Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Multicenter Study
. 2018 Oct 19;18(1):993.
doi: 10.1186/s12885-018-4934-0.

Clinical characteristics of Japanese patients with epithelioid hemangioendothelioma: a multicenter retrospective study

Satoshi Shiba  1 , Hiroshi Imaoka  2 , Kazuhiko Shioji  3 , Eiichiro Suzuki  4 , Shigeru Horiguchi  5 , Takeshi Terashima  6 , Yasushi Kojima  7 , Tatsuya Okuno  8 , Yasutaka Sukawa  9 , Kunihiko Tsuji  10 , Kumiko Umemoto  11 , Akinori Asagi  12 , Akiko Todaka  13 , Makoto Ueno  14 , Masafumi Ikeda  11 , Chigusa Morizane  15 , Junji Furuse  16
Affiliations
Multicenter Study

Clinical characteristics of Japanese patients with epithelioid hemangioendothelioma: a multicenter retrospective study

Satoshi Shiba et al. BMC Cancer. .

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.

Keywords: Chemotherapy; Epithelioid hemangioendothelioma; Ki-67 index; Prognosis; Sarcoma; Tumor size.

PubMed Disclaimer

Conflict of interest statement

Ethics approval and consent to participate

This study was approved by the Institutional Review Board of National Cancer Center (reference number, 2014–233). Approval for the review of hospital records was obtained from the Institutional Review Board of National Cancer Center and the patients’ informed consent was waived given the retrospective nature of the study.

Competing interests

The authors declare that they have no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Overall survival of the 42 patients with EHEs
Fig. 2
Fig. 2
Survival curve according to the presence of symptoms at the time of diagnosis
Fig. 3
Fig. 3
Survival curve according to the Ki-67 labeling index
See this image and copyright information in PMC

References

    1. Lau K, Massad M, Pollak C, et al. Clinical patterns and outcome in epithelioid hemangioendothelioma with or without pulmonary involvement: insights from an internet registry in the study of a rare cancer. Chest. 2011;140(5):1312–1318. doi: 10.1378/chest.11-0039. - DOI - PubMed
    1. Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer. 1982;50(5):970–981. doi: 10.1002/1097-0142(19820901)50:5<970::AID-CNCR2820500527>3.0.CO;2-Z. - DOI - PubMed
    1. Weiss SW, Ishak KG, Dail DH, et al. Epithelioid hemangioendothelioma and related lesions. Semin Diagn Pathol. 1986;3(4):259–287. - PubMed
    1. Fletcher CDM, Bridge JA, Hogendoorn P, Mertens F. WHO classification of tumors of soft tissue and bone. Lyon: IARC Press; 2013.
    1. Rosenberg A, Agulnik M. Epithelioid hemangioendothelioma: update on diagnosis and treatment. Curr Treat Options in Oncol. 2018;19(4):19. doi: 10.1007/s11864-018-0536-y. - DOI - PubMed

Publication types