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Long-term results after liver transplantation for primary hepatic epithelioid hemangioendothelioma

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Abstract

Background: Hepatic epithelioid hemangioendothelioma (PHEHE) is a multifocal, low-grade malignant neoplasia characterized by its epithelial-like appearance and vascular endothelial histogenesis. The outcome of 16 patients treated with orthotopic liver transplantation (OLT) is the subject of this report.

Methods: A retrospective study of 16 patients with HEHE (7 men, 9 women) with ages ranging from 24 to 58 years (mean 37 ± 10.6 years). Follow-up intervals ranged from 1 to 15 years (median of 4.5 years).

Results: Actual patient survival at 1, 3, and 5 years was 100, 87.5, and 71.3%, respectively. Disease-free survival at 1, 3, and 5 years was 81.3, 68.8, and 60.2%, respectively. The 90-day operative mortality was 0. Involvement of the hilar lymph nodes or vascular invasion did not affect survival. The 5-year survival of HEHE compares favorably with that of hepatocellular carcinoma at the same stage (stage 4A): 71.3 versus 9.8% (p=0.001)

Conclusions: The long-term survival obtained in this series justifies OLT for these tumors even in the presence of limited extrahepatic disease.

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References

  1. Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma.Cancer 1982;50:970–81.

    CAS  PubMed  Google Scholar 

  2. Ishak KG, Sesterhen IA, Goodman MZD, Rabin L, Stromeyer FW. Epithelioid hemangioendothelioma of the liver: a clinicopathologic and follow-up study of 32 cases.Hum Pathol 1984;15:839–52.

    CAS  PubMed  Google Scholar 

  3. American Joint Committee on Cancer.Manual for staging of cancer, 3rd ed. Philadelphia: JB Lippincott, 1987:87–92.

    Google Scholar 

  4. Hermanek P, Sobin LH, eds.TNM classification of malignant tumors, 4th ed. Berlin: Springer-Verlag, 1987:53–5.

    Google Scholar 

  5. Starzl TE, Iwatsuki S, Esquivel CO, et al. Refinements in the surgical technique of liver transplantation.Semin Liver Dis 1985;5:349–56.

    CAS  PubMed  Google Scholar 

  6. Marino IR, Todo S, Tzakis AG, Klintmalm G, Kelleher M, Iwatsuki S, Starzl TE, et al. Treatment of hepatic hemangioendothelioma with liver transplantation.Cancer 1988;2079–84.

  7. Koneru B, Cassavilla A, Bowman J, Iwatsuki S, Starzl TE. Liver transplantation for malignant tumors.Gastroenerol Clin North Am 1988;17:177–93.

    CAS  Google Scholar 

  8. Iwatsuki S, Starzl TE, Sheahan DG, Yokoyama I, Demetris AJ, Todo S, Tzakis AG, et al. Hepatic resection versus transplantation for hepatocellular carcinoma.Ann Surg 1991;214:221–9.

    CAS  PubMed  Google Scholar 

  9. Weiss SW, Ishak KG, Dail DH, Enzinger FM. Epithelioid hemangioendothelioma—a vascular tumor often mistaken for a carcinoma.Cancer 1982;50:970–81.

    CAS  PubMed  Google Scholar 

  10. Dean PJ, Haggitt C, O'Hara CJ. Malignant epitheliohemangioendothelioma of the liver in young women: relationship to oral contraceptive use.Am J Surg Pathol 1985;695–704.

  11. Kelleher MB, Iwatsuki S, Sheahan DG. Epithelioid hemangioendothelioma of liver.Am J Surg Pathol 1989;13:999–1008.

    CAS  PubMed  Google Scholar 

  12. Gordon RD, Fung J, Tzakis AG, Todo S, Stieber A, Bronsther O, Martin M, et al. Liver transplantation at the University of Pittsburgh 1984–1990. In:Clinical transplants 1991. Los Angeles: UCLA Tissue Typing Laboratory, 1991:105–88.

    Google Scholar 

  13. Scoazec JY, Lamy P, Degott C, Reynes M, Feldmann G, Bismuth H, Benhamon GP. Epithelioid hemangioendothelioma of the liver: diagnostic features and role of liver transplantation.Gastroenterology 1988;94:1447–53.

    CAS  PubMed  Google Scholar 

  14. O'Grady JG, Polson RJ, Rollo SK, Calne RY, Williams R. Liver transplantation for malignant disease: results in 93 consecutive patients.Ann Surg 1988;207:373–9.

    PubMed  Google Scholar 

  15. Tereda T, Hoso M, Kono N, Watanabe K, Nakanuma Y. Epithelioid hemangioendothelioma of the liver in primary biliary cirrhosis: a case report.Acta Pathol Jpn 1989;39:607–11.

    Google Scholar 

  16. Dean PJ, Haggitt RC, O'Hara CJ. Malignant epithelioid hemangioendothelioma of the liver in young women: relationship to oral contraceptive use.Am J Surg Pathol 1985;9:695–704.

    CAS  PubMed  Google Scholar 

  17. Holley MP, Cuschieeri A. Epithelioid hemangioendothelioma of the liver: objective response to hepatic intraarterial 5-FU.Eur J Surg Oncol 1989;15:73–8.

    CAS  PubMed  Google Scholar 

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Madariaga, J.R., Marino, I.R., Karavias, D.D. et al. Long-term results after liver transplantation for primary hepatic epithelioid hemangioendothelioma. Annals of Surgical Oncology 2, 483–487 (1995). https://doi.org/10.1007/BF02307080

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  • DOI: https://doi.org/10.1007/BF02307080

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