Clinical characteristics of Japanese patients with epithelioid hemangioendothelioma: A multicenter retrospective study 11 Medical and Health Sciences 1103 Clinical Sciences

Satoshi Shiba, Hiroshi Imaoka, Kazuhiko Shioji, Eiichiro Suzuki, Shigeru Horiguchi, Takeshi Terashima, Yasushi Kojima, Tatsuya Okuno, Yasutaka Sukawa, Kunihiko Tsuji, Kumiko Umemoto, Akinori Asagi, Akiko Todaka, Makoto Ueno, Masafumi Ikeda, Chigusa Morizane, Junji Furuse

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7 Citations (Scopus)

Abstract

Background: Epithelioid hemangioendothelioma is an exceedingly rare sarcoma often occurring as an indolent angiocentric vascular tumor at various anatomic sites. Few reports have evaluated large case series of epithelioid hemangioendothelioma. Methods: We conducted a retrospective analysis of the clinical data of 42 consecutive patients with epithelioid hemangioendothelioma who were pathologically diagnosed between 1990 and 2014 at 13 Japanese tertiary hospitals. We analyzed their clinical characteristics, tumor features and prognostic factors. Results: The study included 22 men and 20 women, with a median age of 54 (range, 18-78) years. Pain was the most common symptom, occurring in 15 (68%) of the 22 symptomatic patients. The median maximum tumor diameter was 4.0 (range, 1.0-12.8) cm. The most commonly involved organs were the liver (81%), lungs (57%), and bones (12%). The overall survival rates were 79.5% at 1 year and 72.0% at 5 years. Substantially better survival was observed in asymptomatic patients than in symptomatic patients (P = 0.03), and better survival was also ovserved in patients with Ki-67 index ≤10% than in those with Ki-67 index > 10% (P = 0.04). By multivariate analysis, tumor size > 3.0 cm was associated with decreased survival (P = 0.049, hazard ratio 13.33). Conclusions: This study showed the clinical characteristics of Japanese patients with epithelioid hemangioendothelioma. Tumor size > 3.0 cm is an independent indicator of a poor prognosis in epithelioid hemangioendothelioma. The presence of symptoms at the time of diagnosis and high Ki-67 index implied poor survival.

Original languageEnglish
Article number993
JournalBMC cancer
Volume18
Issue number1
DOIs
Publication statusPublished - Oct 19 2018

Keywords

  • Chemotherapy
  • Epithelioid hemangioendothelioma
  • Ki-67 index
  • Prognosis
  • Sarcoma
  • Tumor size

ASJC Scopus subject areas

  • Genetics
  • Oncology
  • Cancer Research

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    Shiba, S., Imaoka, H., Shioji, K., Suzuki, E., Horiguchi, S., Terashima, T., Kojima, Y., Okuno, T., Sukawa, Y., Tsuji, K., Umemoto, K., Asagi, A., Todaka, A., Ueno, M., Ikeda, M., Morizane, C., & Furuse, J. (2018). Clinical characteristics of Japanese patients with epithelioid hemangioendothelioma: A multicenter retrospective study 11 Medical and Health Sciences 1103 Clinical Sciences. BMC cancer, 18(1), [993]. https://doi.org/10.1186/s12885-018-4934-0