ALTERNATE NAMES
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Fibrolamellar Hepatocellular Carcinoma; Fibrolamellar Carcinoma; Eosinophilic Hepatocellular
Carcinoma with Lamellar Fibrosis; Eosinophilic Hepatocellular Cancer with Lamellar
Fibrosis; Polygonal Cell Hepatocellular Carcinoma with Fibrous Stroma; Polygonal Cell
Hepatocellular Carcinoma with Fibrous Stroma; Polygonal Cell Hepatocellular Carcinoma
with Fibrous Stroma; Polygonal Cell Hepatocellular Carcinoma with Fibrous Stroma;
Polygonal Cell Hepatocellular Cancer with Fibrous Stroma; Hepatocellular Carcinoma
with Increased Stromal Fibrosis; Hepatocellular Cancer with Increased Stromal Fibrosis;
Eosinophilic Glassy Cell Hepatoma; Fibrolamellar Oncocytic Hepatoma; Fibrolamellar
Variant of Hepatocellular Carcinoma; Fibrolamellar Variant of Hepatocellular Cancer;
FL-HCC
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DESCRIPTION
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Fibrolamellar Carcinoma (FL-HCC) is a rare and aggressive form of liver cancer, which is generally diagnosed in adolescents
and young adults (before age 40). Many people with early FL-HCC have no signs or symptoms
of the condition. When present, symptoms are often nonspecific (i.e. abdominal pain,
weight loss, malaise) and blamed on other, more common conditions. The exact underlying
cause of FL-HCC is poorly understood, although a novel DNAJB1-PRKACA fusion gene resulting
from a chromosome 19 mutation is present in most cases and distinguishes it from other
liver cancers. FL-HCC cells are about three times larger than normal liver cells.
Unlike other forms of liver cancer, FL-HCC typically occurs in the absence of underlying
liver inflammation, scarring or infection by hepatitis B or C; thus, specific risk
factors for this condition remain unidentified.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING
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Diagnostic testing: The diagnosis of FL-HCC is made by imaging study such as ultrasound, CT scan or
MRI scan, and by microscopic evaluation of biopsy material.
Physical findings: The signs and symptoms of FL-HCC may include:
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Migratory thrombophlebitis or venous thrombosis; and
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Gynecomastia (excessive breast tissue in males).
ICD-9: 155.0
ICD-10: C22.0
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PROGRESSION
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The long-term prognosis for people with FL-HCC is generally better than other forms
of liver cancer, particularly when treated with surgical resection. The reported 5-year
survival rates range from 51 to 70% following liver resection. The prognosis is less favorable in people with unresectable disease. The median survival
of these patients is approximately 14 months. FL-HCC has a recurrence rate of about
50% within three years and about 80% within five years. Metastases to the lymph nodes
and distant sites are present in about half of patients at diagnosis.
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TREATMENT
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FL-HCC is usually treated with surgery to remove the tumor and the surrounding lymph
nodes (resection) the same as other hepatocellular carcinomas. Liver transplantation
may be considered for individuals who are not candidates for surgical resection. The
Food and Drug Administration (FDA) has approved the use of Sorafenib (Nexavar) an
orphan chemotherapy drug for treatment of FL-HCC. However, Sorafenib has shown only
limited efficacy against FL-HCC.
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SUGGESTED PROGRAMMATIC
ASSESSMENT*
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Suggested MER for
Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment;
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Pathology/biopsy report of the cancer; and
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CT scan, MRI scan, or ultrasound reports.
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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13.19
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Meets 13.19 for adults.
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113.03
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Meets 113.03 for children with initial or recurrent disease.
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Equals
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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