Natural History of Epithelioid Hemangioendothelioma of the Liver: CT Findings of 15 Cases

Onishi, Yasuyuki; Kusumoto, Masahiko; Motoi, Noriko; Hiraoka, Nobuyoshi; Sugawara, Shunsuke; Itou, Chihiro; Sone, Miyuki

doi:10.1016/j.acra.2020.04.015

Cited by 12 publications

(18 citation statements)

References 11 publications

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“…Currently, no standard therapy has been established for HEH. Although, spontaneously tumor regression of HEH was reported, we found that the tumor progressed slowly for most patients (3,4,17). Surgical resection has been reported with good long-term results (1,18).…”

Section: Discussionmentioning

confidence: 64%

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Sirolimus combined with interferon-alpha 2b therapy for giant hepatic epithelioid hemangioendothelioma: a case report

Zhou¹,

Si²,

Liu³

et al. 2022

Front. Oncol.

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Hepatic epithelioid hemangioendothelioma (HEH) is a very rare tumor originated from vascular endothelial cells, with unpredictable malignancy. No standard treatment has been established yet. Although surgical resection and liver transplantation have been reported to be effective treatments with favorable long-term outcomes, the multiple intrahepatic lesions or extrahepatic metastasis makes these procedures unsuitable to most patients. Sirolimus was reported to be an effective drug for epithelioid hemangioendothelioma but only about 10% achieved partial response. Interferon-alpha 2b (IFN-a 2b) has also been used for the treatment of HEH, and the rate of tumor regression was more than 50%. Here, we report a HEH patient with giant intrahepatic tumor (>15cm), who achieved partial response after the combined therapy of sirolimus and IFN-a 2b. The giant intrahepatic lesion (>15 cm) regressed obviously after 8 months treatment and no severe adverse event was reported. The good response and safety of combined therapy with sirolimus and IFN-a 2b provide a promising guidance for future clinical study.

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Section: Discussionmentioning

confidence: 64%

“…The rarity of the disease limits the implementation of clinical trials and no standard treatment paradigm has been established yet. Surgical resection has been retrospectively studied to be effective for HEH ( 3 ). However, according to our previous study, recurrence was very common after surgical resection ( 4 ).…”

Section: Introductionmentioning

confidence: 99%

Sirolimus combined with interferon-alpha 2b therapy for giant hepatic epithelioid hemangioendothelioma: a case report

Zhou¹,

Si²,

Liu³

et al. 2022

Front. Oncol.

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“…The clinical prognosis of HEH is variable and unpredictable, ranging from an aggressive and fulminant course to possible long-term survival without definitive therapy [ 31 ]. The usual treatment for HEH includes liver resection, transcatheter arterial chemoembolisation, chemotherapy, radiation therapy and liver transplantation.…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics and CT features of hepatic epithelioid haemangioendothelioma and comparison with those of liver metastases

Wang

Liang

et al. 2022

Insights Imaging

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Background To analyse clinical characteristics and computer tomography (CT) findings of hepatic epithelioid haemangioendothelioma (HEH) and to determine differential features compared with liver metastasis (LM). Methods This retrospective study included 80 patients with histopathologically confirmed HEH (n = 20) and LM (n = 60) of different primary tumours who underwent dynamic contrast-enhanced CT scans. CT findings included the location, contour, size, number, margin, and density of lesions, the patterns and degree of contrast enhancement of lesions, vascular invasion and changes in other organs. The enhancement ratio (ER) and tumour-to-normal parenchyma ratio (TNR) were calculated. Receiver operating characteristic curves (ROCs) were used to determine areas under the curve (AUCs). Results About 65% of HEH lesions were located in submarginal areas. Significant differences were observed between HEH and LM patients in age, sex, and tumour marker positivity (p < 0.05). HEH showed minimal to slight enhancement, thin ring-like enhancement in arterial phase, and slight, homogeneous, progressive enhancement in the portal phase. HEH presented capsule retraction, and the “target” sign and the “lollipop” sign were significantly more frequent than in LM (p < 0.05). The ER and TNR in the arterial phase of HEH were lower than those of LM (p < 0.05). AUCs of ER and TNR in the arterial phase were 0.74 and 0.73, respectively. Conclusion Lesions in subcapsular locations, capsular retraction, slight and thin ring-like enhancement, “target” and “lollipop” signs and lower ER and TNR in the arterial phase may represent important features of HEH compared with LM.

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“…It has been observed that hepatic EHE can remain stable for years or even spontaneously reduce. A computed tomography (CT) evaluation of 15 patients with hepatic EHE found that six patients (40%) had reduction and five patients (33%) had no change in the growth of the largest tumor during a median follow-up of 36 months [11]. For this reason, it is controversial to consider patients with hepatic EHE as candidates for liver transplants.…”

Section: Surgical Abstentionmentioning

confidence: 99%

Surgery in Soft Tissue Sarcoma: the Thin Line between a Surgical or more Conservative Approach

Gronchi

2021

Future Oncol.

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Surgery is the primary treatment for localized, clinically resectable soft tissue sarcoma (STS). Chemotherapy and radiotherapy, administered in the pre- or post-operative settings, have important ancillary roles in the multimodal management of primary STS. Some sarcoma centers also employ locoregional therapies such as isolated limb perfusion and deep wave hyperthermia in multimodal therapy. In advanced or metastatic STS, surgery is recommended when complete resection is feasible. Nevertheless, in certain situations there is thin line between a surgical or non-surgical approach, generally related to STS histological type/subtype, disease stage and technical considerations. In advanced STS, factors favoring surgery are isolated oligometastatic disease, long disease-free interval, suitable histology, response to chemotherapy and high probability of a complete resection.

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Natural History of Epithelioid Hemangioendothelioma of the Liver: CT Findings of 15 Cases

Cited by 12 publications

References 11 publications

Sirolimus combined with interferon-alpha 2b therapy for giant hepatic epithelioid hemangioendothelioma: a case report

Sirolimus combined with interferon-alpha 2b therapy for giant hepatic epithelioid hemangioendothelioma: a case report

Clinical characteristics and CT features of hepatic epithelioid haemangioendothelioma and comparison with those of liver metastases

Surgery in Soft Tissue Sarcoma: the Thin Line between a Surgical or more Conservative Approach

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