Fibrolamellar hepatocellular carcinoma
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 most commonly in children and young adults.
This variant usually arises in non-cirrhotic livers of adolescents or young adults {353}. It is rare in Asian and African countries but not so rare in Western countries. The tumour cells grow in sheets or small trabeculae that are separated by hyalinized collagen bundles with a characteristic lamellar pattern. They are large and polygonal and have a deeply eosinophilic and coarsely granular cytoplasm and distinct nucleoli. The eosinophilic granularity is due to the presence of a large number of mitochondria. Pale bodies are frequently present, and stainable copper, usually in association with bile, can occasionally be shown
Fibrolamellar hepatocellular carcinoma most commonly in children and young adults. [1]
Historical Perspective
Classification
- There is no classification for fibrolamellar hepatocellular carcinoma.
Pathophysiology
- The pathogenesis of fibrolamellar hepatocellular carcinoma is characterized by:
- Lack of cirrhosis
- The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
- 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
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 [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].
Age
- Patients of all age groups may develop [disease name].
- Fibrolamellar hepatocellular carcinoma is more commonly observed among patients aged [age range] years old.
- Fibrolamellar hepatocellular carcinoma is more commonly observed among [elderly patients/young patients/children].
Gender
- Fibrolamellar hepatocellular carcinoma affects men and women equally.
- [Gender 1] are more commonly affected with [disease name] than [gender 2].
- The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
Race
- There is no racial predilection for [disease name].
- Fibrolamellar hepatocellular carcinoma usually affects individuals of the [race 1] race.
- [Race 2] individuals are less likely to develop [disease name].
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 [complication 1], [complication 2], and [complication 3].
- Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].
Diagnosis
Diagnostic Criteria
- The diagnosis of fibrolamellar hepatocellular carcinoma is made with the following diagnostic criteria:
- [criterion 1]
- [criterion 2]
- [criterion 3]
- [criterion 4]
Symptoms
- Fibrolamellar hepatocellular carcinoma is usually asymptomatic.
- Symptoms of [disease name] may include the following:
- [symptom 1]
- [symptom 2]
- [symptom 3]
- [symptom 4]
- [symptom 5]
- [symptom 6]
Physical Examination
- Patients with [disease name] usually appear [general appearance].
- Physical examination may be remarkable for:
- [finding 1]
- [finding 2]
- [finding 3]
- [finding 4]
- [finding 5]
- [finding 6]
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].
References
- ↑ 1.0 1.1 Michael Torbenson. Fibrolamellar Carcinoma: 2012 Update. http://www.hindawi.com/journals/scientifica/2012/743790/ Access on April 15, 2016
- ↑ 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.