Fibrolamellar hepatocellular carcinoma: Difference between revisions

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:*Elevated serum levels of aspartate aminotransferase (AST)  
:*Elevated serum levels of aspartate aminotransferase (AST)  
:*Elevated serum levels of alanine aminotransferase (ALT)
:*Elevated serum levels of alanine aminotransferase (ALT)
:*Elevated serum levels  of alpha-fetoprotein (unspecific)
:*Elevated transcobalamin I level


===Imaging Findings===
===Imaging Findings===

Revision as of 19:18, 15 April 2016

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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.[1][2] Fibrolamellar hepatocellular carcinoma most commonly in children and young adults.

Historical Perspective

  • Fibrolamellar hepatocellular carcinoma was first described Edmondson in 1956.[1][2]

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

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

  • 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].


References

  1. 1.0 1.1 Michael Torbenson. Fibrolamellar Carcinoma: 2012 Update. http://www.hindawi.com/journals/scientifica/2012/743790/ Access on April 15, 2016
  2. 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. 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.