Fibrolamellar hepatocellular carcinoma: Difference between revisions
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=== Surgery === | === Surgery === | ||
* | *Surgical resection is the treatment of choice for fibrolamellar hepatocellular carcinoma | ||
=== Prevention === | === Prevention === | ||
*There are no primary preventive measures available for [disease name]. | *There are no primary preventive measures available for [disease name]. | ||
Revision as of 19:36, 15 April 2016
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.[1][2] Fibrolamellar hepatocellular carcinoma most commonly in children and young adults.
Historical Perspective
Classification
- There is no classification for fibrolamellar hepatocellular carcinoma.
Pathophysiology
- The pathogenesis of fibrolamellar hepatocellular carcinoma is characterized by the 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
- In 2012, the incidence of fibrolamellar hepatocellular carcinoma was estimated to be 0.02 cases per 100,000 individuals in United States.
Age
- The median age of fibrolamellar hepatocellular carcinoma diagnosis is 33 years
- Fibrolamellar hepatocellular carcinoma is more commonly observed among patients aged 15 to 40 years old.[3]
- Fibrolamellar hepatocellular carcinoma is more commonly observed among young patients.[3]
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
- Laboratory findings consistent with the diagnosis of fibrolamellar hepatocellular carcinoma, include
- Elevated serum levels of aspartate aminotransferase (AST)
- Elevated serum levels of alanine aminotransferase (ALT)
- Elevated serum levels of alpha-fetoprotein (unspecific)
- Elevated transcobalamin I level
Imaging Findings
- CT is the imaging modality of choice for fibrolamellar hepatocellular carcinoma
- On CT, findings of fibrolamellar hepatocellular carcinoma, include:
- On MRI, findings of fibrolamellar hepatocellular carcinoma, include:
- T1: typically iso to hypointense to the liver
- T2: hypo to slightly hyperintense
- T1C+: arterial phase: heterogeneous enhancement/ portal delayed phase: iso to hypointense
Other Diagnostic Studies
- Fibrolamellar hepatocellular carcinoma may also be diagnosed using PET.
- Findings on PET scan, include:
- Technetium-99m sulphur colloid scans (taken up by Kupffer cells) are useful as these tumours will not accumulate the agent, whereas FNH does.
Treatment
Medical Therapy
- The medical therapies for fibrolamellar hepatocellular carcinoma, include:
Surgery
- Surgical resection is the treatment of choice for fibrolamellar hepatocellular carcinoma
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].
References
- ↑ 1.0 1.1 Michael Torbenson. Fibrolamellar Carcinoma: 2012 Update. http://www.hindawi.com/journals/scientifica/2012/743790/ Access on April 15, 2016
- ↑ 2.0 2.1 EDMONDSON HA (1956). "Differential diagnosis of tumors and tumor-like lesions of liver in infancy and childhood". AMA J Dis Child. 91 (2): 168–86. PMID 13282629.
- ↑ 3.0 3.1 Aramaki M, Kawano K, Sasaki A, Ohno T, Tahara K, Kai S, Iwashita Y, Kitano S (2005). "Hepatocellular carcinoma in young adults". Hepatogastroenterology. 52 (66): 1795–7. PMID 16334779.