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      Hemangiopericytoma/Solitary Fibrous Tumor of the Parietal Bone: A Case Report

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          Abstract

          Hemangiopericytoma/solitary fibrous tumor (HPC/STF) is a rare tumor arising from Zimmerman's pericytes and it is characterized by an aggressive malignancy, with a high tendency for local and distant recurrence. The authors report the case of a middle-aged woman with HPC/SFT of the right parietal bone, which is an extremely rare primary location of involvement. The patient presented with a painful deformity of insidious growth at the right parietal region. Assessment with cranial computed tomography scan and magnetic resonance imaging revealed an expansive lesion at the right parietal bone, with exocranial extension and 27 mm of maximal diameter. Craniotomy with gross tumor removal, duraplasty, and cranioplasty was performed, and the diagnosis of HPC/SFT, WHO grade III, was established by pathological and immunohistochemical analysis. The patient was then evaluated for adjuvant radiation therapy and received a dose of 60 Gy (2 Gy/fraction) with 3D conformal radiotherapy to the surgical bed. The adjuvant treatment was uneventful and, after 8 months of follow-up, there was no suspected local or distant recurrence. The rarity of this diagnosis, its aggressive behavior, and the lack of published data posed several challenges for the treatment management.

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          HEMANGIOPERICYTOMA: A VASCULAR TUMOR FEATURING ZIMMERMANN'S PERICYTES.

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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.
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              The evolving classification of soft tissue tumours: an update based on the new WHO classification.

              Tumour classifications have become an integral part of modern oncology and, for pathologists, they provide guidelines which facilitate diagnostic and prognostic reproducibility. In many organ systems and most especially over the past decade or so, the World Health Organization (WHO) classifications have become pre-eminent, partly enabled by the timely publication of new "blue books" which now incorporate detailed text and copious illustrations. The new WHO classification of soft tissue tumours was introduced in late 2002 and, because it represents a broad consensus view, it has gained widespread acceptance. This review summarizes the changes, both major and minor, which were introduced and briefly describes the significant number of tumour types which have been first recognized or properly characterized during the past decade. Arguably the four most significant conceptual advances have been: (i) the formal recognition that morphologically benign lesions (such as cutaneous fibrous histiocytoma) may very rarely metastasize; (ii) the general acceptance that most pleomorphic sarcomas can be meaningfully subclassified and that so-called malignant fibrous histiocytoma is not a definable entity, but instead represents a wastebasket of undifferentiated pleomorphic sarcomas, accounting for no more than 5% of adult soft tissue sarcomas; (iii) the acknowledgement that most lesions formerly known as haemangiopericytoma show no evidence of pericytic differentiation and, instead, are fibroblastic in nature and form a morphological continuum with solitary fibrous tumour; and (iv) the increasing appreciation that not only do we not know from which cell type(s) most soft tissue tumours originate (histogenesis) but, for many, we do not recognize their line of differentiation or lineage--hence an increasing number of tumours are placed in the "uncertain differentiation" category.
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                Author and article information

                Journal
                Case Rep Oncol
                Case Rep Oncol
                CRO
                Case Reports in Oncology
                S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.com )
                1662-6575
                Sep-Dec 2022
                10 November 2022
                10 November 2022
                : 15
                : 3
                : 1021-1026
                Affiliations
                [1] aDepartment of Radiotherapy, Portuguese Institute of Oncology of Coimbra, Coimbra, Portugal
                [2] bDepartment of Radiotherapy, Algarve Radiation Oncology Unit − Joaquim Chaves Saúde (JCS), Faro, Portugal
                [3] cHospital of the Divine Holy Spirit, Ponta Delgada, Portugal
                Author notes
                *Ana Rita Dias Neto, anardneto@ 123456gmail.com
                Article
                cro-0015-1021
                10.1159/000525724
                9830289
                36636675
                11d0dd22-9107-4526-9c58-4ecb3cff1528
                Copyright © 2022 by The Author(s). Published by S. Karger AG, Basel

                This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.

                History
                : 30 November 2021
                : 21 June 2022
                : 2022
                Page count
                Figures: 3, References: 13, Pages: 6
                Funding
                The authors received no fund for this case report.
                Categories
                Case Report

                Oncology & Radiotherapy
                hemangiopericytoma,solitary fibrous tumor,bone neoplasms,parietal bone,adjuvant radiotherapy

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