On-line version ISSN 2078-5151
Print version ISSN 0038-2361
S. Afr. j. surg. vol.47 n.4 Cape Town Nov. 2009
Feriyl BhaijeeI; Michael L. LocketzII; Jake E. J. KrigeIII
IM.B. CH.B; Surgical Gastroenterology Unit, Groote Schuur Hospital, Cape Town
IIM.B. CH.B., F.C.PATH. (S.A.) ANAT; Department of Pathology, Faculty of Health Sciences, University of Cape Town
IIIM.B. CH.B., F.R.C.S. (EDIN.), F.A.C.S., F.C.S. (S.A.); Department of Surgery and Surgical Gastroenterology Unit, Faculty of Health Sciences, University of Cape Town
BACKGROUND: Fibrolamellär carcinoma (FLC) is an uncommon malignant tumour of hepatocyte origin that differs from hepatocellular carcinoma (HCC) in aetiology, demographics, condition of the affected liver, and tumour markers. Controversy exists whether FLC demonstrates a more favourable prognosis than typical HCC. A review of existing literature reveals a dearth of FLC data from the African continent.
METHODS: We utilised the prospective liver resection database at Groote Schuur Hospital to identify all patients who underwent surgery for FLC between 1990 and 2008.
RESULTS: Seven patients (median age 21 years, range 19 - 42, 5 men, 2 women) underwent surgery for FLC. No patient had underlying liver disease or an elevated alpha feto-protein (AFP) at either initial presentation or recurrence. Six patients had a solitary tumour at diagnosis (mean largest diameter = 12cm), and underwent left hepatectomy (N=2), right hepatectomy (N=1), extended right hepatectomy (N=1), and segmentectomies (N=2). Three patients underwent a portal lymphadenectomy for regional lymphatic tumour involvement. One patient with advanced extrahepatic portal nodal metastasis was unresectable. No peri-operative deaths occurred. Recurrence occurred post resection in all 6 patients. Median overall survival was 60 months, and overall 5-year survival was 4 out of 7 (57%). Post-resection survival (N=6) was 61 months, with a 5-year survival rate of 4 out of 6 (67%). The patient with unresectable disease survived 38 months after tumour embolisation with Lipiodol.
CONCLUSION: Our series suggests that despite (i) a high resection rate of solitary lesions with clear tumour resection margins, and (ii) absence of underlying liver disease, FLC has a high recurrence rate with an ultimately poor clinical outcome. These findings concur with recent international experience of FLC.
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1. MacSween RNM, Burt AD, Portmann BC, Ishak KG, Scheuer PJ, Anthony PP. Pathology of the Liver, 4th ed. Toronto: Churchill Livingstone, 2002. [ Links ]
2. McLarney JK, Rucker PT, Bender GN, Goodman ZD, Kashitani N, Ros PR. Fibrolamellar carcinoma of the liver: Radiologic-pathologic correlation. Radiographics 1999; 19: 453-471. [ Links ]
3. Berman MM, Libbey NP, Foster JH. Hepatocellular carcinoma: polygonal cell type with fibrous stroma- an atypical variant with a favorable prognosis. Cancer 1980; 46: 1448-1455. [ Links ]
4. Braunwald E, Fauci AS, Kasper DL, Hauser SL, Longo DL, Jameson JL. Harrison's Principles of Internal Medicine, 15th ed. Vol 1. NewYork: McGraw-Hill, 2001. [ Links ]
5. Ichikawa T, Federle MP, Grazioli L, Marsh W. Fibrolamellar hepatocellular carcinoma: Pre- and posttherapy evaluation with CT and MR imaging. Radiology 2000; 217: 145-151. [ Links ]
6. Stevens WR, Johnson CD, Stephens DH, Nagorney DM. Fibrolamellar hepatocellular carcinoma: stage at presentation and results of aggressive surgical management. Am J Roentgenol 1995; 164: 1153-1158. [ Links ]
7. Torbenson M. Review of the clinicopathologic features of fibrolamellar carcinoma. Adv Anat Pathol 2007; 14: 217-223. [ Links ]
8. Moreno-Luna LE, Arrieta O, Garcia-Leiva J, et al. Clinical and pathological factors associated with survival in young adult patients with fibrolamellar carcinoma. BMC Cancer 2005; 5: 142-149. [ Links ]
9. Couinaud C. Anatomical principles of left and right regulated hepatectomy: technics. J Chir (Paris) 1954; 70: 933-966. [ Links ]
10. El-Serag HB, Davila JA. Is fibrolamellar carcinoma different from hepato-cellular carcinoma? A US population-based study. Hepatology 2004; 39(3): 798-803. [ Links ]
11. Berman MA, Burnham JA, Sheahan DG. Fibrolamellar carcinoma of the liver: an immunohistochemical study of 19 cases and a review of literature. Hum Pathol 1988; 19: 784-792. [ Links ]
12. Friedman AC, Lichtenstein JE, Goodman ZD, Fishman EK, Siegelman SS, Dachman AH. Fibrolamellar hepatocellular carcinoma. Radiology 1985; 157: 583-857. [ Links ]
13. Katzenstein HM, Krailo MD, Malogolowkin MH, et al. Fibrolamellar hepatocellular carcinoma in children and adolescents. Cancer 2003; 97: 2006-2012. [ Links ]
14. Stipa F, Yoon SS, Liau KH, et al. Outcome of patients with fibrolamellar hepatocellular carcinoma. Cancer 2006; 106: 1331-1338. [ Links ]
15. Kakar S, Burgart LJ, Batts KP, et al. Clinicopathological features and survival in fibrolamellar carcinoma: comparison with conventional hepatocellular carcinoma with and without cirrhosis. Mod Pathol 2005; 18: 1417-1423. [ Links ]
16. Maniaci V, Davidson BR, Rolles K, et al. Fibrolamellar hepatocellular carcinoma - prolonged survival with multimodal therapy. Eur J Surg Oncol 2009 Jan 12; doi:10.1016/j.ejso.2008.12.009. [ Links ]
17. Pinna AD, Iwatsuki S, Lee RG. Treatment of fibrolamellar hepatoma with subtotal hepatectomy or transplantation. Hepatology 1997; 26: 877-883. [ Links ]