Elsevier

Journal of Surgical Research

Volume 264, August 2021, Pages 481-489
Journal of Surgical Research

Tumor Biology Impacts Survival in Surgically Managed Primary Hepatic Vascular Malignancies

https://doi.org/10.1016/j.jss.2021.02.043Get rights and content

Abstract

Background

Hepatic angiosarcoma (AS) and hepatic epithelioid hemangioendothelioma (HEHE) are rare primary hepatic vascular malignancies (PHVM) that remain poorly understood. To guide management, we sought to identify factors and trends predicting survival after surgical intervention using a national database.

Materials and methods

In a retrospective analysis of the National Cancer Database patients with a diagnosis of PHVM were identified. Clinicopathologic factors were extracted and compared. Overall survival (OS) was estimated and predictors of survival were identified.

Results

Three hundred ninty patients with AS and 216 with HEHE were identified. Only 16% of AS and 36% of HEHE patients underwent surgery. The median OS for patients who underwent surgical intervention was 97 months, with 5-year OS of 30% for AS versus 69% for HEHE patients (P< 0.001). Tumor biology strongly impacted OS, with AS histology (Hazard Ratio [HR] of 3.61 [1.55-8.42]), moderate/poor tumor differentiation (HR = 3.86 [1.03-14.46]) and tumor size (HR = 1.01 [1.00-1.01]) conferring worse prognosis. The presence of metastatic disease in the surgically managed cohort (HR = 5.22 [2.01-13.57]) and involved surgical margins (HR = 3.87 [1.59-9.42]), were independently associated with worse survival.

Conclusions

In this national cohort of PHVM, tumor biology, in the form of angiosarcoma histology, tumor differentiation and tumor size, was strongly associated with worse survival after surgery. Additionally, residual tumor burden after resection, in the form of positive surgical margins or the presence of metastasis, was also negatively associated with survival. Long-term clinical outcomes remain poor for patients with the above high-risk features, emphasizing the need to develop effective forms of adjuvant systemic therapies for this group of malignancies.

Section snippets

Materials and Methods

After approval of the study protocol by the Institutional Review Board (IRB), we performed a retrospective analysis of the 2004-2013 NCDB liver Participant User File (PUF). The International Classification of Diseases (ICD) for Oncology, third edition histology codes 9120 (angiosarcoma), 9130 (hemangioendothelioma), 9133 (epithelioid hemangioendothelioma) and 9150 (hemangiopericytoma) were combined with the liver site code C22.0 to identify patients with primary hepatic vascular malignancies.

Results

One hunderd thirty-seven thousand fifty-one liver neoplasms were captured in the NCDB between 2004 and 2013, with primary hepatic vascular malignancies accounting for 610 (0.5%) of all liver tumors. Amongst them, angiosarcoma was the most common 390 (64%), followed by hemangioendothelioma 216 (35%), and hemangiopericytoma 4 (<1%). The annual rate of new cases did not differ across the study years, averaging 61 new diagnoses of primary hepatic vascular malignancies per year (Table 1).

Discussion

Primary hepatic vascular malignancies are a diverse group of uncommon neoplasms for which surgery, in the form of liver resection or transplantation, is considered the mainstay of curative-intent treatment.5 However, our knowledge of the clinicopathologic factors impacting long-term prognosis following surgical management is limited, potentially resulting in the offering of surgery to patients who may not derive the most benefit from liver resection. In this national cohort of patients with

Conclusions

In this national cohort of primary hepatic vascular malignancies, we discovered that a minority of patients with localized disease receive surgical treatment, despite evidence that those who receive it had a better prognosis. Amongst the patients who receive surgery, tumor biology, in the form of angiosarcoma histology, tumor differentiation and tumor size was strongly associated with worse survival. Additionally, residual tumor burden after resection, in the form of positive surgical margins

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Disclosure: None.

Acknowledgment: Disclosure of funding: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Authors contributions: Design of the work: Dogeas and Augustine. Acquisition of data: Dogeas, Mokdad, Augustine. Interpretation of data: Dogeas, Mokdad, Porembka, Polanco, Mansour, Choti, Augustine. Drafting the work: Dogeas, Mokdad, Bhattatiry, Augustine. Final approval: Dogeas, Mokdad, Bhattatiry, Porembka, Polanco, Mansour, Choti, Augustine. Agreement to be accountable for all aspects of the work: Dogeas, Mokdad, Bhattatiry, Porembka, Polanco, Mansour, Choti, Augustine.

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