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      Epithelioid hemangioendothelioma of the retroperitoneal giant type treated with Toripalimab: A case report

      case-report

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          Abstract

          Epithelioid hemangioendotheliomas (EHEs), low-grade malignant tumors of vascular endothelial cell origin, are characterized by vascular endothelial proliferation. In 2002, the World Health Organization classified EHEs as locally aggressive tumors with the potential to metastasize. Currently, the diagnosis of EHE is based on pathology, histological and immunohistochemical examinations. There are no standard treatment guidelines. We here report a 69-year-old man who presented with left-sided chest and abdominal pain for more than 2 months. Enhanced computed tomography of the thorax and abdomen in another hospital suggested a mass in the left adrenal region that was considered malignant. Positron emission tomography- computed tomography in our hospital suggested a large multi-loculated, hypermetabolic, cystic mass in the left adrenal region that was considered malignant. Accordingly, a puncture biopsy of the mass was performed and the diagnosis of EHE confirmed by pathological examination, including immunohistochemical staining. This patient was treated with the programmed death 1 (PD-1) immune checkpoint inhibitor toripalimab with long-term success. The best response was stable disease (SD) with a progression-free survival (PFS) of more than 13 months. The patient is still alive now. Because the sample size of previous studies was small, further studies are needed to determine the safety and efficacy of toripalimab in the treatment of EHE.

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          Most cited references18

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          Application of PD-1 Blockade in Cancer Immunotherapy

          The programmed cell death protein 1 (PD-1) pathway has received considerable attention due to its role in eliciting the immune checkpoint response of T cells, resulting in tumor cells capable of evading immune surveillance and being highly refractory to conventional chemotherapy. Application of anti-PD-1/PD-L1 antibodies as checkpoint inhibitors is rapidly becoming a promising therapeutic approach in treating tumors, and some of them have successfully been commercialized in the past few years. However, not all patients show complete responses and adverse events have been noted, suggesting a better understanding of PD-1 pathway mediated immunosuppression is needed to predict patient response and improve treatment efficacy. Here, we review the progresses on the studies of the mechanistic role of PD-1 pathway in the tumor immune evasion, recent clinical development and commercialization of PD-1 pathway inhibitors, the toxicities associated with PD-1 blockade observed in clinical trials as well as how to improve therapeutic efficacy and safety of cancer immunotherapy.
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            PD-1/PD-L1 pathway: Basic biology and role in cancer immunotherapy

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              Novel YAP1-TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma.

              Conventional epithelioid hemangioendotheliomas (EHE) have a distinctive morphologic appearance and are characterized by a recurrent t(1;3) translocation, resulting in a WWTR1-CAMTA1 fusion gene. We have recently encountered a fusion-negative subset characterized by a somewhat different morphology, including focally well-formed vasoformative features, which was further investigated for recurrent genetic abnormalities. Based on a case showing strong transcription factor E3 (TFE3) immunoreactivity, fluorescence in situ hybridization (FISH) analysis for TFE3 gene rearrangement was applied to the index case as well as to nine additional cases, selected through negative WWTR1-CAMTA1 screening. A control group, including 18 epithelioid hemangiomas, nine pseudomyogenic HE, and three epithelioid angiosarcomas, was also tested. TFE3 gene rearrangement was identified in 10 patients, with equal gender distribution and a mean age of 30 years old. The lesions were located in somatic soft tissue in six cases, lung in three and one in bone. One case with available frozen tissue was tested by RNA sequencing and FusionSeq data analysis to detect novel fusions. A YAP1-TFE3 fusion was thus detected, which was further validated by FISH and reverse transcription polymerase chain reaction (RT-PCR). YAP1 gene rearrangements were then confirmed in seven of the remaining nine TFE3-rearranged EHEs by FISH. No TFE3 structural abnormalities were detected in any of the controls. The TFE3-rearranged EHEs showed similar morphologic features with at least focally, well-formed vascular channels, in addition to a variably solid architecture. All tumors expressed endothelial markers, as well as strong nuclear TFE3. In summary, we are reporting a novel subset of EHE occurring in young adults, showing a distinct phenotype and YAP1-TFE3 fusions. Copyright © 2013 Wiley Periodicals, Inc.
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                Author and article information

                Contributors
                Journal
                Front Immunol
                Front Immunol
                Front. Immunol.
                Frontiers in Immunology
                Frontiers Media S.A.
                1664-3224
                17 March 2023
                2023
                : 14
                : 1116944
                Affiliations
                [1] 1 Department of Oncology, Hebei General Hospital , Shijiazhuang, Hebei, China
                [2] 2 Department of Medicine, Hebei North University , Zhangjiakou, Hebei, China
                [3] 3 Department of Medical Affairs, Shanghai Junshi Biosciences Co., Ltd. , Shanghai, China
                Author notes

                Edited by: Yutian Zou, Sun Yat-sen University Cancer Center (SYSUCC), China

                Reviewed by: Xing Zhang, State Key Laboratory of Translational Medicine and Innovative Drug Development, China; Xue Bai, Beijing Cancer Hospital, Peking University, China

                *Correspondence: Hongzhen Zhang, hongzhenzhang456@ 123456sohu.com

                This article was submitted to Cancer Immunity and Immunotherapy, a section of the journal Frontiers in Immunology

                Article
                10.3389/fimmu.2023.1116944
                10063782
                37006308
                4091ebaf-1153-4276-87de-cf68f3b2d2b8
                Copyright © 2023 Bu, Peng, Liu, He, Wang, Li, Niu and Zhang

                This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

                History
                : 06 December 2022
                : 27 February 2023
                Page count
                Figures: 3, Tables: 1, Equations: 0, References: 18, Pages: 5, Words: 1632
                Funding
                This work was supported by grants from the Administration of Traditional Chinese Medicine of Hebei Province, China (2019155) and the Medical Science Research Project of Hebei Provincial Health Commission, China (20200782).
                Categories
                Immunology
                Case Report

                Immunology
                epithelioid hemangioendothelioma,retroperitoneal tumor,immunotherapy,toripalimab,pd-1 inhibitor

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