ALTERNATE NAMES

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 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

DESCRIPTION

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.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

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:

• Abdominal pain;

• Loss of appetite;

• Weight loss;

• Malaise;

• Jaundice;

• Nausea and/or vomiting;

• Palpable liver mass;

• Migratory thrombophlebitis or venous thrombosis; and

• Gynecomastia (excessive breast tissue in males).

ICD-9: 155.0

PROGRESSION

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.

TREATMENT

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.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for evaluation:

• Clinical history and examination that describes the diagnostic features of the impairment.

• Pathology/biopsy report of the cancer.

• CT scan, MRI scan, or ultrasound reports.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

13.19

113.03

Meets 13.19 for adults. Meets 113.03 for children with initial or recurrent disease.