Clinical Patterns and Outcome in Epithelioid Hemangioendothelioma With or Without Pulmonary Involvement

Lau, Kenneth; Massad, Malek G.; Pollak, Cynthia; Rubin, Charles M.; Yeh, Joannie T.; Wang, Jing; Edelman, Guy; Yeh, Jenny; Prasad, Sunil; Weinberg, Guy

doi:10.1378/chest.11-0039

Cited by 213 publications

(237 citation statements)

References 23 publications

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“…In a survey of EHE patients from an EHE support group, overall survival was 73% at 5 years. 3 EHE of soft tissue was first described by Weiss and Enzinger. 4 As its clinical behavior is intermediate between a benign hemangioma and a high-grade angiosarcoma, the term 'hemangioendothelioma' was suggested.…”

Section: Epithelioid Hemangioendotheliomamentioning

confidence: 99%

“…3 It has long been debated if this occurrence is truly multifocal or represents an unusual pattern of loco-regional metastasis. This is particularly relevant in visceral EHE, where the multifocal disease is limited to one organ (ie, liver, Figure 1 Conventional epithelioid hemangioendothelioma, pathological findings: (a) gross appearance of an epithelioid hemangioendothelioma (EHE) of the superior vena cava, tumor being entirely confined to the vein wall; (b) van Gieson elastic stain highlighting the elastic structure of a vessel wall showing complete obliteration with tumor; (c) angiocentric growth involving a small vessel wall in a soft tissue EHE; microscopic appearance showing cords (d) or solid sheets (e) of epithelioid cells with moderate amount of pale eosinophilic cytoplasm; (f) the presence of intracytoplasmic lumens (blisters cells) is a common feature, as is the myxochodnroid quality of the extracellular stroma; occasionally the matrix may more myxoid (g) or hyalinized (h) in appearance.…”

Section: Geneticsmentioning

confidence: 99%

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Malignant vascular tumors—an update

2014

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Section: Epithelioid Hemangioendotheliomamentioning

confidence: 99%

Section: Geneticsmentioning

confidence: 99%

Malignant vascular tumors—an update

2014

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“…La distribución según género es de 1:4 mayor en mujeres [5][6][7] . Su etiología está aún en estudio, se asocia a moduladores de la angiogénesis y a la Proteína de Quimiotaxis de Monocitos 1, que serían necesarios para la proliferación tumoral, a su vez, Budousquie et al describieron gran variedad de alteraciones clonales destacando translocación 7 y 22, translocaciones robertsonianas del cromosoma 14 y la pérdida del cromosoma Y [8][9][10] .…”

Section: Discussionunclassified

Hemangioendotelioma epitelioide pulmonar: Reporte de un caso

et al. 2017

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“…As the most common presentations are liver alone (21%), liver plus lung (18%), lung alone (12%) and bone alone (14%), the available literature focuses above all on these three directions [10]. The general issue seems to be that, when bone or pulmonary lesions are small and limited in number, surgical, curative resection (that is amputation, en-bloc resection, wedge resection) achieves good outcomes [11,12].…”

Section: Introductionmentioning

confidence: 99%

Pulmonary Epithelioid Hemangioendothelioma: Advances in Treatment Options despite a Rare Vascular Tumor

Sardaro¹,

Petruzzelli²,

Portaluri³

2015

Chemotherapy (Los Angel)

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IntroductionWe have already discussed about dail and liebow describing pulmonary epithelioid hemangioendothelioma (PEH) as an aggressive bronchoalveolar cell carcinoma with a remarkable propensity to invade adjacent blood vessels and small airways, the following Weiss and Enzinger description of epithelioid hemangioendothelioma (EHE) as a vascular bone and soft tissues tumor showing intermediate malignancy between hemangioma and angiosarcoma, and the final confirmation from Weldon-Linne of a factor-VIII-related antigen onto malignant cells [1]. The recent World Health Organization (WHO 2002) classification has described EHE as lesions that fall into the category of locally aggressive tumors with metastatic potential [2,3]. The etiology of EHE is still a dilemma, as several clonal abnormalities in tumor cells, and different angiogenic stimulators may act as promoters of endothelial cell proliferation [4][5][6][7][8]. A new etiopathogenetic hypothesis suggests a causal relationship between chronic Bartonella infection and the development of this rare vascular tumor: if this was confirmed, it would be plausible that eradicating the bacterial infection or interrupting Bartonella-induced angiogenic and proliferative cell signals could slow the tumor progression and improve patient outcomes [9].Due to its rarity (EHE represents less than 1% of all vascular tumors), because of EHE clinical presentation is as heterogeneous as its clinical localization can be, there is no standard for treatment and few therapeutic options are available. As the most common presentations are liver alone (21%), liver plus lung (18%), lung alone (12%) and bone alone (14%), the available literature focuses above all on these three directions [10]. The general issue seems to be that, when bone or pulmonary lesions are small and limited in number, surgical, curative resection (that is amputation, en-bloc resection, wedge resection) achieves good outcomes [11,12]. Locally advanced hepatic EHE seems to benefit from transplantation, with good results [13]. However, a complete surgical resection is not usually feasible: Pinet et al. reported a case of an aggressive form of pleural EHE resulting in complete remission after treatment with carboplatin plus etoposide [14], as well as bilateral multicentric PEH seems to respond well to interferon 2α, with some partial spontaneous regression [7]. Nonetheless, when a pathological fracture occurs, a great alternative to plate-and-screws surgical stabilization is radiofrequency ablation: by creating small, carefully controlled, thermal injuries of bone, the extent of resection can be reduced [15]. On the other side, with the aim of controlling residual desease given the recurrence of EHE, radiation therapy (RT) is chosen for localized EHE, while chemotherapy is preferred for widespread disease. In any case, the beneficial effect of both is still not confirmed [14,[16][17][18][19]. Given the vascular origin of this tumor, the use of antiangiogenetic molecules like thalidomide and lenalidomide reasonably migh...

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Clinical Patterns and Outcome in Epithelioid Hemangioendothelioma With or Without Pulmonary Involvement

Cited by 213 publications

References 23 publications

Malignant vascular tumors—an update

Malignant vascular tumors—an update

Hemangioendotelioma epitelioide pulmonar: Reporte de un caso

Pulmonary Epithelioid Hemangioendothelioma: Advances in Treatment Options despite a Rare Vascular Tumor

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