Hepatocellular adenoma (patient information)

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Hepatocellular adenoma
Overview
What are the symptoms?
What are the causes?
Who is at highest risk?
Diagnosis
When to seek urgent medical care?
Treatment options
Where to find medical care for Hepatocellular adenoma?
Prevention
What to expect (Outlook/Prognosis)?
Possible complications
Hepatocellular adenoma On the Web
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Images of Hepatocellular adenoma
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Directions to Hospitals Treating Hepatocellular adenoma
Risk calculators and risk factors for Hepatocellular adenoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

Hepatocellular adenoma is an uncommon benign liver adenoma that is most commonly associated with oral contraceptive use in women of childbearing age. It is generally asymptomatic, the typical clinical manifestations include spontaneous rupture or hemorrhage leading to acute abdominal pain with progression to hypotension and even death. There are no specific physical examination findings associated with adenoma. It is more commonly seen in western countries where they are exposed to higher doses of oral contraceptive medications. The estimated incidence is 3 per 1,000,000/year and is 3 to 4 per 100,000 with long term oral contraceptive use. Hepatocellular adenomas are classified on the basis of molecular patterns called phenotypic genotypic classification into 04 groups including; HNF1 alpha inactivated adenoma, beta catenin activated adenoma, inflammatory hepatic adenoma and unclassified type adenoma. The gold standard method for diagnosis of hepatocellular adenoma is excision biopsy of the liver lesions either by surgery or laparoscopically. There is no specific medical therapy for the adenoma, wait & watch policy is recommended for hepatocellular adenomas <5 cm following cessation of oral contraceptives. Annual followup with MRI or ultrasound is recommended until menopause. Surgical resection is the treatment of choice for adenomas that are >5 cm in diameter, that increase in size, lesions with intra tumoral hemorrhage and male patients (irrespective of the adenoma size). The radiofrequency ablation (RFA) and transcatheter arterial embolization (TAE) may be tried in patients who are poor candidates for surgery.

What are the symptoms of Hepatocellular adenoma?

Small hepatocellular adenomas are generally asymptomatic.

• Abdominal pain is the most common presenting symptom in some patients, and the pain is usually related to tumoral hemorrhage.
• Right upper quadrant abdominal fullness or discomfort is present in 40% of cases due to mass effect.
• Eventually, spontaneous rupture or hemorrhage may occur, leading to acute abdominal pain with progression to hypotension and even death.
• Patients with hepatocellular adenomas typically have a history of oral contraceptive use (females) and long term anabolic steroids use (males).

What causes Hepatocellular adenoma?

• The causes of hepatocellular adenoma include;
  • Oral contraceptive medications
    • The causal relationship is proportional to the hormonal dose and duration of medication, highest in women over 30 years of age and after 24 months of using oral contraceptives.
  • Pregnancy
    • It appears to stimulate rapid growth in these lesions with the risk of potentially fatal spontaneous rupture.
  • Glycogen storage disease types I,II and IV
  • Long term use of anabolic androgenic steroids
  • Metabolic syndrome
  • Maturity onset diabetes of young (MODY)
  • Obesity
  • Clomiphene
  • Familial adenomatous polyposis
  • Vascular disorders such as portal vein agenesis, Budd-Chiari syndrome and hereditary hemorrhagic telangiectasia.

Who is at highest risk?

• The most important risk factor in the development of hepatocellular adenoma is use of oral contraceptive medications.

• Drospirenone and Ethinyl estradiol
• Norethindrone acetate and Ethinyl estradiol
• Norgestimate and Ethinyl estradiol
• Norgestrel and Ethinyl estradiol
• The risk is proportional to:

• Hormonal dose
• Duration of medication

• Other risk factors include:

• Clomiphene
• Methyltestosterone
• Danazol
• Klinefelter's syndrome
• Glycogen storage diseases I, II and IV.
• Familial adenomatous polyposis

Risk factors for malignant transformation

The risk factor for malignant transformation of hepatic adenoma to hepatocellular carcinoma is:

• Gender (men)
• Size (> 8 cm)
• Subtype (beta-catenin-activated HCA)

Diagnosis

• If a liver tumor is suspected, your doctor might suggest tests to identify the tumor and its cause. They might also suggest tests to rule out other potential diagnoses.

• An ultrasound is often one of the first steps your doctor will take to help them make a diagnosis. If your doctor finds a large mass through an ultrasound, additional tests might be required to confirm that the mass is a hepatic adenoma.

• Other imaging tests, such as CT scans and MRIs, can be used to learn more about the tumor.

• If the tumor is large, your doctor might also suggest a biopsy. During a biopsy, a small tissue sample is removed from the mass and evaluated under a microscope.

Treatment options

Medical Therapy

• There is no specific medical therapy for the hepatocellular adenoma.
• Historically, hepatocellular adenomas were treated with a wait and watch policy, with surgical intervention recommended for larger (>5cm) tumors.
• In asymptomatic female patients suffering from hepatocellular adenomas, the first step is to stop the offending drug (such as OCPs) and check adenoma size on follow-up.
• The wait and watch policy is recommended when hepatocellular adenomas are <5cm or regress (to <5cm) following cessation of offending drug (OCPs) and no further growth is detected.
• An annual follow-up with MRI or ultrasound is scheduled for patients untill menopause.

Surgical Therapy

• Surgery is the treatment of choice for hepatocellular adenoma, as it can achieved in a controlled and safe manner.
• Elective surgical resection of hepatocellular adenoma is considered for all adenoma lesions >5cm in diameter, lesions that increase in size, lesions with intratumoral hemorrhage and male patients (irrespective of adenoma size).
• Liver transplantation may be considered for patients of hepatocellular adenoma associated with glycogen storage disease type 1.
• In adenoma patients who are poor candidates for surgery (centrally located lesions, multiple adenomas, morbid obesity), radiofrequency ablation (RFA) and transcatheter arterial embolization (TAE) may be considered.
• Radiofrequency ablation (RFA) is a minimally invasive technique that can be used for hepatocellular adenomas, hepatocellular carcinoma and colorectal metastases as well.
• Transcatheter arterial embolization (TAE) is used in adenoma patients with hemodynamic instability due to bleeding hypervascular arterial lesions.

Where to find medical care for Hepatocellular adenoma?

Directions to Hospitals Treating Hepatocellular adenoma

Prevention of Hepatocellular adenoma

An annual follow-up with MRI or ultrasound may be scheduled for female patients until menopause, when the adenoma is < 5cm or regress to < 5cm after discontinuation of oral contraceptive medications.

What to expect (Outlook/Prognosis)?

• The prognosis is usually good for hepatocellular adenoma.
• When diagnosed, the discontinuation of oral contraception or androgen intake leads to regression of hepatocellular adenoma.
• In cases that do not regress after the withdrawal of oral contraception or androgen, surgical treatment is the management of choice.

Possible complications

• When left untreated, hepatic adenomas can rupture spontaneously. This can cause abdominal pain and internal bleeding. A ruptured hepatic adenoma requires immediate medical treatment.

• In rare cases, untreated hepatic adenomas can become cancerous. This is more likely when the tumor is large.

• Several studies suggest that β-catenin activated hepatic adenomas are more likely to become cancerous. Additional research is needed to understand the link between hepatic adenoma types and cancer.