Preprint Article Version 1 Preserved in Portico This version is not peer-reviewed

Prognostic Factors in Epithelioid Hemangioendothelioma: Analysis of a Nationwide Molecularly/Immunohistochemically Confirmed Cohort of 57 Cases

Tess Tomassen
* ,
Yvonne M H Versleijen-jonkers
ORCID logo ,
Melissa H S Hillebrandt-roeffen
,
Patricia H J Van Cleef
,
Thijs Van Dalen
,
Marije E Weidema
,
Ingrid M E Desar
,
Uta Flucke
,
Joost M Van Gorp
Version 1 : Received: 5 June 2023 / Approved: 5 June 2023 / Online: 5 June 2023 (14:26:22 CEST)

A peer-reviewed article of this Preprint also exists.

Tomassen, T.; Versleijen-Jonkers, Y.M.H.; Hillebrandt-Roeffen, M.H.S.; Van Cleef, P.H.J.; van Dalen, T.; Weidema, M.E.; Desar, I.M.E.; Flucke, U.; van Gorp, J.M. Prognostic Factors in Epithelioid Hemangioendothelioma: Analysis of a Nationwide Molecularly/Immunohistochemically Confirmed Cohort of 57 Cases. Cancers 2023, 15, 3304. Tomassen, T.; Versleijen-Jonkers, Y.M.H.; Hillebrandt-Roeffen, M.H.S.; Van Cleef, P.H.J.; van Dalen, T.; Weidema, M.E.; Desar, I.M.E.; Flucke, U.; van Gorp, J.M. Prognostic Factors in Epithelioid Hemangioendothelioma: Analysis of a Nationwide Molecularly/Immunohistochemically Confirmed Cohort of 57 Cases. Cancers 2023, 15, 3304.

Abstract

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 molecular/immunohistochemical confirmed nationwide multicenter cohort of 57 EHE. 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 69.6% at 1 year and 50.5% at 5 years. Survival did not correlate with sex, age or histo-pathological 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. Larger tumor size (>3cm) and higher mitotic count (>1/10HPF) was asso-ciated with progressive unifocal disease course. Lymph node metastases at time of diagnosis oc-curred in 14.0% of the cases and was 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 previous described risk stratification model and may provide new insights regarding tumor focality, therapeutic strategies and prognosis.

Keywords

Epithelioid hemangioendothelioma; Aggressive; Malignant; Prognosis; Risk stratification; Lymph node

Subject

Medicine and Pharmacology, Oncology and Oncogenics

Copyright: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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