Neoplasms/Malignancies

     

    Liver Tumors / Cysts

    Hepatocellular Carcinoma

    • HCC is a 3rd leading cancer cause worldwide.
    • Incidence rates rising in Western countries, and 80% have cirrhosis.
    • Risk factors: 
      • Male sex
      • Chronic Hep B/C infection.
      • Hemochromatosis
      • Alpha-1-antitrypsin deficiency 
      • (Any kind of cirrhosis)
    • Screening / Surveillance
      • Cross-sectional imaging (Ultrasound is least epensive, CT - better resolution, detection of early stage).
    • Screening guidelines for HCC:

      • All patients with cirrhosis should be screened for HCC q6mo (every 6 months) with cross-sectional imaging.
      • Serum AFP --> Not sufficient diagnostic accuracy alone for early detection.

      (Can do q12mo if hard to follow)

     

    • Some patients do q12mo if hard to get back to clinic to do this screening.
    • Diagnosis:
      • Lesions >2cm in diameter can be diagnosed with contrast enhanced CT/MRI/Angiography.
        • HCC derive their blood supply from neovascularization (from branches of hepatic artery).  This characteristic allows it to be identified by contrast-enhanced imaging (contrast CT or MRI). 
        • If positive for HCC --> can diagnose and treat as HCC. (Biopsy may not be required)
        • If negative --> does not rule out malignancy (can consider biopsy?)
        • Diagnosis of HCC:

          • At least TWO imaging studies showing lesion with HCC features
            (Arterial hypervascularization with delayed venous contrast washout)
            OR
          • If only one imaging study available, can diagnose if serum AFP level ≥ 400
      • Image guided biopsy: ONLY for patients in whom radiologic criteria are equivocal or inconsistent with HCC.
        • Negative biopsy result in cirrhosis does not confidently exlude HCC.
        • Tumor seeding from biopsy described, but rare.
      • 1 - 2 cm indeterminate lesions --> enhanced follow-up with more frequent imaging is required.
    • Treatment:
      • Guided by Barcelona Classification for Liver Cancer (BCLC)  
        ---> see flow chart (look in  MKSAP16  PMID: 18477802)
        • Manage based on stage of liver disease (Childs-Pugh Classification - A/B/C/D)
        • Examples:
          • Early stage liver disease with small lesion + stable liver function: resect lesion. (enough liver preserved)
          • Multiple small lesions (3 of ≤ 3cm) and early stage disease --> liver transplant. 
          • Indeterminant stage or multinodular disease --> Transarterial hepatic artery chemoembolization (TACE)
          • Advanced stage, portal invasion --> Sorafenib
      • Transplantation is most effective for early disease.

     

    Hepatic Cysts

    • Hepatic cysts --> benign liver finding commonly on liver imaging.
      • Usually a thin wall with no internal structures, asymptomatic.
    • Classified:
      • Small Simple cysts < 4cm --> not significant.
      • Large Simple cysts > 4cm  --> rarely symptoms (abdo pain, nausea, discomfort)
        • Laparoscopic fenestration can be done (slits made in cysts allowing to drain).
        • Needle aspiration not effective (recur).
        • Usually not symptomatic, if symptoms --> concern for cystadenoma
      • Non-Simple Cysts: (On ultrasound)
        • Wall irregularity + Internal echos --> cystadenomas.
    • Cystadenoma:
      • Risk of malignant transformation to a cystadenocarcinoma.
      • Must resect all suspected cystadenomas.
        • b/c hard to distinguish cystadenoma from cystadenocarcinoma on imaging and histology.

     

    Focal Nodular Hyperplasia

    • Benign liver lesion --> hypertrophic liver reaction to an anomlaous artery
    • FNH: most found incidentally.
    • Diagnosis: Radiographically (see anomalous artery as a pathognemonic "central scar")
      • If no central scar, hard to distinguish hyperplasia vs. adenoma.
      • Sometimes liver biopsy is needed.
    • 5cm indeterminate lesion in a non-cirrhotic liver can be monitored by U/S q6mo to confirm no growth. (avoids liver biopsy).
    • Management:
      • No malignant potential, no other complications.
      • Reasurre patient.

     

    Hepatic Adenoma

    • Commonly confused with focal nodular hyperplasia (FNH) (sometimes hard to distinguish radiologically)
    • Histologically sheets of hepatocytes without biliary structures.
    • Common in women, associated with oral contraceptives.
    • Management:
      • Risk of malignant transformation or rupture associated with bleeding.
      • If > 5cm OR pregnancy considered
        • Surgical resection (to reduce risk of rupture or malignant transformation)
      • If < 5cm in diameter:
        • Discontinue OCP AND sequential imaging (check regression)

     

    Hepatic Hemangioma

    • Common benign liver findings.
      • >5cm --> called giant hemangioma.
    • Symptoms:
      • RUQ fullness / discomfort.
    • Diagnosis: Contrast CT or MRI (peripheral nodular enhancement, followed by centripetal filling).
    • Management:
      • Excellent prognosis.
      • Small hemangiomas can be reassured. 
      • Even giant hemangioma: risk of bleeding too low to warrant resection.
      • Consider surgery for:
        • Symptomatic hemangiomas or sequential growth.

     

     

    Biliary Neoplasms

    Gallbaldder Cancer

    • Most common biliary malignancy
    • Risk Factors: >50yo, Female, Gall stones, obesity, gallbladder pollyps >1cm, chronic infection (salmonela typhii), porcelain gall bladder.
    • To prevent gall bladder cancer, prophylactic cholecystectomy considered in:
      • Gall bladder polyps >1cm
      • Gall stones >3cm
      • Porcelain gall bladder

     

    • Uncommon, but fatal, diagnosed in advanced stage.
    • Symptoms: Nonspecific: abdo discomfort, nausea, weight loss
    • Labs: normal until obstructive jaundice develops
    • Diagnosis:
      • Often diagnosed intra-op during cholecystectomy for gall bladder symptoms OR on abdo imaging.
      • U/S, CT, MRI can reveal abnormalities in gall bladder wall.
    • Management:
      • Depends on stage.
      • Localized --> Open cholecystectomy.
      • Advanced Tumors --> Curative therapy not possible.
        • Palliative: operative diversion of bile flow, endoscopic stenting.
        • Chemotherapy/Radiation benefits not demonstrated.

     

    Cholangiocarcinoma

    • Cancer derived from biliary tree (not gall bladder or ampulla)
    • Risk Factors: Primary sclerosing cholangitis, biliary atresia, chronic infection (liver flukes), biliary cists.
    • Screening: No evidence for screening (imaging +/- tumor markers possible)
    • Suspect in patients with PSC with signs of clinical decompensation.
    • Management:
      • Classified:
        • Intrahepatic:
          • Often asymptomatic until advanced (RUQ discomfort, weight loss, fever, ALP can be up)
          • If advanced: poor prognosis, curative resection often not possible.  
        • Hilum (Klatskin tumor​) 
          • Jaundice (complete obstruction of biliary tree)
          • Suspect diagnosis: imaging shows bile duct dilatation + obstructing lesion
          • Diagnosis: Endoscopic cholangiography with brushings/biopsy
          • Management: Resection (if not invated intrahepatic ducts), poor survival
            • Liver transplant also done, but no data yet.
        • Extrahepatic (distal): 
          • ​Presentation / diagnosis is same as Hilum Tumor.
          • Management: resection (curative intent)
      • Chemo/Rads benefit unknown

     

    Ampullary Adenocarcinoma

    • RARE, often in hereditary polyposis syndromes (FAP).
    • FAP patients should undergo regular endoscopy to screen.
    • Presents: obstructive jaundice.  Dx: endoscopic biopsy and imaging with CT.
    • Resectable, good prognosis. (Whipple procedure is recommended)

     

     

    Biliary Cysts

    • Diagnosis considered when dilatation of bile duct is found without evidence of obstructing lesion.
    • Symptoms: chronic intermittent abdo pain, recurrent bouts of cholangitis / jaundice.
    • Diagnosis:
      • Abdo CT / US can suggest cysts --> DO MRCP or ERCP
        • To diagnose use: MRCP, ERCP, or EUS (Endoscopic Ultrasound)
        • --> define cystic dilatation with no obstruction
    • Management:
      • 20-fold increase risk of cholangiocarcinoma  --> should be resected.
        • Surgical resection of the entire cyst + hepaticojejunostomy formation indicated.
        • Reduces risk of malignancy and alleviates further cholangitis.
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