Treatment of fibrolamellar hepatoma with subtotal hepatectomy or transplantation
Authors: A Pinna
Journal: Hepatology (1997)

][9][10][11][12][13][14][15][16][17] In our series, we have compared the of hepatocellular carcinoma (HCC), distinguished by histopathological features suggesting greater differentiation than survival of patients with FL-HCC with that of patients with conventional HCC who were treated by the same team over conventional HCC.However, the optimal treatment and the prognosis of FL-HCC have been controversial.Follow-up a 27-year period.studies are available from 1 year to 27 years, after 41 patients with FL-HCC were treated with partial hepatectomy (PHx) PATIENTS AND METHODS (28 patients) or liver transplantation (13 patients). In this Case Materialretrospective study, the effect on outcome was determined for the pTNM stage and other prognostic factors routinely Between 1968 and 1995, 477 patients with hepatoma underwent recorded at the time of surgery.Cumulative survival at 1, 3, hepatic resection or orthotopic liver transplantation (OLT).Forty- 5, and 10 years was 97.6%, 72.3%, 66.2%, and 47.4%.Tumorone (8.9%) of the 477 patients had FL-HCC.Four were treated at free survival at these times was 80.3%, 49.4%, 33%, and the University of Colorado (before 1980), and the remaining 37 at the University of Pittsburgh Medical Center.There were 23 males 29.3%.The TNM stage was significantly associated with tuand 18 females.The 41 patients were 29.5 { 13.7 (SD) years old mor-free survival.Patients with positive nodes had a shorter (range, 9-66; median, 25) (Fig. 1).The median follow-up was 58 tumor-free survival than those with negative nodes (P õ .015).{ 9.3 months. Patient survival was most adversely affected by the presence of vascular invasion (P õ .05). FL-HCC is an indolently grow- Clinicopathological Characteristicsing tumor of the liver, which usually was diagnosed in our patients at a stage too advanced for effective surgical treatmentThe pathology reports and operative findings were used to deterof most conventional HCC.Nevertheless, long-term survival mine: the principal tumor size, number of lesions, lobar distribu- frequently was achieved with aggressive surgical treatment.tion, vascular and lymphatic tumor extension, surgical margins, When a subtotal hepatectomy could not be performed, total distant metastases, and the presence or absence of associated cirrho- sis.When available, the tumor markers were recorded as well as hepatectomy (THx) with liver transplantation was a valuable the virus markers (hepatitis C virus, hepatitis B virus). option. (HEPATOLOGY 1997;26:877-883.)Staging was with the pTNM classification proposed by the International Union Against Cancer and the American Joint Committee Fibrolamellar hepatoma (FL-HCC) was first described by on Cancer.18,19 Thirty-seven of the 41 patients had stage IV tumors Edmondson 1 as a rare histological variant of hepatocellular at the time of surgery (Table 1).carcinoma (HCC).Although several reports have emphasized the indolent growth of this subtype as well as a higher sur-Surgical Procedures vival than with surgical treatment of conventional HCC, the better postoperative prognosis has been questioned or de-Partial hepatectomy (PHx) was the procedure of choice.Total hepatectomy (THx) and OLT were performed when tumor exten-nied. 2,3on or the presence of underlying liver disease precluded PHx.Both Resolution of this controversy has been hampered by the PHx and THx were frequently performed under highly unfavorable paucity of long-term follow-up data after surgical therapy for conditions, such as lymph node involvement and/or direct invasion FL-HCC.Exclusive of the 41 patients described herein (some of tumor into the adjacent organs. 4ubtotal Hepatectomy (PHx).In 8 of the 28 patients, resection included one or more surrounding organs in an attempt to secure tumor-free margins.The types of PHx performed in this group are