Outcome of patients with fibrolamellar hepatocellular carcinoma

Abstract

BACKGROUNDFibrolamellar hepatocellular carcinoma (FL‐HCC) is a rare variant of hepatocellular carcinoma, has distinct pathologic features, and typically occurs in young patients without underlying hepatitis or cirrhosis.METHODSForty‐one patients with the pathologic diagnosis of FL‐HCC evaluated at our institution between 1986 and 2003 were identified from a prospective database.RESULTSMedian age of all patients was 27 years. None of these patients had underlying hepatitis or cirrhosis, and only 3 (7%) patients had an α‐fetoprotein level > 200 ng/mL. Twenty‐eight patients with primary disease underwent complete gross resection, and 13 patients were unresectable. In patients treated with resection, median tumor size was 9 cm (range, 3–17), 9 (36%) had vascular invasion, and 14 (50%) had lymph node metastases. There were no perioperative deaths. With a median follow‐up of 34 months, 5‐year overall survival for resected patients was 76%. However, 5‐year recurrence‐free survival was only 18%, and of the 9 resected patients with more than 5 years of follow‐up, 7 had recurrences. Lymph node metastasis was the only significant negative prognostic factor. Seventeen (61%) patients underwent a second operation for recurrent disease. Median survival for unresected patients with FL‐HCC was only 12 months, and no patient survived beyond 5 years.CONCLUSIONSFL‐HCC occurs in a distinctly different population of patients than common HCC, and patients with FL‐HCC generally fare better after complete resection. These tumors have a relatively indolent tumor biology, and late recurrences are common. Repeat resections for recurrence should be considered given the lack of other effective treatment options. Cancer 2006. © 2006 American Cancer Society.

mTORC1 and FGFR1 signaling in fibrolamellar hepatocellular carcinoma

“…However, this latter finding is controversial. [18][19][20] A recent study of 95 patients from the Fibrolamellar Carcinoma Consortium reported a median survival of 6.7 years, 2 and based on data from SEER 18 registries from 2000 to 2010 the overall 5-year survival was significantly better among fibrolamellar carcinoma patients (34%) compared with other patients with hepatocellular carcinoma (16%). 20 However, in patients who have received a potentially curative form of treatment (resection, transplantation, or radiofrequency ablation), there was no difference in survival between the two groups.…”

Place of liver transplantation in the treatment of hepatocellular carcinoma in the normal liver

“…Separate Note on FL-HCC [43][44][45][46][47][48][49][50] Because the analysis of the literature shows that NC-HCC and FL-HCC are still perceived differently on account of their different clinical presentations, courses, imaging results, and outcomes, some brief remarks about this tumor variant are made here.…”

“…Tumor recurrence can occur late, even after 5 or 10 years, and repeat hepatectomy (reported in 20%-60% of patients) can significantly prolong survival. 45,48 Vascular involvement and lymph node involvement are significant prognostic factors, but the tumor number, the tumor size, and the use of chemotherapy are not. 48 The somewhat better results reported after hepatectomy versus LT can be explained by the more advanced tumor stages and immunosuppression in liver recipients, as shown in the transplant series from Hannover (8 patients), 48 Birmingham, UK (9 patients), 44 and Pittsburgh (13 patients).…”

“…43 The 5-year OS rate of the partial hepatectomy series from the Memorial Sloan-Kettering Cancer Center (New York, NY), which included 28 patients, reached 56%. 48 There is some indication that adjuvant chemotherapy (a combination of 5-fluorouracil and interferon) may be useful and more applicable in these patients because they present with normal residual liver tissue. 49,50 In conclusion, at the request of the consensus conference committee, we have examined the reported results of partial liver resection and transplantation in NC-HCC patients.…”

Carcinoma hepatocelular variedad fibrolamelar metastásico en menores de 20 años: Reporte de 2 casos tratados con intención curativa y revisión de la literatura

“…Se presenta fundamentalmente en adolescentes y adultos jóvenes como una masa tumoral hepática única, frecuentemente de gran tamaño, no asociada a cirrosis y rodeada de una cápsula fibrosa. Su diseminación es preferentemente a través de metástasis linfáticas regionales 2,3 . En nuestro país sólo se han comunicado casos aislados 4 .…”