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Transcript of LIVER 3 KISS 2018 [Kompatibilis üzemmód] · PDF file 2018. 3. 12. ·...

  • 2018. 03. 12.

    1

    LIVER PATHOLOGY(3)

    Prof. Andras Kiss. M.D., Ph.D., D.Sc.

    Semmelweis University

    2nd Department of Pathology

    Budapest

    February 26. 2018

    Vascular disorders

    � Inflow – A.hepatica thromb., embolia – infarctus

    – V.portae obstruction, thrombosis (pylethrombosis) – portal hypertension, causes

    � Trough – congestion, hepar moschatum, peliosis hepatis

    � Outflow – Budd-Chiari syndrome

    – VOD

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    Budd-Chiari syndrome (Extended hemorrhages in the liver parenchyma caused by thrombosis of hepatic veins)

    Nemesánszky-Schaff-Szalay Hepatologia Oktató CD 2004 Falk

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    Liver alterations associated with pregnancy

    � Acute steatosis in pregnancy – rare, from mild to severe (could be fatal), 3.trimester,

    perinatal, microvesicular steatosis, pancreatitis (common)

    � Intrahepatic cholestasis in pregnancy – 3. trimester, icterus, iching, cholestasis, ??

    � Praeeclampsy, eclampsy – HELLP-syndrom (hemolysis, elevated liver enzymes,

    low platelets), pale liver with red foci, fibrin deposits in sinusoids, hemorrhages

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    LIVER PATHOLOGY (3)

    • Focal liver lesions

    – Benign tumors and tumor-like lesions

    – Malignant tumors

    • Liver transplantation

    • Diseases of gallbladder and bile ducts

    – Diseases of gall bladder

    – Diseases of extrahepatic bile ducts

    – Tumors

    • (**)= important , (×), not for exam (extra)!!!

    Focal liver lesions (**)

    • Tumor-like lesions of the liver

    – FNH, NRH, mesenchymal hamartoma, cysts,

    inflammatory pseudotumor, abscessus, infarctus

    • Benign liver tumors

    – Non epithelial: haemangioma, fibroma,

    angiomyolipoma etc

    – Epithelial: adenoma (HCA, CCA)

    • Malignant liver tumors

    – Non epithelial: haemangiosarcoma, -endothelioma

    embryonal sarcoma, lymphoma

    – Epithelial: hepatocellular cc, cholangiocellular cc.,

    mixed, hepatoblasoma

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    Classification of liver cysts (1) (×)

    I. Parasitic

    II. Non parasitic

    A. Soliter

    B. Hereditary

    1. Non-communitating ductal

    2. DPM („ductal plate malformation”- communitating)

    • CHF (cong. hepatic fibrosis)

    • ARPKD

    • syndromes (Meckel-Gruber, Ivemark)

    3. Isolated hepatic

    *Witzleben, G. L., Ruchelli, E.

    II. Non parasitic

    C. Systemic biliary dilatative

    1. Without choledochus cyst

    („simple” Caroli disease)

    2. With choledochus cyst

    D. Other

    1. Traumatic, infarction

    2. Duodenal duplication

    3. Tumors with cyst

    • cystadenoma/-carcinoma

    • mesenchymal hamartoma

    • giant cavernous haemangioma

    • teratoma

    • other

    4. Peliosis

    *Witzleben, G. L., Ruchelli, E.

    Classification of liver cysts (2) (×)

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    Ecchinococcus cyst

    1 cm

    Hepar polycysticum

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    1 cm

    Mesenchymal hamartoma (children, benign)

    Tumor-like focal liver lesions (**)

    – Focal nodular hyperplasia (FNH)

    – Inflammatoric pseudotumor

    – Mesenchymal hamartoma

    – Nodular regenerativ hyperplasia

    – Infarct

    – Granulomas (Boeck, tbc etc)

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    Focal nodular hyperplasia (FNH)(**) - Female predominance,

    - Well circumsized, - No capsule

    - Central scar (fibrous septa radiate,

    „focal cirrhosis”)

    - Color (pale, fatty, haemorrhagic etc.)

    - Bile ducts: numerous, tortuous

    - Inflammatory cells

    -important!!!

    Focal Nodular Hyperplasia (FNH) (central scar!!!)

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    1 cm

    FNH

    1 cm

    4319-88

    FNH

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    Haemangioma hepatis (giant form)

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    1 cm

    1 cm

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    Focal Nodular Hyperplasia (FNH)

    FibroLamellar hepatocellular Carcinoma (FLC)

    Focal Liver Lesions

    FNH HCA (prev. FNH – teleang.)

    Hemang.

    FLC

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    Hepatocellular adenoma (HCA) (**)

    - Female predominance

    - Associated with oral contraceptives,

    anabolic steroids

    - Sharply demarcated, - Encapsulated

    - Homogenous structure, but hemorrhage,

    necrosis common,

    - Steatosis, no bile ducts in the tumor

    adenoma hepatocellulare (HCA) (yellow, steatosis, capsule)

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    HCA: Variant 3

    „teleangiectatic”

    • Monoclonal

    • Dilated vessels

    • Haemorrhagies

    • Less or no steatosis

    • Biliary vessels (less in number)

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    10 cm

    HCA Variant 3

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    CK7

    Zucman-Rossi et al., Hepatology 2006;43;515

    Classification of hepatocellular adenomas:

    association with HCC or borderline lesion

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    1 cm

    Adenoma hepatocellulare (extended bleeding, rupture might occur)

    Hepatocellular carcinoma (**)

    - Cirrhosis (70%)

    - Association with HBV/HCV/alkohol etc

    - Gross: uneven border, usually no capsule,

    haemorrhage, necrosis

    - Hist: trabecular, pseudoglandular (acinar),

    clear cell, scirrhous, fibrolamellar (grades

    I-IV)

    - Progression: infiltration of capsule (if exists),

    venous invasion

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    HCC

    extracapsular

    cirrhosis

    HCC

    necrosis

    HCC

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    1 cm

    Fibrolamellar HCC

    SCIENCE 2014 343:1010

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    HCC

    cirrhosis

    cirrhosis

    HCC HCC, trabecular form

    Different histological types of HCC

    HCC, trabecular HCC, pseudoglandular

    HCC, anaplastic

    HCC, venous invasion

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    Staging of HCC

    TNM-Classification

    T1 solitary tumor without vascular invasion

    T2 solitary tumor with vascular invasion or

    multiple tumors < 5 cm diameter

    T3 multiple tumors > 5 cm diameter or tumor invasion of major veins

    T4 tumor(s) with invasion of adjacent organs

    or perforation of visceral peritoneum

    Stage Grouping

    Stage I T1 N0 M0

    Stage II T2 N0 M0

    Stage IIIA T3 N0 M0

    Stage IIIB T4 N0 M0

    Stage IIIC any T N1 M0

    Stage IV any T any N M1

    Therapeutic (surgery) relevance

    Llovet J.M., Bruix J. Hepatology 2008.48:1312-27

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    Llovet J.M., Bruix J. Hepatology 2008.48:1312-27

    Llovet J.M., Bruix J.

    Hepatology 2008.48:1312-27

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    • Proangiogenic factors overexpressed in HCC:

    –Vascular endothelial growth factor (VEGF)

    –Platelet-derived growth factor (PDGF)

    –Placental growth factor

    –Transforming growth factor α and β

    –Basic fibroblast growth factor

    –Epidermal growth factor (EGF)

    –Hepatocyte growth factor

    –Angiopoietinek

    –Interleukin (IL)-4, IL-8

    Angiogenesis and HCC

    Semela D, Dufour J-F. J Hepatol 2004;41:864–80

    Characteristics of HCC

    • 5% of malignant tumors

    • 564 000 new cases annually

    • (in 2000) and similar death

    • Incidence is dubbled in the past

    20 yrs (Japan, USA, Sweden,

    France)

    • 7. in males

    • 9. in femels

    • Characteristic geography

    • Etiological factors: HBV, HCV,

    AFB1 (80%), alkohol etc

    • 5% of malignant tumors

    • 564 000 new cases annually

    • (in 2000) and similar death

    • Incidence is dubbled in the past

    20 yrs (Japan, USA, Sweden,

    France)

    • 7. in males

    • 9. in femels

    • Characteristic geography

    • Etiological factors: HBV, HCV,

    AFB1 (80%), alkohol etc

    Unknown

    35%

    HBV/HDV

    5% HBV

    15%

    HCV

    45%

    Koff RS, et al. Viral Hepatitis. 2nd ed. 1994.

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    HBV, HCV

    HCC

    Mutagen effects

    Cirrhosis !

    Aflatoxin

    Fusarium toxin

    Hepatocarcinogenesis

    Etiological

    factors Alcohol

    Androgens

    Metabolic diseases

    Schistosoma

    Classification of tumors of bile ducts

    Cholangiocarcinoma (CC) intrahepatic CC perihilar CC (previously Klatskin tumor) distal (CC)

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    Cholangiocarcinoma.

    Perihilar CC

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    Cholan