Fibrolamellar hepatocellular carcinoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Fibrolamellar carcinoma; FLC

Overview

Fibrolamellar hepatocellular carcinoma (FLC) is a rare subtype of primary liver cancer. Fibrolamellar hepatocellular carcinoma was first described Edmondson in 1956. Fibrolamellar hepatocellular carcinoma most commonly in children and young adults.

Historical Perspective

• Fibrolamellar hepatocellular carcinoma was first described Edmondson in 1956.

Classification

• There is no classification for fibrolamellar hepatocellular carcinoma.

Pathophysiology

• The pathogenesis of fibrolamellar hepatocellular carcinoma is characterized by:

• Lack of cirrhosis

• The overexpression of DNAJB1-PRKACA gene has been associated with the development of fibrolamellar hepatocellular carcinoma.
• On gross pathology characteristic findings of fibrolamellar hepatocellular carcinoma, include:

• Hard, scirrhous, and well-circumscribed
• Tumor bulging
• white-brown tumor with fibrous bands throughout and central stellate scar

• On microscopic histopathological analysis, characteristic findings of fibrolamellar hepatocellular carcinoma, include:

• Tumor cells grow in sheets
• Trabeculae that are separated by collagen bundles (lamellar pattern)
• Large cells that contain abundant mitochondria
• Coarsely granular cytoplasm

• On immunohistochemistry, characteristic findings of fibrolamellar hepatocellular carcinoma, include:

• Positive staining for hepatocyte paraffin 1 (HepPar1)
• Positive staining for glypican-3 (GPC3)
• Positive staining polyclonal carcinoembryonic antigen (pCEA)
• CD10 positivity

Causes

• Common causes of fibrolamellar hepatocellular carcinoma, include:

• Active hepatic inflammation
• Hepatitis B or C viral infection
• Alcohol-related liver disease
• Nonalcoholic fatty liver disease
• Dietary aflatoxin B1

Differentiating Fibrolamellar Hepatocellular Carcinoma from Other Diseases

• Fibrolamellar hepatocellular carcinoma must be differentiated from other diseases that cause abdominal pain, weight loss, and malaise such as:

• Hepatocellular carcinoma
• Focal nodular hyperplasia
• Hepatic adenoma
• Hepatic metastasis

Epidemiology and Demographics

• The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
• In 2012, the incidence of fibrolamellar hepatocellular carcinoma was estimated to be 0.02 cases per 100,000 individuals in United States.

Age

• Fibrolamellar hepatocellular carcinoma is more commonly observed among patients aged 15 to 30 years old.
• Fibrolamellar hepatocellular carcinoma is more commonly observed among young patients.

Gender

• Fibrolamellar hepatocellular carcinoma affects men and women equally.

Race

• There is a racial predilection for Caucasian race in patients with fibrolamellar hepatocellular carcinoma.

Risk Factors

• There are no risk factors for the development of fibrolamellar hepatocellular carcinoma.

Natural History, Complications and Prognosis

• The majority of patients with fibrolamellar hepatocellular carcinoma remain asymptomatic for years.
• Early clinical features include abdominal pain, weight loss, and malaise.
• If left untreated, the majority of patients with fibrolamellar hepatocellular carcinoma may progress to develop metastasis to abdominal lymph nodes, peritoneum, and lung.
• Common complications of fibrolamellar hepatocellular carcinoma, include:

• Hepatic failure
• Caval compression syndrome
• Gynecomastia
• Cold agglutinin disease

• Prognosis will depend on stage at diagnosis. The average survival of patients with fibrolamellar carcinoma in the United States is 73% at 1 year and 32% at 5 years.

Diagnosis

Diagnostic Criteria

• The diagnosis of fibrolamellar hepatocellular carcinoma is made with the following diagnostic criteria:

• Positive imaging findings

• Central scar
• Small calcifications
• Single large tumor

• [criterion 3]
• [criterion 4]

Symptoms

• Fibrolamellar hepatocellular carcinoma is usually asymptomatic.
• Symptoms of fibrolamellar hepatocellular carcinoma may include the following:

• Fatigue
• Weight loss
• Abdominal distension
• Nausea

Physical Examination

• Patients with fibrolamellar hepatocellular carcinoma may be well-appearing, or cachectic.
• Physical examination of the abdomen may be remarkable for:

Auscultation

• Positive liver scratch test for enlarged liver size.
• Cruveilhier-Baumgarten murmur

• A venous hum in patients with portal hypertension

Percussion

• Dull percussion

Palpation

• Abdominal mass
• Tenderness in right upper quadrant
• Hepatomegaly
• Other physical signs for fibrolamellar hepatocellular carcinoma, may include:
• Pallor
• Jaundice
• Plantar and palmar erythema

Laboratory Findings

• There are no specific laboratory findings associated with [disease name].

• A [positive/negative] [test name] is diagnostic of [disease name].
• An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
• Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

• There are no [imaging study] findings associated with [disease name].

• [Imaging study 1] is the imaging modality of choice for [disease name].
• On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
• [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

• Fibrolamellar hepatocellular carcinoma may also be diagnosed using [diagnostic study name].
• Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

• There is no treatment for [disease name]; the mainstay of therapy is supportive care.

• The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

• Surgery is the mainstay of therapy for [disease name].
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
• [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

• There are no primary preventive measures available for [disease name].

• Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].