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Clinical characteristics of Japanese patients with epithelioid hemangioendothelioma: a multicenter retrospective study
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Clinical characteristics of Japanese patients with epithelioid hemangioendothelioma: a multicenter retrospective study
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Clinical characteristics of Japanese patients with epithelioid hemangioendothelioma: a multicenter retrospective study
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Journal Article
Clinical characteristics of Japanese patients with epithelioid hemangioendothelioma: a multicenter retrospective study
2018
Overview
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.
Publisher
BioMed Central,BioMed Central Ltd,Springer Nature B.V,BMC
Subject
/ Adult
/ Aged
/ Biomedical and Life Sciences
/ Epithelioid hemangioendothelioma
/ Female
/ Genes
/ Health Promotion and Disease Prevention
/ Hemangioendothelioma, Epithelioid - diagnosis
/ Hemangioendothelioma, Epithelioid - mortality
/ Humans
/ Liver
/ Male
/ Medical and radiation oncology
/ Neoplasms, Vascular Tissue - diagnosis
/ Neoplasms, Vascular Tissue - mortality
/ Oncology
/ Pain
/ Patients
/ Sarcoma
/ Soft Tissue Neoplasms - diagnosis
/ Soft Tissue Neoplasms - mortality
/ Studies
/ Survival
/ Women
