Plain Language Summaries

Summary of “A Systematic Review: Treatment and Prognosis of Patients with Fibrolamellar Hepatocellular Carcinoma.”

Published August, 2012 in Journal of the American College of Surgeons

Fibrolamellar hepatocellular carcinoma (FLC) is a rare primary liver tumor presenting earlier in life than nonfibrolamellar hepatocellular carcinoma (NFL-HCC), with distinct epidemiologic and clinical characteristics. Although FLC is believed to have a better prognosis than NFL-HCC, data on treatment and prognosis are scarce. Writing in 2012, the authors performed a systematic review of scientific literature and pooled analysis of individual patient data to investigate treatment options and clinical outcomes of patients with FLC.

A total of 35 series were analyzed, reporting on 575 patients (52% female, elevated alpha-fetoprotein in 10%, cirrhosis in 3%, hepatitis B in 2%), most of whom were treated with partial hepatectomy (55%) or liver transplantation (23%). Nineteen studies provided data on 206 individual patients with a median age of 21 years and tumor size of 12 cm. Median overall survival (OS) was 39 months; 1-year, 3-year, and 5-year OS rates were 85%, 53%, and 44%, respectively. For patients treated with liver resection, median OS was 18.5 years and 1-year, 3-year, and 5-year OS were 93%, 80%, and 70%, respectively. Based on data from 15 studies, FLC appeared to follow a relatively indolent course compared with NFL-HCC.

Patients with FLC treated with partial hepatectomy have excellent long-term survival, with 5-year overall survival reaching 70%. Patients fared worse with the use of other therapeutic options including chemotherapy, intra-arterial therapy, and transplantation, although data directly comparing resection vs transplantation were limited.

Aggressive surgical management of recurrent lymph node and pancreatic head metastases of resected fibrolamellar hepatocellular carcinoma: a case report.

Plain English Summary
Surgery is almost always performed when Fibrolamellar is found to be exclusively in the liver.  In cases where the Fibrolamellar has metastasized elsewhere, the course of action is less clear.  The authors present a case of a women who was diagnosed with Fibrolamellar at the age of 28.  Upon removal (resection) of the tumor, one of her lymph nodes was found to contain signs of the tumor cells (they were rated “positive” for the disease).  A metastatic tumor was removed 23 months after the initial surgery, then a second 18 months later, then third sixteen months later.  At the 90-month mark, a tumor was found in the pancreas.  This was removed and 18 months later the patient is still clear. While this is just one example, the results suggest that continued surgical removal may continue to be warranted, even after repeated metastases.

Authors’ Abstract
JOP. 2012 Sep 10;13(5):529-32. doi: 10.6092/1590-8577/839.
Aggressive surgical management of recurrent lymph node and pancreatic head metastases of resected fibrolamellar hepatocellular carcinoma: a case report.Wojcicki MLubikowski JPost MChmurowicz TWiechowska-Kozlowska AKrawczyk M.
Department of Hepatobiliary Surgery and Liver Transplantation, Marie Curie Hospital. Szczecin, Poland.
Context Fibrolamellar hepatocellular carcinoma is a rare liver tumor with the propensity to metastasize to the lymph nodes months or years after initial surgery. However, its metastatic spread to the pancreas was previously reported only in a child. 
Case report We present an unusual case of a young female patient who was repeatedly treated by surgical excision of abdominal and mediastinal lymph node recurrences between 2 and 6 years after left hepatic lobectomy for fibrolamellar hepatocellular carcinoma. At 8 years following her initial surgery, the patient was diagnosed with pancreatic head metastasis and a pancreaticoduodenectomy was performed. Postoperative course was uneventful and the patient did not experience recurrence within the last 18 months. 
Conclusion The metastasis of fibrolamellar hepatocellular carcinoma to the pancreas is highly exceptional but possible and its excision appears warranted as well.

Prolonged Complete Response after GEMOX Chemotherapy in a Patient with Advanced Fibrolamellar Hepatocellular Carcinoma.

Plain English summary: 
The only drug approved for patients with advanced primary liver cancer  (hepatocellular carcinoma, or HCC) is sorafenib, also known by its brand name of Nexavar.  In HCC there is often significant damage to the liver (cirrhosis).  The authors of this study wondered if chemotherapeutics that are considered too dangerous for use with patients with significant liver damage, might work in patients with liver cancer who do not also have significant damage from cirrhosis.  Fibrolamellar is unlikely to be the result of long-term damage to the liver.  The study examined a 25 year old women who, after her primarly tumor was removed (resected) from her liver, was found to have metasteses in her lymph nodes.   The patient received alternating mixtures of two drugs: gemcitabine-oxaliplatin, and five years after treatment was stopped, the woman was still clear of any signs of fibrolamellar. 

Author’s abstract

Gras P, Truant S, Boige V, Ladrat L, Rougier P, Pruvot FR, Hebbar M.
Case Rep Oncol. 2012 Jan;5(1):169-72. Epub 2012 Apr 3

The following is the published abstract for this paper.  The complete paper is available for free.  
The only currently validated treatment for advanced hepatocellular carcinoma (HCC) is sorafenib. However, sorafenib has been mainly studied in patients with HCC developed in cirrhotic liver. Chemotherapy might be more suitable for patients with HCC in non-cirrhotic liver. We report the case of a young woman with fibrolamellar HCC in a non-cirrhotic liver, with histologically proven metastatic ganglionary relapse after surgical resection of the primary tumour. Chemotherapy with gemcitabine and oxaliplatin (GEMOX regimen) achieved a complete response without relapse five years after discontinuation of chemotherapy. This exceptional case raises the question of clinical trials specifically designed for patients with HCC in non-cirrhotic liver.