The Leukemia & Lymphoma Society
Search

.




HEPATOBILIARY (LIVER AND GALLBLADDER) CANCERS

CANCER OF THE LIVER

  • one of the commonest malignancies in the world
  • the highest concentrations in China and Africa
  • the development of this cancer is associated with:
    • Hepatitis B and C infection
    • Carcinogen: aflatoxin is produced in stored grains by Aspergillus flavus
    • Tannic acid
    • Safrole
    • Pyrrollizidine alkaloids
    • Alcohol
    • Cirrhosis
    • Alpha-1 antitrypsin deficiency
    • Hemochromatosis
    • Tyrosinemia
    • Congenital cholestasis
    • Androgens
    • Thorotrast
    • Porphyria
    • Galactosemia
    • Carbon tetrachloride
    • Vinyl chloride


SIGNS AND SYMPTOMS

  • common sites of metastasis include lymph nodes, bones, adrenals, peritoneal surface and bones
    1. abdominal pain
    2. significant weight loss
    3. jaundice
    4. vomiting
    5. hepatomegaly
    6. splenomegaly
    7. bruit over the liver
    8. fever
    9. generalized weakness
    10. ascites
    11. testicular atrophy
    12. palmar erythema
    13. paraneoplastic syndromes: many of these disease states are benign conditions that require no intervention. Some of these are erythrocytosis, hypoglycemia, gynecomastia, testicular atrophy, dysfibrinogenemia, hypercalcemia and porphyria cutanea tarda.
    14. Gastrointestinal bleeding
    15. Pleural effusion
    16. Bone pain
    17. Spontaneous rupture of the liver
      • this is an acute emergency
      • patients usually present with severe right upper quadrant pain
      • common in endemic areas
      • patients may also present with hypovolemic shock
      • treatment : liver resection



FIBROLAMELLAR HEPATOCELLULAR CARCINOMA (HCC)

  • synonym: eosinophilic HCC with lamellar fibrosis
  • usually occurs in younger patients
  • liver is usually non-cirrhotic
  • lesions usually more resectable and it seems to have a higher cure rate
  • lesions are usually calcified hence the hard palpable mass


DIAGNOSIS

  • fine needle aspiration (FNA) or liver biopsy
  • pathognomonic lesion consists of malignant cells containing bile
  • ultrasonography: very sensitive but specificity not as high in detecting small lesions <1cm
  • CT Scan of the liver is not as sensitive as ultrasound scan in detecting small lesions
  • Angiography: demonstration of vascular abnormalities helps in planning surgery and locoregional treatment.
  • Magnetic resonance imaging: better than CT scan
  • Bone scan
  • Chest X-ray
  • Alpha fetoprotein level: patients with anaplastic lesions tend not to have AFP elevation
  • DCP (des-g-carboxy prothrombin) level is more specific in diagnosing HCC
  • Serum glutathione -S-transferase -pi level is also increased


SCREENING

  • necessary in high risk areas
  • effective and cheapest way is to combine AFP measurement with ultrasonography


TREATMENT

  • prognosis is very poor in the vast majority of these cases
  • this depends on a variety of factors e.g. extent of the disease, coexisting cirrhosis and the performance status of the patient.
  • Liver function assessment may be done by measuring the Intrinsic hepatic clearance of Indocyanine green (ICG)
  • RESECTION is the optimal treatment . This is not possible in patients with poor performance status and patients with little or no hepatic reserve.
  • Cryosurgery: used in multiple or unresectable lesions.
  • Liver transplantation: patients with fibrolamellar HCC seem to do better with liver transplant.
  • Percutaneous ethanol injection : can be curative in patients with less than 5cm. In larger lesions, this treatment is palliative.
  • Intra-arterial chemotherapy
  • the use of FUDR, adriamycin, 5-FU, cisplatin, vincristine, mitomycin and vinblastine in various combinations does not produce any significant therapeutic advantage
  • Chemoembolization of the hepatic artery
  • Radiation therapy
  • Radioimmunotherapy

 

CHOLANGIOCARCINOMAS (Cancer of the bile ducts)

  • usually occurs in the fifth and sixth decade of life


SIGNS AND SYMPTOMS

  • abdominal pain and swelling
  • ascites
  • jaundice
  • hepatomegaly (enlargement of the liver)
  • hypercalcemia:may occur without any bone involvement
  • parasitic infestations e.g. Clonorchis sinensis, Opisthorchis viverrini
  • sclerosing cholangitis especially in patients with inflammatory bowel disease
  • cholelithiasis
  • hepatolithiasis
  • polycystic liver disease
  • choledochal cysts


DIAGNOSIS

  • liver function test including bilirubin level
  • alkaline phosphatase
  • CEA, CA 19-9 and less often, alpha fetoprotein may be elevated


TREATMENT

  • most patients present with advanced disease
  • small lesions may be resected
  • liver transplant may be considered but majority of patients present with metastatic deposits at other sites
  • prognosis is very poor in advanced lesions and palliative care might be a reasonable alternative.

 

KLATSKIN'S TUMORS (variant of Cholangiocarcinomas)

  • these are small adenocarcinomas
  • cancer at the junction of the right and left hepatic ducts
  • very rare
  • may be confused with pancreatic cancer
  • diagnostic studies include CT scans, ultrasonography and cholangiography
  • ERCP is also useful
  • Arteriography
  • Surgical resection is the only hope for long term survival
  • In patients with locoregional (local) recurrence, stent placement may be necessary for bile drainage
  • Palliative treatments in patients with advanced disease

 

CANCER OF THE GALLBLADDER

  • common in American Indians
  • usually presents with advanced lesions at diagnosis
  • associated with cholecystitis, cholelithiasis and gallbladder polyps
  • signs and symptoms include right upper quadrant pain, nausea, vomiting, jaundice, anorexia and significant weight loss
  • Ultrasonography is useful in making a diagnosis
  • Treatment is palliative if there is a preoperative evidence of metastasis
  • Surgical resection in patients with small lesions or localized and contiguous liver lesions
  • The extent of the disease determines the extent of the surgical procedure
  • Radiation therapy may be used for palliation of symptoms and in some cases, some degree of tumor shrinkage is possible.
  • Chemotherapy is not effective.