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Malignes epitheloides Hämangioendotheliom der Leber

Ergebnisse der chirurgischen Behandlung

Malign epithelioid hemangioendothelioma of the liver. Results of surgical treatment strategies

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Zusammenfassung

Hintergrund

Mit dem Begriff malignes epitheloides Hämangioendotheliom wird ein seltener vaskulärer Tumor, der hauptsächlich im Weichgewebe, der Lunge und der Leber beschrieben worden ist, bezeichnet. Da die Verläufe mit und ohne Therapie sehr variabel sind, berichten wir hier unsere Erfahrungen mit der resektiven Therapie und der Lebertransplantation für diesen seltenen Lebertumor.

Material und Methoden

Wir beobachteten 7 Patienten in einem Zeitraum von 5 Jahren mit der histologischen Diagnose eines hepatischen epitheloiden Hämangioendothelioms. Die Leberveränderungen wurden in 5 von 7 Fällen zufällig bemerkt und die Patientenverläufe prospektiv beobachtet. Therapie und Verlauf wurden in Form von Fallberichten dargestellt.

Ergebnisse

Drei Patienten wurden mit einer formalen Leberteilresektion versorgt und je ein Patient erhielt ein Lebendspende-Teillebertransplantat und ein komplettes Lebertransplantat. Ein Patient befindet sich zz. auf der Warteliste zur Transplantation und bei einer Patientin ist der Tumorstatus unter alternativen Heilmethoden zz. stationär. Alle Patienten erholten sich gut vom operativen Eingriff, sind 2 Monate bis 4 Jahre nach der Operation beschwerdefrei und zeigen keinen Anhalt für ein Tumorrezidiv.

Schlussfolgerungen

Die formale Leberteilresektion oder bei diffusem Befall die orthotope Lebertransplantation zeigen gute Ergebnisse beim hepatischen, malignen, epitheloiden Hämangioendotheliom. Daher wird die Resektion als Therapie der Wahl für diese Erkrankung vorgeschlagen.

Abstract

Background

Malignant epithelioid hemangioendothelioma is a rare vascular tumor described mostly in soft tissue, lungs, or liver. The outcome after a wide variety of therapeutic measures, ranging from extended surgical therapy to no therapy, is reported to be variable. Therefore, we reviewed our experience with resective therapy for this rare liver tumor, including orthotopic liver transplantation.

Material and methods

During a period of 5 years, seven patients with the histological diagnosis of hepatic epithelia hemangioendothelioma were seen. In five of them, the liver pathology was detected at random, and all patients were prospectively followed. The therapeutic measures and course of disease are given as case reports.

Results

Three patients received formal liver resection and two received liver grafts, one partial and one whole. One further patient is scheduled for transplantation and one is undergoing alternative therapy, with the tumor remaining stable. All resected patients recovered quickly and are alive and well 2 months to 4 years later without signs of tumor recidivism.

Conclusion

Anatomic liver resection, or in the case of diffuse tumor spread, orthotopic liver transplantation, show favorable long-term results for the treatment of hepatic epithelioid hemangioendothelioma. Therefore, surgical therapy is proposed as the treatment of choice for this entity.

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Literatur

  1. Bancel B, Patricot LM, Caillon P, Ducerf C, Pouyet M (1993) Hepatic epitheloid hemangioendotheloima. One case with liver transplantation . Review of the literature. Ann Pathol 13:23–28

    PubMed  Google Scholar 

  2. Ben-Haim M, Roayaie S, Ye MQ et al. (1999) Hepatic epitheloid hemangioendothelioma. Resection or transplantation, which and when? Liver Transplant Surg 6:526–531

    Article  Google Scholar 

  3. Blumgart LH, Baer HU, Czerniak A, Zimmermann A, Dennison AR (1993) Extended left hepatectomy: technical aspects of an evolving procedure. Br J Surg 80:903–906

    PubMed  Google Scholar 

  4. Bruix J, Sherman M, Llovet JM et al. (2001) Clinical management of hepatocellular carcinoma. J Hepatol 35:421–430

    Article  PubMed  Google Scholar 

  5. Clements D, Hubscher S, West R, Elias E, McMaster P (1986) Epithelioid haemangioendothelioma. A case report. J Hepatol 2(3): 441–449

    Article  PubMed  Google Scholar 

  6. Dail DH, Liebow AA (1975) Am J Pathol 78 [Suppl]: 6–7

  7. Daller JA, Bueno J, Gutierrez J et al. (1999) Hepatic hemangioendothelioma: clinical experience and management strategy. J Pediatr Surg 34(1): 98–105

    Article  PubMed  Google Scholar 

  8. d’Annibale M, Piovanello P, Carlini P et al. (2002) Epithelioid hemangioendothelioma of the liver: Case report and review of the literature. Trans Proc 34:1248–1251

    Article  Google Scholar 

  9. Demetris AJ, Minervini M, Raikow RB, Lee RG (1997) Hepatic epitheloid hemangioendothelioma. Biological question based on pattern of recurrence in an allograft and tumor immunophenotype. Am J Surg Pathol 21:263–270

    Article  PubMed  Google Scholar 

  10. Enzinger FM, Weiss SW (1995) Hemangioendothelioma: vascular tumors of intermediate malignancy. In: Enzinger FM, Weiss SW (Hrsg) Soft tissue tumors. Mosby, St Louis, pp 627–639

  11. Flemming P, Maschek H, Ringe B, Lang H, Georgh A (1995) Das epitheloide Hämangioendotheliom der Leber: Bericht über 5 mit orthotoper Lebertransplantation behandelte Fälle und eine Übersicht der Literatur. Verh Dtsch Ges Path 79:160

    Google Scholar 

  12. Fletcher CDM, Unni KK, Mertens F (eds) (2002) World Health Organization Classification of Tumours. Pathology and genetics of tumours of soft tissue and bond. IARC Press, Lyon, pp 173–175

  13. Harris EJ, Taylor RM, Porter JM (1989) Epitheloid hemangioendothelioma of the external iliac vein: a primary vascular tumor presenting as traumatic venous obstruction. J Vasc Surg 10:693–699

    Article  PubMed  Google Scholar 

  14. Hung C-F, Jeng L-B, Lee W-C, Lin D-Y, Tan PPC, Chen M-F (1998) Liver transplantation for epithelioid hemangioendothelioma. Trans Proc. 30:3307–3309

    Google Scholar 

  15. Ishak KG, Sesterhenn IA, Goodman ZD, Rabin L, Stromeyer FW (1984) Epithelioid hemangioendothelioma of the liver: a clinicopathologic and follow-up study of 32 cases. Hum Pathol 15:839–852

    PubMed  Google Scholar 

  16. Jonas S, Bechstein WO, Steinmüller T et al. (2001) Vascular invasion and histopathologic grading determine outcome after liver transplantation for hepatocellular carcinoma in cirrhosis. Hepatology 33:1080–1086

    Article  PubMed  Google Scholar 

  17. Kelleher MB, Iwatsuki S, Sheahan DG (1989) Epithelioid hemangioendothelioma of liver. Clinicopathological correlation of 10 cases treated by orthotopic liver transplantation. Am J Surg Pathol 13(12): 999–1008

    PubMed  Google Scholar 

  18. Lauffer JM, Zimmermann A, Krahenbuhl L, Triller J, Baer HU (1996) Epithelioid hemangioendothelioma of the liver. A rare hepatic tumor. Cancer 78(11): 2318–2327

    Article  PubMed  Google Scholar 

  19. Lerut JP, Orlando G, Sempoux C et al. (2004) Hepatic haemangioendothelioma in adults: excellent outcome following liver transplantation. Transpl Int 17:202–207

    Article  PubMed  Google Scholar 

  20. Madariaga JR, Marino IR, Karavia DD et al. (1995) Lomg-term results after liver transplantation for primary hepatic epitheloid hemangioendothelioma. Ann Surg Oncol 2:483–487

    PubMed  Google Scholar 

  21. Makhlouf HR, Ishak KG, Goodman ZD (1999) Epithelioid hemangioendothelioma of the liver. Am Cancer Society 85:562–582

    Article  Google Scholar 

  22. Marino IR, Todo S, Tzakis AG et al. (1988) Treatment of hepatic epithelioid hemangioendothelioma with liver transplantation. Cancer 62:2079–2084

    PubMed  Google Scholar 

  23. Mentzel T (1998) Hemangioendotheliomas — evolution of an concept of a heterogeneous group of vascular neoplasms. Verh Dtsch Ges Path 82:99–111

    Google Scholar 

  24. O’Grady JG (2000) Treatment options for other hepatic malignancies. Liver Transpl 6: 23–29

    Google Scholar 

  25. Penn I (1991) Hepatic transplantation for primary and metastastic cancers of the liver. Surgery 110:734–735

    Google Scholar 

  26. Pfitzmann R, Neuhaus P (2004) Fortschritte auf dem Gebiet der Lebertransplantation. Chir Gastroenterol 20:1–8

    Article  Google Scholar 

  27. Ruebner BH, Egglestone JC (1987) What is new in epitheloid hemangioendothelioma of the liver. Pathol Res Pract 182:110–112

    PubMed  Google Scholar 

  28. Scoazec JY, Lamy P, Degott C, Reynes M, Feldmann G, Bismuth H, Benhamou JP (1988) Epithelioid hemangioendothelioma of the liver. Diagnostic features and role of liver transplantation. Gastroenterology 94:1447–1453

    PubMed  Google Scholar 

  29. Selby DM, Stocker JT, Waclawiw MA, Hitchcock L, Ishak KG (1994) Infantile hepatic hemangioendothelioma of the liver. Hepatology 20:39–45

    Article  PubMed  Google Scholar 

  30. Sharif K, English M, Ramani P et al. (2004) Management of hepatic epithelioid haemangio-endothelioma in children: what option? Br J Cancer 90:1498–1501

    Article  PubMed  Google Scholar 

  31. Shen CH, Tsai MH, Chen TC, Liu NJ, Sheen IS (1999) Primary hepatic epithelioid hemangioendothelioma: case report. Changgeng Yi Xue Za Zhi 22:486–491

    PubMed  Google Scholar 

  32. Simpson ND, Ahmed AM, Simpson PW, Parkar JA, Keeffe EB, Ahmed A (2003) Living donor liver transplantation in a patient with hepatic epithelioid hemangioendothelioma. J Clin Gastroenterol 37:349–350

    Article  PubMed  Google Scholar 

  33. St Peter SD, Moss AA, Huettl EA, Leslie KO, Mulligan DC (2003) Chemoembolization followed by orthotopic liver transplant for epithelioid hemangioendotherlioma. Clin Transplant 17:549–553

    Article  PubMed  Google Scholar 

  34. Taege C, Holzhausen H-J, Günter G, Flemming P, Rodeck B, Rath FW (1999) Das maligne epitheloide Hämangioendotheliom der Leber. Pathologe 20:345–350

    Article  PubMed  Google Scholar 

  35. Weiss SW, Enzinger FM (1982) Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer 50:970–981

    PubMed  Google Scholar 

  36. Uchimura K, Nakamuta M, Osoegawa M et al. (2001) Hepatic epithelioid hemangioendothelioma. J Clin Gasteroenterol 32(5): 431–434

    Article  Google Scholar 

  37. Van Beers B, Roche A, Mathieu D et al. (1992) Epitheloid hemangioendothelioma of the liver. MR and CT findings. J Comput Assist Tomogr 16:420–424

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Charité — Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin

    J. M. Langrehr & R. Pfitzmann

  2. Institut für Pathologie, Charité — Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin

    I. Petersen

  3. Klinik für Strahlenheilkunde, Charité — Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin

    E. Lopez-Hänninen

  4. Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Charité — Universitätsmedizin Berlin, Campus Virchow-Klinikum, Augustenburger Platz 1, 13353 , Berlin

    J. M. Langrehr

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  1. J. M. Langrehr
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  2. I. Petersen
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Correspondence to J. M. Langrehr.

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Langrehr, J.M., Petersen, I., Pfitzmann, R. et al. Malignes epitheloides Hämangioendotheliom der Leber. Chirurg 76, 1161–1167 (2005). https://doi.org/10.1007/s00104-005-1070-6

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