TY - JOUR
T1 - Clinical characteristics of Japanese patients with epithelioid hemangioendothelioma
T2 - A multicenter retrospective study 11 Medical and Health Sciences 1103 Clinical Sciences
AU - Shiba, Satoshi
AU - Imaoka, Hiroshi
AU - Shioji, Kazuhiko
AU - Suzuki, Eiichiro
AU - Horiguchi, Shigeru
AU - Terashima, Takeshi
AU - Kojima, Yasushi
AU - Okuno, Tatsuya
AU - Sukawa, Yasutaka
AU - Tsuji, Kunihiko
AU - Umemoto, Kumiko
AU - Asagi, Akinori
AU - Todaka, Akiko
AU - Ueno, Makoto
AU - Ikeda, Masafumi
AU - Morizane, Chigusa
AU - Furuse, Junji
N1 - Funding Information:
This study was supported by a National Cancer Center Research and Development Fund (26-A-4), which plays a role of scientific research on multi-institutional trials to establish new standard treatment of solid tumors in adults, from the Ministry of Health, Labour, and Welfare of Japan.
Publisher Copyright:
© 2018 The Author(s).
PY - 2018/10/19
Y1 - 2018/10/19
N2 - 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.
AB - 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.
KW - Chemotherapy
KW - Epithelioid hemangioendothelioma
KW - Ki-67 index
KW - Prognosis
KW - Sarcoma
KW - Tumor size
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U2 - 10.1186/s12885-018-4934-0
DO - 10.1186/s12885-018-4934-0
M3 - Article
C2 - 30340559
AN - SCOPUS:85055077259
VL - 18
JO - BMC Cancer
JF - BMC Cancer
SN - 1471-2407
IS - 1
M1 - 993
ER -