Das fibrolammeläre hepatozelluläre Karzinom - ein seltener Tumor mit ungewöhnlicher Wachstumstendenz

Zusammenfassung

Wir berichten über eine 18-jährige Patientin mit einem fibrolamellären Leberzellkarzinom (FLC), welches eine ungewöhnliche Wachstumstendenz zeigt. Vom linken Leberlappen ausgehend, wächst der Tumor nach kaudal in die Bauchhöhle bis auf Nabelhöhe. Die Läsion konnte im Rahmen einer atypischen Leberteilresektion mit einem Klammernahtgerät im Gesunden abgesetzt werden. Es wird eine Übersicht der Literatur zu Prognose und Therapie des FLC gegeben und anhand des vorliegenden Falles kritisch diskutiert.

Abstract

We report on a case of fibrolamellar carcinoma of the liver (FLC) of an 18-year-old female patient, which shows an uncommon growing pattern. Originated from the left liver lobe, it grows into the abdominal cavity descending to the umbilicus. The lesion could be removed in sano by atypical liver resection with a linear stapler. We give a review about literature concerning prognosis and therapy of FLC discussing it by means of our actual case.

Schlüsselwörter

fibrolamelläres hepatozelluläres Karzinom - Prognose und Therapie

Key words

fibrolamellar hepatocellular carcinoma - prognosis and therapy

Literatur

• 1 Edmondsen H A. Differential diagnosis of tumors and tumor-like lesions of the liver in infancy and childhood.  Arch Dis Child. 1956;  1 168-186
• 2 Craig J R, Peters R L, Edmondson H A, Omata M. Fibrolamellar carcinoma of the liver: A tumor of adolescents and young adults with distinctive clinico-pathologic features.  Cancer. 1980;  46 372-379
• 3 Berman M M, Libbey N P, Foster J H. Hepatocellular carcinoma: Polygonal cell type with fibrous stroma: An atypical variant with a favourable prognosis.  Cancer. 1980;  46 1448-1455
• 4 Soreide O, Czerniak A, Bradpiece H, Bloom S, Blumgart L. Characteristics of fibrolamellar hepatocellular carcinoma. A study of nine cases and a rewiev of the literature.  AM J Surg. 1986;  151 518-523
• 5 Collier N A, Weinbren K, Bloom S R, Lee Y C, Hodgson H J, Blumgart L H. Neurotensin secretion by fibrolamellar carcinoma of the liver.  Lancet. 1984;  1 538-540
• 6 Paradinas F J, Melia W M, Wilkinson M L. et al . High serum vitamin B12 binding capacity as a marker of the fibrolamellar variant of hepatocellular carcinoma.  BMJ. 1982;  285 840-842
• 7 Kanai T, Takabayashi T, Kawano Y, Kuramochi S, Miyazawa N. A case of postoperative recurrence of fibrolamellar hepatovellular carcinoma with increased vitamin B12 binding capacity in a young japanese female.  Japanese Journal of Clinical Oncology. 2004;  34 346-351
• 8 El-Serag H B, Davila J A. Is fibrolamellar carcinoma different from hepatocellular carcinoma? A US population-based study.  Hepatology. 2004;  39 798-803
• 9 Kakar S, Burgart L J, Batts K P, Garcia J, Jain D, Ferrell L. Clinicopathologic features and survival in fibrolamellar carcinoma: Comparison with conventional hepatocellular carcinoma with and without cirrhosis.  Modern Pathology. 2005;  18 1417-1423
• 10 Stipa F, Yoon S S, Liau K H, Fong Y, Jarnagin W R, D'Angelica M, Abou-Alfa G, Blumgart L H, DeMatteo R P. Outcome of patients with fibrolamellar hepatocellular carcinoma.  Cancer. 2006;  106 1331-1338
• 11 Hemming A W, Langer B, Sheiner P, Greig P D, Taylor B R. Aggressive surgical management of fibrolammelar carcinoma.  J Gastrointest Surg. 1997;  1 342-346
• 12 Stevens W R, Johnson C D, Stephens D H, Nagorney D M. Fibrolamellar hepatocellular carcinoma: Stage at presentation and results of aggressive surgical management.  AJR. 1995;  164 1153-1158
• 13 Pinna A D, Iwatsuki S, Lee G R, Todo S, Madarigaga J R, Marsch J W, Casavilla A, Dvorchik I, Fung J J, Starzl E. Treatment of fibrolamellar heparoma with subtotal hepatectomy or transplantation.  Hepatology. 1997;  26 877-833
• 14 Starzl T E, Iwatsuki S, Shaw Jr  B W, Nalesnik M A, Fahri D C, Van Thiel D H. Treatment of fibrolamellar hepatoma with partial or total hepatectomy and transplantation of the liver.  Surg Gynecol Obstet. 1986;  162 145-148
• 15 Zografos G N, Palmer S, Papastratis G, Habib N A. Aggressive surgical management of fibrolamellar carcinoma in puberty.  Eur J Surg Oncol. 1997;  23 570-572
• 16 Titelbaum D S, Burke D R, Meranze S G, Saul S H. Fibrolamellar hepatocellular carcinoma: Pitfalls in non operative diagnosis.  Radiology. 1988;  167 25-30
• 17 Blachar A, Federle M P, Ferris J V, Lacomis J M, Waltz J S, Armfield D R, Chu G, Almusa O, Grazioli L, Balzano E, Li W. Radiologists' performance in the diagnosis of liver tumors with central scars by using specific CT criteria.  Radiology. 2002;  223 532-539
• 18 Friedman A C, Lichtenstein J E, Goodman Z. et al . Fibrolamellar hepatocellular carcinoma.  Radiology. 1985;  157 583-587
• 19 Rieber A, Brambs H J, Lauchart W, Gartner V. Diagnosis of fibrolamellar cancer.  Radiologe. 1994;  34 196-201
• 20 Caseiro-Alves F, Zins M, Mahfouz A E, Rahmouni A, Vilgrain V, Menu Y, Mathieu D. Calcification in focal nodular hyperplasia: A new problem for differentiation from fibrolamellar hepatocellular carcinoma.  Radiology. 1996;  198 889-892
• 21 McLarney J K, Rucker P T, Bender G N, Goodman Z D, Kashitani N, Ros P R. Fibrolammellar carcinoma of the liver: Radiologic-pathologic correlation.  Radiographics. 1999;  19 453-471
• 22 Ringe B, Wittekind C, Weimannn A, Tusch G, Pichelmayer R. Results of hepatic resection and transplantation for fibrolamellar carcinoma.  Surg Gynecol Obstet. 1992;  175 299-305
• 23 El Gazzaz G, Wong W, El Hadary M K. et al . Outcome of liver resection for fibrolamellar hepatocellular carcinoma.  Transpl Int. 2000;  13 406-409
• 24 Hiramatsu K, Okamato K, Uesaka K, Mukaiyama H, Seno T. Surgical management for lymph node recurrence of resected fibrolamellar hepatocellular carcinoma: A case report.  Hepato-Gastroenerology. 1999;  46 1160-1163
• 25 Tomoaki I, Federle M, Grazioli L, Marsh W. Fibrolamellar Hepatocellular Carcinoma: Pre- and Posttherapie Evaluation with CT and MR Imaging.  Radiology. 2000;  217 145-151
• 26 O'Grady G, Polson R J, Rolles K, Calne R Y, Williams R. Liver Transplantation for malignant disease. Results in 93 consecutive patients.  Ann Surg. 1988;  207 373-379