Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts

Abstract

Epithelioid hemangioendothelioma (EHE) is an ultra-rare, translocated, vascular sarcoma. EHE clinical behavior is variable, ranging from that of a low-grade malignancy to that of a high-grade sarcoma and it is marked by a high propensity for systemic involvement. No active systemic agents are currently approved specifically for EHE, which is typically refractory to the antitumor drugs used in sarcomas. The degree of uncertainty in selecting the most appropriate therapy for EHE patients and the lack of guidelines on the clinical management of the disease make the adoption of new treatments inconsistent across the world, resulting in suboptimal outcomes for many EHE patients. To address the shortcoming, a global consensus meeting was organized in December 2020 under the umbrella of the European Society for Medical Oncology (ESMO) involving >80 experts from several disciplines from Europe, North America and Asia, together with a patient representative from the EHE Group, a global, disease-specific patient advocacy group, and Sarcoma Patient EuroNet (SPAEN). The meeting was aimed at defining, by consensus, evidence-based best practices for the optimal approach to primary and metastatic EHE. The consensus achieved during that meeting is the subject of the present publication.

Malignant Vascular Tumors of the Head and Neck—Which Type of Therapy Works Best?

“…Approximately 25% of EHE show immunoreactivity to cytokeratins, and 45% react to smooth muscle actin. Some studies have proposed the use of podoplanin as an immunohistochemical marker in order to differentiate EHE from nonvascular tumors [ 69 ].…”

“…Systemic treatment should only be performed in symptomatic patients with metastatic disease [ 69 ]. In asymptomatic patients with advanced metastatic EHE without the possibility of resection with acceptable morbidity, active surveillance is the preferred approach [ 69 ].…”

“…Systemic treatment should only be performed in symptomatic patients with metastatic disease [ 69 ]. In asymptomatic patients with advanced metastatic EHE without the possibility of resection with acceptable morbidity, active surveillance is the preferred approach [ 69 ]. In patients with localized, resectable EHE, there is no evidence for the effectiveness of systemic neoadjuvant or adjuvant therapy.…”

“…In case reports and small case series, cytotoxic chemotherapy has been explored in EHE patients. The regimens included carboplatin, paclitaxel, adriamycin, dacarbazine, and ifosfamide [ 39 , 69 , 101 ]. In all, these studies scarcely include patients with primary EHE in the head and neck region.…”

Massive Calcified Epithelioid Hemangioendothelioma With Multifocal Involvement: An Imaging Diagnosis Dilemma and a Rare Case Report

“…Surgical resection can achieve good outcomes for patients with a limited number of lesions and no metastases. Chemotherapy or immunotherapy can be considered in patents with asymptomatic metastatic disease (21,22). Some studies have proven that radiotherapy can help patients with exclusive bone presentations achieve local pain control and better quality of life when combined with bone surgery (23,24).…”