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

Abstract

BackgroundEpithelioid 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.MethodsWe 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.ResultsThe 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).ConclusionsThis 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.

Clinical Characteristic and Prognosis of Epithelioid Hemangioendothelioma: 35 Cases from Single Center in Recent Decade

“…Although there was no statistical difference in the p value, there was a tendency for symptomatic patients to be worse than asymptomatic patients. This was similar with the study by Satoshi Shiba [14], however, the author found the difference to be signi cant. We suggest that larger samples of EHE are needed to analyze the relationship between the main complaint and the overall survival rate.…”

Natural history of epithelioid hemangioendothelioma that progressed over 20 years

“…A Japanese study on EHE by Shiba et al. revealed that tumor size > 3.0 cm and a high Ki‐67 index implied poor survival 2 . In light of these criteria, our case (tumor size: 0.3–1.2 cm and a Ki‐67 index of about 15%) should have had a better prognosis.…”

“…EHE often involves the liver (81%), lungs (57%), and bones (12%) 2 . Although there are various chemotherapy regimens that show limited efficacy, 1,3 this patient received none until the terminal stage.…”

“…(PBC 2020 Feb; 67(2): e28045) describing 24 cases of epithelioid hemangioendothelioma (EHE); of 15 evaluable patients, overall survival was 73% at 5 years 1 . EHE is a rare sarcoma of vascular origin and uncertain biological behavior, often occurring as an indolent angiocentric vascular tumor at various anatomic sites 2 . Another study of 15 pediatric cases of EHE in the liver/lungs reported that two patients entered remission and six had a stable disease for 1.2–5.3 years without therapy.…”

Prognostic Determinants Analysis and Nomogram for Bone Malignant Vascular Tumors: A Surveillance, Epidemiology and End Results (SEER) Analysis

“…A retrospective analysis of 46 patients with malignant vascular tumors in the spine suggested that age, surgery, metastasis, and malignant grade were prognostic factors for overall survival [ 20 ]. A multicenter retrospective study of Japanese patients with epithelioid hemangioendothelioma showed that tumor size >3.0 cm is an independent indicator of poor prognosis [ 21 ].…”