TAILIEUCHUNG - 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. | 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 .

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