Primary malignant hepatic epithelioid hemangioendothelioma: A comprehensive review of the literature with emphasis on the surgical therapy

Publikation: Beitrag in FachzeitschriftÜbersichtsartikel (Review)BeigetragenBegutachtung

Beitragende

  • Arianeb Mehrabi - , Universität Heidelberg (Autor:in)
  • Arash Kashfi - , Universität Heidelberg (Autor:in)
  • Hamidreza Fonouni - , Universität Heidelberg (Autor:in)
  • Peter Schemmer - , Universität Heidelberg (Autor:in)
  • Bruno M. Schmied - , Universität Heidelberg (Autor:in)
  • Peter Hallscheidt - , Universität Heidelberg (Autor:in)
  • Peter Schirmacher - , Universität Heidelberg (Autor:in)
  • Jurgen Weitz - , Universität Heidelberg (Autor:in)
  • Helmut Friess - , Universität Heidelberg (Autor:in)
  • Markus W. Buchler - , Universität Heidelberg (Autor:in)
  • Jan Schmidt - , Universität Heidelberg (Autor:in)

Abstract

Malignant hepatic epithelioid hemangioendothelioma (HEH) is a rare malignant tumor of vascular origin with unknown etiology and a variable natural course. The authors present a comprehensive review of the literature on HEH with a focus on clinical outcome after different therapeutic strategies. All published series on patients with HEH (n = 434 patients) were analyzed from the first description in 1984 to the current literature. The reviewed parameters included demographic data, clinical manifestations, therapeutic modalities, and clinical outcome. The mean age of patients with HEH was 41.7 years, and the male-to-female ratio was 2:3. The most common clinical manifestations were right upper quadrant pain, hepatomegaly, and weight loss. Most patients presented with multifocal tumor that involved both lobes of the liver. Lung, peritoneum, lymph nodes, and bone were the most common sites of extrahepatic involvement at the time of diagnosis. The most common management has been liver transplantation (LTx) (44.8% of patients), followed by no treatment (24.8% of patients), chemotherapy or radiotherapy (21% of patients), and liver resection (LRx) (9.4% of patients). The 1-year and 5-year patient survival rates were 96% and 54.5%, respectively, after LTx; 39.3% and 4.5%, respectively, after no treatment, 73.3% and 30%, respectively, after chemotherapy or radiotherapy; and 100% and 75%, respectively, after LRx. LRx has been the treatment of choice in patients with resectable HEH. However, LTx has been proposed as the treatment of choice because of the hepatic multicentricity of HEH. In addition, LTx is an acceptable option for patients who have HEH with extrahepatic manifestation. Highly selected patients may be able to undergo living-donor LTx, preserving the donor pool. The role of different adjuvant therapies for patients with HEH remains to be determined.

Details

OriginalspracheEnglisch
Seiten (von - bis)2108-2121
Seitenumfang14
FachzeitschriftCancer
Jahrgang107
Ausgabenummer9
PublikationsstatusVeröffentlicht - 1 Nov. 2006
Peer-Review-StatusJa
Extern publiziertJa

Externe IDs

PubMed 17019735

Schlagworte

Ziele für nachhaltige Entwicklung

ASJC Scopus Sachgebiete

Schlagwörter

  • Hepatic epithelioid hemangioendothelioma, Liver resection, Liver transplantation, Treatment