TY - JOUR
T1 - Prognostic Factors in Epithelioid Hemangioendothelioma
T2 - Analysis of a Nationwide Molecularly/Immunohistochemically Confirmed Cohort of 57 Cases
AU - Tomassen, Tess
AU - Versleijen-Jonkers, Yvonne M.H.
AU - Hillebrandt-Roeffen, Melissa H.S.
AU - Van Cleef, Patricia H.J.
AU - van Dalen, Thijs
AU - Weidema, Marije E.
AU - Desar, Ingrid M.E.
AU - Flucke, Uta
AU - van Gorp, Joost M.
N1 - Publisher Copyright:
© 2023 by the authors.
PY - 2023/6/23
Y1 - 2023/6/23
N2 - Epithelioid hemangioendothelioma (EHE) is an extremely rare vascular sarcoma with variable aggressive clinical behavior. In this retrospective study, we aimed to investigate prognostic factors based on clinicopathologic findings in a molecularly/immunohistochemically confirmed nationwide multicenter cohort of 57 EHE cases. Patients had unifocal disease (n = 29), multifocal disease (n = 5), lymph node metastasis (n = 8) and/or distant metastasis (n = 15) at the time of diagnosis. The overall survival rate was 71.4% at 1 year and 50.7% at 5 years. Survival did not correlate with sex, age or histopathological parameters. No survival differences were observed between multifocal and metastatic disease, suggesting that multifocality represents early metastases and treatment options are limited in comparison to unifocal disease. In unifocal tumors, survival could be predicted using the risk stratification model of Shibayama et al., dividing the cases into low- (n = 4), intermediate- (n = 15) and high- (n = 3) risk groups. No clinical or histopathological parameters were associated with progressive unifocal disease course. Lymph node metastases at the time of diagnosis occurred in 14.0% of the cases and were mainly associated with tumor localization in the head and neck area, proposing lymph node dissection. In conclusion, our results demonstrate the aggressive behavior of EHE, emphasize the prognostic value of a previously described risk stratification model and may provide new insights regarding tumor focality, therapeutic strategies and prognosis.
AB - Epithelioid hemangioendothelioma (EHE) is an extremely rare vascular sarcoma with variable aggressive clinical behavior. In this retrospective study, we aimed to investigate prognostic factors based on clinicopathologic findings in a molecularly/immunohistochemically confirmed nationwide multicenter cohort of 57 EHE cases. Patients had unifocal disease (n = 29), multifocal disease (n = 5), lymph node metastasis (n = 8) and/or distant metastasis (n = 15) at the time of diagnosis. The overall survival rate was 71.4% at 1 year and 50.7% at 5 years. Survival did not correlate with sex, age or histopathological parameters. No survival differences were observed between multifocal and metastatic disease, suggesting that multifocality represents early metastases and treatment options are limited in comparison to unifocal disease. In unifocal tumors, survival could be predicted using the risk stratification model of Shibayama et al., dividing the cases into low- (n = 4), intermediate- (n = 15) and high- (n = 3) risk groups. No clinical or histopathological parameters were associated with progressive unifocal disease course. Lymph node metastases at the time of diagnosis occurred in 14.0% of the cases and were mainly associated with tumor localization in the head and neck area, proposing lymph node dissection. In conclusion, our results demonstrate the aggressive behavior of EHE, emphasize the prognostic value of a previously described risk stratification model and may provide new insights regarding tumor focality, therapeutic strategies and prognosis.
KW - aggressive
KW - epithelioid hemangioendothelioma
KW - lymph node
KW - malignant
KW - prognosis
KW - risk stratification
KW - survival
UR - http://www.scopus.com/inward/record.url?scp=85164769923&partnerID=8YFLogxK
U2 - 10.3390/cancers15133304
DO - 10.3390/cancers15133304
M3 - Article
C2 - 37444414
AN - SCOPUS:85164769923
SN - 2072-6694
VL - 15
SP - 1
EP - 13
JO - Cancers
JF - Cancers
IS - 13
M1 - 3304
ER -