Fibrolamellar hepatocellular carcinoma: Difference between revisions
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{{SK}}Eosinophilic hepatocellular carcinoma with lamellar fibrosis, Polygonal cell hepatocellular carcinoma with fibrous stroma, Hepatocellular carcinoma with increased stromal fibrosis, Eosinophilic glassy cell hepatoma, and fibrolamellar oncocytic hepatoma. | {{SK}}Eosinophilic hepatocellular carcinoma with lamellar fibrosis, Polygonal cell hepatocellular carcinoma with fibrous stroma, Hepatocellular carcinoma with increased stromal fibrosis, Eosinophilic glassy cell hepatoma, and fibrolamellar oncocytic hepatoma. | ||
==Risk Factors== | ==Risk Factors== | ||
Revision as of 17:36, 2 April 2019
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Synonyms and keywords:Eosinophilic hepatocellular carcinoma with lamellar fibrosis, Polygonal cell hepatocellular carcinoma with fibrous stroma, Hepatocellular carcinoma with increased stromal fibrosis, Eosinophilic glassy cell hepatoma, and fibrolamellar oncocytic hepatoma.
Risk Factors
There are no established risk factors for [disease name].
OR
The most potent risk factor in the development of [disease name] is [risk factor 1]. Other risk factors include [risk factor 2], [risk factor 3], and [risk factor 4].
OR
Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
OR
Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral.
Screening
There is insufficient evidence to recommend routine screening for [disease/malignancy].
OR
According to the [guideline name], screening for [disease name] is not recommended.
OR
According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3].
Natural History, Complications, and Prognosis
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
OR
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
OR
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
Diagnosis
Diagnostic Study of Choice
The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].
OR
The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].
OR
The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].
OR
There are no established criteria for the diagnosis of [disease name].
History and Symptoms
The majority of patients with [disease name] are asymptomatic.
OR
The hallmark of [disease name] is [finding]. A positive history of [finding 1] and [finding 2] is suggestive of [disease name]. The most common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3]. Common symptoms of [disease] include [symptom 1], [symptom 2], and [symptom 3]. Less common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3].
Physical Examination
Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].
OR
Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
The presence of [finding(s)] on physical examination is diagnostic of [disease name].
OR
The presence of [finding(s)] on physical examination is highly suggestive of [disease name].
Laboratory Findings
An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].
OR
Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
OR
[Test] is usually normal among patients with [disease name].
OR
Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].
OR
There are no diagnostic laboratory findings associated with [disease name].
Electrocardiogram
There are no ECG findings associated with [disease name].
OR
An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
X-ray
There are no x-ray findings associated with [disease name].
OR
An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
Echocardiography or Ultrasound
There are no echocardiography/ultrasound findings associated with [disease name].
OR
Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
CT scan
There are no CT scan findings associated with [disease name].
OR
[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
MRI
There are no MRI findings associated with [disease name].
OR
[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
Other Imaging Findings
There are no other imaging findings associated with [disease name].
OR
[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
Other Diagnostic Studies
There are no other diagnostic studies associated with [disease name].
OR
[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].
Treatment
Medical Therapy
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
OR
Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
OR
The majority of cases of [disease name] are self-limited and require only supportive care.
OR
[Disease name] is a medical emergency and requires prompt treatment.
OR
The mainstay of treatment for [disease name] is [therapy].
OR The optimal therapy for [malignancy name] depends on the stage at diagnosis.
OR
[Therapy] is recommended among all patients who develop [disease name].
OR
Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
OR
Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
OR
Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
OR
Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
Surgery
Surgical intervention is not recommended for the management of [disease name].
OR
Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]
OR
The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].
OR
The feasibility of surgery depends on the stage of [malignancy] at diagnosis.
OR
Surgery is the mainstay of treatment for [disease or malignancy].
Primary Prevention
There are no established measures for the primary prevention of [disease name].
OR
There are no available vaccines against [disease name].
OR
Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
OR
[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].
Secondary Prevention
There are no established measures for the secondary prevention of [disease name].
OR
Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].
References
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [3]
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 is most commonly seen in children and young adults. The pathogenesis of fibrolamellar hepatocellular carcinoma is characterized by the lack of cirrhosis. Common causes of fibrolamellar hepatocellular carcinoma, include: active hepatic inflammation, hepatitis B or C viral infection, alcohol-related liver disease, nonalcoholic fatty liver disease, and dietary aflatoxin B1. 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, and cold agglutinin disease.
Historical Perspective
Classification
- There is no classification for fibrolamellar hepatocellular carcinoma.[1]
Pathophysiology
- The pathogenesis of fibrolamellar hepatocellular carcinoma is characterized by the lack of cirrhosis.[1]
- 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 growing 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:[1]
- 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:[1]
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.[1]
- FLC is a very rare tumor, although the incidence varies geographically. In the United States and Thailand, less than 1 percent of all primary liver tumors are FLC [3,4], while in Mexico, FLC represents 5.8 percent of all primary liver cancers
Age
- The median age of fibrolamellar hepatocellular carcinoma diagnosis is 33 years.[1]
- 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.[1]
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.[1]
- 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:[1]
- Positive imaging findings
- Central scar
- Small calcifications
- Single large tumor
- Clinical criteria:
- Young onset
- No previous history of liver disease
Symptoms
- Fibrolamellar hepatocellular carcinoma is usually asymptomatic.
- Symptoms of fibrolamellar hepatocellular carcinoma may include the following:[1]
- 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:[1]
Auscultation
- Positive liver scratch test for enlarged liver size.
Percussion
- Dull percussion
Palpation
- Abdominal mass
- Tenderness in right upper quadrant
- Hepatomegaly
- Other physical signs for fibrolamellar hepatocellular carcinoma, may include:[1]
- Pallor
- Jaundice
- Plantar and palmar erythema
Laboratory Findings
- Laboratory findings consistent with the diagnosis of fibrolamellar hepatocellular carcinoma, include:[1]
- 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:
- Single large tumors
- Central scar (seen in ~75% of cases)
- Central scar shows persistent enhancement on delayed contrast enhanced CT.
- On MRI, findings of fibrolamellar hepatocellular carcinoma, include:[1]
- 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.[1]
- Findings on PET scan, include:
- Technetium-99m sulphur colloid scans (taken up by Kupffer cells) are useful as these tumors will not accumulate the agent, whereas FNH does.
Treatment
Medical Therapy
- Chemotherapy is the treatment of choice for fibrolamellar hepatocellular carcinoma.[1]
Surgery
- Surgical resection is the treatment of choice for fibrolamellar hepatocellular carcinoma.
Prevention
- There are no primary preventive measures available for fibrolamellar hepatocellular carcinoma.
- Once diagnosed and successfully treated, patients with fibrolamellar hepatocellular carcinoma are followed-up every 3, 6 or 12 months.[1]
- Follow-up testing include ultrasound, physical exam, and laboratory testing.
References
- ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 1.12 1.13 1.14 1.15 1.16 1.17 1.18 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.