New Molecular Insights, and the Role of Systemic Therapies and Collaboration for Treatment of Epithelioid Hemangioendothelioma (EHE)

Stacchiotti, Silvia; Tap, William D.; Leonard, Hayley C.; Zaffaroni, Nadia; Baldi, Giacomo Giulio

doi:10.1007/s11864-023-01076-1

Cited by 8 publications

(4 citation statements)

References 34 publications

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“…Previously described clinical parameters related to worse outcome such as pulmonary and/or pleura involvement did not predominate within this cohort, suggesting that the aggressiveness is likely due to the tumor biology. It might be that secondary (epi)genetic alterations enhance tumor progression, demonstrated by a lower survival rate within our study [ 28 , 29 ].…”

Section: Discussionmentioning

confidence: 99%

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

Tomassen

Versleijen-Jonkers

Hillebrandt-Roeffen

et al. 2023

Cancers

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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.

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Section: Discussionmentioning

confidence: 99%

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

Tomassen

Versleijen-Jonkers

Hillebrandt-Roeffen

et al. 2023

Cancers

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“…Compared to other malignancies, it has a less aggressive course than angiosarcoma. Metastasis is detected in 27% of cases and can be seen in the liver, lungs, regional lymph nodes, spleen, urinary system, and bones [ 5 ]. The clinical manifestations of the disease are non-specific.…”

Section: Introductionmentioning

confidence: 99%

“…These cells typically spread within small veins, forming groups. Positive dendritic and/or epithelioid cells regarding endothelial cell markers confirm the diagnosis [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

“…There is no established therapeutic algorithm. Although chemotherapy has been administered in some patients, it has been reported that the most durable solution is complete surgical removal of the tumor tissue [ 3 - 5 ]. This article reports the case of a 15-year-old girl with massive hemangioendothelioma at the cervicothoracic junction.…”

Section: Introductionmentioning

confidence: 99%

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Anteroposterior Combined Surgery of a Rare Massive Epithelioid Hemangioendothelioma at the Cervicothoracic Junction

Cine,

Uysal,

Senturk

et al. 2023

Cureus

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Epithelioid hemangioendothelioma is a rare mesenchymal tumor of vascular endothelial origin. Non-soft tissue epithelioid hemangioendothelioma can also be seen in different organs. Although chemotherapy has been used in some patients, complete surgical removal of the tumor tissue has proven to be the most durable solution. A 15-year-old female patient was admitted to our institution with right arm and neck pain. The patient complained of numbness and weakness in the right hand. Computerized tomography indicated an expansile lesion exhibiting osteolytic features located predominantly on the right side of the corpus, pedicle, lamina, and lateral processes of the C7-T1 vertebra. The patient underwent a surgical procedure involving the application of a bilateral C4-5-6 lateral mass screw, left C7-T1 pedicle screw, and bilateral T2-3 pedicle screw and fusion. The complete residual neoplasm was surgically removed during the procedure. Due to the rarity of epithelioid hemangioendothelioma, the existing literature on this topic is confined to case reports, supplemented by a small number of retrospective descriptive case series that aimed to improve our understanding of the clinical, pathological, and molecular features of the condition, as well as to guide potential treatment strategies.

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Primary vascular tumors of bone: A comprehensive literature review on classification, diagnosis and treatment

Tortorelli,

Bellan,

Chiusole

et al. 2024

Critical Reviews in Oncology/Hematology

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New Molecular Insights, and the Role of Systemic Therapies and Collaboration for Treatment of Epithelioid Hemangioendothelioma (EHE)

Cited by 8 publications

References 34 publications

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

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

Anteroposterior Combined Surgery of a Rare Massive Epithelioid Hemangioendothelioma at the Cervicothoracic Junction

Primary vascular tumors of bone: A comprehensive literature review on classification, diagnosis and treatment

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