The Role of Local Therapy in Multi-focal Epithelioid Haemangioendothelioma

Tong, Daniel; Constantinidou, Anastasia; Engelmann, Bodil Elisabeth; Chamberlain, Florence; Thway, Khin; Fisher, Cyril; Hayes, Andrew; Fotiadis, Nicos; Messiou, Christina; Miah, Aisha; Zaidi, Shane; Benson, Charlotte; Graaf, Winette van der; Jones, Robin L.

doi:10.21873/anticanres.13675

Cited by 16 publications

(11 citation statements)

References 13 publications

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“…Resection should be carried out in referral centers with experience in sarcoma surgery. 53 , 54 , 55 , 56 …”

Section: Treatmentmentioning

confidence: 99%

“…On the contrary, active surveillance is the initial recommended option in cases presenting with asymptomatic locoregional or systemic metastases 53 [V, A].…”

Section: Treatmentmentioning

confidence: 99%

“… 58 Although active surveillance has never been formally studied, it is common practice in experienced centers to keep asymptomatic patients on active surveillance before engaging in treatment, including surgery. 2 , 5 , 9 , 53 …”

Section: Treatmentmentioning

confidence: 99%

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

et al. 2021

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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.

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“…Resection should be carried out in referral centers with experience in sarcoma surgery. 53 , 54 , 55 , 56 …”

Section: Treatmentmentioning

confidence: 99%

“…On the contrary, active surveillance is the initial recommended option in cases presenting with asymptomatic locoregional or systemic metastases 53 [V, A].…”

Section: Treatmentmentioning

confidence: 99%

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

et al. 2021

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“…Given the unpredictability of EHE evolution, surveillance is often offered upfront in patients with naturally stable or asymptomatic disease, reserving medical treatment to symptomatic or progressive cases. [14][15][16] However, the definition of radiological progression in this disease is an issue. The possible occurrence of SE and the limited increase in size observed over a short period in slow-growing variants might not meet promptly the Response Assessment Criteria in Solid Tumor (RECIST) definition of progression, making its use often unsatisfactory in the identification of those patients who might benefit from an active treatment.…”

Section: Introductionmentioning

confidence: 99%

Clinical prognostic factors in advanced epithelioid haemangioendothelioma: a retrospective case series analysis within the Italian Rare Cancers Network

et al. 2021

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Background: This multicentric, retrospective study conducted within the Italian Rare Cancer Network describes clinical features and explores their possible prognostic relevance in patients with advanced epithelioid haemangioendothelioma (EHE) started on surveillance. Patients and methods: We collected data on adult patients with molecularly confirmed, advanced EHE consecutively referred at five sarcoma reference centres between January 2010 and June 2018, with no evidence of progressive disease (PD) and started on surveillance. Overall survival (OS) and progression-free survival (PFS) univariable and multivariable Cox analyses were performed. In the latter, due to the low number of cases and events, penalized likelihood was applied, and variable selection was performed using a random forest model. Results: Sixty-seven patients were included. With a median follow-up of 50.2 months, 51 (76%) patients developed PD and 16 (24%) remained stable. PD at treatment start did not meet RECIST version 1.1 in 15/51 (29%) patients. The 3year PFS and OS were 25.4% and 71.1%, respectively, in the whole population. Tumour-related pain (TRP) was the most common baseline symptom (32.8%), followed by temperature (20.9%), fatigue (17.9%), and weight loss (16.4%). Baseline TRP (P ¼ 0.0002), development of TRP during follow-up (P ¼ 0.005), baseline temperature (P ¼ 0.002), and development of fatigue during follow-up (P ¼ 0.007) were associated with a significantly worst PFS. An association between baseline TRP (P < 0.0001), development of TRP during follow-up (P ¼ 0.0009), evidence of baseline serosal effusion (P ¼ 0.121), and OS was recorded. Conclusion: Because of the poor outcome observed in EHE patients presenting with serosal effusion, TRP, temperature, or serosal effusion, upfront treatment in this subgroup could be considered.

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“…Local treatments should be considered for patients who have indolent disease with oligoprogressing sites. 16 There is biologic rationale for antiangiogenic agents with immunohistochemical analysis from a small cohort of patients with pulmonary EHE who demonstrated expression of vascular endothelial growth factor (VEGF), vascular endothelial growth factor receptor 2 (VEGFR2), and VEGFR3. 17 However, in retrospective cohorts, mixed efficacy has been reported with antiangiogenic treatments.…”

mentioning

confidence: 99%