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      Case Report: A Malignant Liver and Thoracic Solitary Fibrous Tumor: A 10-Year Journey From the Brain to the Liver and the Spine

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          Solitary fibrous tumors are rare neoplasms that originate from mesenchymal tissues and have been found to occur in any site, including the spine and liver. Although most of solitary fibrous tumors have benign features, only 10–20% are malignant and prone to metastasis. No previous reports have described the malignant and metastatic Solitary fibrous tumor arising in both of the liver and thoracic vertebrae. In this article, we present the case of a 60-year-old woman who underwent gross total resection of a meningeal tumor in 2007. She presented 10 years later with a thoracic vertebral mass that caused relentless pain and a lesion in the right lobe of liver. She underwent marginal excision of the T3 tumor with T2-4 pedicular screw fixation in March 2017, then right hemi-hepatectomy was performed to remove the liver lesion in June 2017. Both of the lesions were confirmed to be a metastatic and malignant tumor after surgery. The literature lacks randomized controlled trials and large studies that define the natural history of malignant solitary fibrous tumors and recommendations of precise management plan for the disease. However, the best choice for treatment is gross total resection, which probably provide the optimal treatment to achieve long-term disease-free survival.

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          Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing.

          A 44-year old woman with recurrent solitary fibrous tumor (SFT)/hemangiopericytoma was enrolled in a clinical sequencing program including whole-exome and transcriptome sequencing. A gene fusion of the transcriptional repressor NAB2 with the transcriptional activator STAT6 was detected. Transcriptome sequencing of 27 additional SFTs identified the presence of a NAB2-STAT6 gene fusion in all tumors. Using RT-PCR and sequencing, we detected this fusion in all 51 SFTs, indicating high levels of recurrence. Expression of NAB2-STAT6 fusion proteins was confirmed in SFT, and the predicted fusion products harbor the early growth response (EGR)-binding domain of NAB2 fused to the activation domain of STAT6. Overexpression of the NAB2-STAT6 gene fusion induced proliferation in cultured cells and activated the expression of EGR-responsive genes. These studies establish NAB2-STAT6 as the defining driver mutation of SFT and provide an example of how neoplasia can be initiated by converting a transcriptional repressor of mitogenic pathways into a transcriptional activator.
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            Atypical and malignant solitary fibrous tumors in extrathoracic locations: evidence of their comparability to intra-thoracic tumors.

            Solitary fibrous tumor (SFT), first described as a pleural lesion, has been reported at numerous extrathoracic sites over the past 10 years. About 10% to 15% of intrathoracic SFTs are histologically or clinically malignant, but such cases have very rarely been described at other locations. Among 92 cases of extrathoracic SFT in our files, we identified 10 that either had recurred (2 cases) or had a least one atypical histologic feature (8 cases). The ten tumors occurred in five men and five women, 32 to 81 years old (median 56), measured 1.9 cm to 20 cm (median 11.5 cm), and were located in the abdomen/pelvis (4 cases), retroperitoneum (3 cases), groin, trunk, and upper arm. Nuclear atypia (8 cases), markedly increased cellularity (6 cases), areas of necrosis (4 cases), and greater than 4 mitoses/10 HPFs (3 cases) were seen in addition to the typical histologic features of SFT. Six tumors had at least two of these atypical histologic features. Nine cases were positive for CD34, six were positive for O-13, and one was focally positive for smooth muscle actin. Eight were excised completely. Subsequent follow-up revealed tumor relapse in eight cases (follow up 6-180 months, median 24). Four patients had local recurrence at 12 to 168 months. Distant metastasis developed at 1 to 6 years in five cases with spread to lung (2 cases), liver (4 cases), and bone. Metastasis or local recurrence developed within 2 years in five patients. To date, no patient has died of their tumor. These findings demonstrate that nuclear atypia, hypercellularity, greater than 4 mitoses/10 HPFs, and necrosis may be seen in up to 10% of extrathoracic SFTs, and are associated with, but are not by themselves predictive of, aggressive clinical behavior. In addition, our findings confirm that the behavior of extrathoracic SFTs is unpredictable, entirely comparable to that of their better known pleural counterparts, and confirm that patients with SFTs in all locations require careful, long-term follow up. It is probably unwise to regard any such lesion as definitely benign.
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              Meningeal hemangiopericytoma and solitary fibrous tumors carry the NAB2-STAT6 fusion and can be diagnosed by nuclear expression of STAT6 protein.

              Non-central nervous system hemangiopericytoma (HPC) and solitary fibrous tumor (SFT) are considered by pathologists as two variants of a single tumor entity now subsumed under the entity SFT. Recent detection of frequent NAB2-STAT6 fusions in both, HPC and SFT, provided additional support for this view. On the other hand, current neuropathological practice still distinguishes between HPC and SFT. The present study set out to identify genes involved in the formation of meningeal HPC. We performed exome sequencing and detected the NAB2-STAT6 fusion in DNA of 8/10 meningeal HPC thereby providing evidence of close relationship of these tumors with peripheral SFT. Due to the considerable effort required for exome sequencing, we sought to explore surrogate markers for the NAB2-STAT6 fusion protein. We adopted the Duolink proximity ligation assay and demonstrated the presence of NAB2-STAT6 fusion protein in 17/17 HPC and the absence in 15/15 meningiomas. More practical, presence of the NAB2-STAT6 fusion protein resulted in a strong nuclear signal in STAT6 immunohistochemistry. The nuclear reallocation of STAT6 was detected in 35/37 meningeal HPC and 25/25 meningeal SFT but not in 87 meningiomas representing the most important differential diagnosis. Tissues not harboring the NAB2-STAT6 fusion protein presented with nuclear expression of NAB2 and cytoplasmic expression of STAT6 proteins. In conclusion, we provide strong evidence for meningeal HPC and SFT to constitute variants of a single entity which is defined by NAB2-STAT6 fusion. In addition, we demonstrate that this fusion can be rapidly detected by STAT6 immunohistochemistry which shows a consistent nuclear reallocation. This immunohistochemical assay may prove valuable for the differentiation of HPC and SFT from other mesenchymal neoplasms.

                Author and article information

                Front Surg
                Front Surg
                Front. Surg.
                Frontiers in Surgery
                Frontiers Media S.A.
                03 December 2020
                : 7
                Department of Orthopedics, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine , Shanghai, China
                Author notes

                Edited by: Christopher William Seder, Rush University Medical Center, United States

                Reviewed by: Giovanni Grignani, Institute for Cancer Research and Treatment (IRCC), Italy; Fabrizio Minervini, University of Lucerne, Switzerland

                *Correspondence: Dianwen Song dianwen.song@ 123456shgh.cn

                This article was submitted to Thoracic Surgery, a section of the journal Frontiers in Surgery

                †These authors have contributed equally to this work and share first authorship

                Copyright © 2020 Mao, Zhou, Huang, Yan, Hu, Yin, Zhao and Song.

                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.

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                Figures: 4, Tables: 0, Equations: 0, References: 20, Pages: 6, Words: 3001
                Case Report


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