. If that fails, then percutaneous biliary drainage will be needed, with the biliary drainage flowing into an external bag. Central tumors often invade the porta hepatis, and locoregional lymph node involvement by tumor is frequent. Cholangiocarcinoma: Treatment Hilar CCC is resectable in ~30% of patients and usually involves bile duct resection and lymphadenectomy. Typical survival is around 24 months, with recurrences being mainly in the operative bed but with ~30% in the lungs and liver. Distal CCC, which involves the main ducts, is normally treated by resection of the extrahepatic bile ducts, often with pancreaticoduodenectomy. Survival is similar. Due to the high rates of locoregional recurrences or positive surgical margins, many patients get treated with postoperative adjuvant radiotherapy. Its effect on survival has not been assessed. Intraluminal brachyradiotherapy has also shown some promise. However, photodynamic therapy enhanced survival in one study. In this technique, sodium porfimer is injected IV and then subjected to intraluminal red light laser photoactivation. OLTX has been assessed for treatment of unresectable CCC, but 5-year survival was previously ~20%, so enthusiasm waned. However, neoadjuvant radiotherapy with sensitizing chemotherapy has shown better survival rates for CCC treated by OLTX from one institution;
confirmation is needed. Multiple chemotherapeutic agents have been assessed for activity and survival in unresectable CCC. Most have been inactive. However, both systemic and hepatic arterial gemcitabine have shown promising results. The combination of this drug with others and with radiotherapy is being explored. Gallbladder Cancer (GB Ca) GB Ca has an even worse prognosis than CCC, with typical survival ~6 months or less. Women are affected much more commonly than men (4:1), unlike in HCC or CCC, and GB Ca is more common than CCC. Most patients have a history of gallstones, but very few patients with gallstones develop GB Ca (~0.2%). It presents similarly to CCC and is often diagnosed unexpectedly during gallstone or cholecystitis surgery. Presentation is typically that of chronic cholecystitis, chronic right upper quadrant pain and weight loss. Useful but nonspecific serum markers include CEA and CA 19-9. CT scans or MRCP typically reveal a gallbladder mass. The mainstay of treatment is surgical, either simple or radical cholecystectomy for stages I or II disease, respectively. Survival is nearly 100% at 5 years for stage I, and ranges from 60–90% at 5 years for stage II. More advanced GB Ca has worse survival, and many are unresectable. Adjuvant radiotherapy, used in the presence of local lymph node disease, has not been shown to enhance survival. Similar to CCC, chemotherapy is not useful in advanced or metastatic GB Ca.