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      Head and Neck Solitary Fibrous Tumor Presenting as Salivary Gland Tumor: Two Case Reports and Review of the Literature

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          Abstract

          Solitary fibrous tumors (SFTs) are soft tumors (mesenchymal origin) that most likely develop from adult mesenchymal stem cells. SFTs are not common in the head and neck region, and the characteristics of tumors in this location are unclear. The present study describes the clinicopathological findings of 2 cases of SFTs arising in the parotid gland and buccal space, presenting as salivary gland tumors. The first case is a 76-year-old man presenting with a painless tumor on the right parotid gland who subsequently underwent partial superficial parotidectomy. According to the results of histopathological analysis, the tumor consisted of stellate and spindle-shaped cells proliferating on a mucous-like substrate. Immunohistochemical staining revealed that neoplastic cells were positive for CD34, vimentin, Bcl2, and STAT6. The second case is of a 64-year-old man presenting with a painless lump on his right cheek. Based on the findings of fine needle aspiration cytology, a tumor derived from myoepithelial cells of the minor salivary gland or a nonepithelial tumor was suspected. The patient underwent surgical resection via an intraoral approach. Histopathologically, the tumor consisted of spindle-shaped cells with rod-shaped or irregular nuclei. Immunohistochemical staining revealed that the neoplastic cells were positive for CD34, CD99, Bcl2, and STAT6. Briefly, SFT should be considered in the differential diagnosis of a well-marginalized lesion in the salivary gland and oral cavity. STAT6 immunohistochemistry is the most specific and sensitive method of diagnosing SFT. A thorough understanding of the morphological changes associated with SFT and their correlation with clinical, immunohistochemical, and molecular characteristics is important to avoid misdiagnosis.

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          Most cited references37

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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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            Clinicopathologic correlates of solitary fibrous tumors.

            Solitary fibrous tumors (SFTs) are rare fibrous neoplasms. Since their initial description as arising from the pleura, SFTs have been reported at a wide range of anatomic sites. To the authors's knowledge, there are no large series reporting both thoracic and extrathoracic SFTs nor are there any large series that analyze clinicopathologic correlates of tumor behavior. Institutional soft tissue tumor and pathology databases were reviewed to identify patients. Pathologic material was reviewed by an experienced soft tissue pathologist and scored for the presence of a histologically malignant component. Clinical information was obtained from medical records and phone calls to patients. Statistical analysis was performed using the Student t test, Pearson chi-square test, and log-rank test. Seventy-nine patients with SFTs treated at a single institution over an 18-year period were identified. These tumors arose in a wide range of anatomic sites. Thoracic and extrathoracic SFTs had similar clinical and pathologic features, although extrathoracic tumors were more likely to be symptomatic on diagnosis. Seventy-five patients underwent surgical excision of a SFT at our institution. Overall, SFTs had a low rate of local recurrence and metastasis after surgical treatment. Extrathoracic SFTs had an increased risk of local recurrence that was small but statistically significant. There was no difference in metastasis-free survival between thoracic and extrathoracic SFTs. Positive surgical margins and the presence of a histologically malignant component were factors predicting worse local recurrence-free survival. Positive surgical margins, tumor size greater than 10 cm, and the presence of a malignant component predicted worse metastasis-free survival. Solitary fibrous tumors are rare tumors that occur at all anatomic sites. Most SFT patients fare well after surgical treatment. Closer surveillance is warranted for those tumors that are larger than 10 cm or with the presence of a histologically malignant component. Copyright 2002 American Cancer Society. DOI 10.1002/cncr.10328
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              Solitary fibrous tumor: a clinicopathological study of 110 cases and proposed risk assessment model.

              Solitary fibrous tumor represents a spectrum of mesenchymal tumors, encompassing tumors previously termed hemangiopericytoma, which are classified as having intermediate biological potential (rarely metastasizing) in the 2002 World Health Organization classification scheme. Few series have reported on clinicopathological predictors with outcome data and formal statistical analysis in a large series of primary tumors as a single unified entity. Institutional pathology records were reviewed to identify primary solitary fibrous tumor cases, and histological sections and clinical records reviewed for canonical prognostic indicators, including patient age, tumor size, mitotic index, tumor cellularity, nuclear pleomorphism, and tumor necrosis. Patients (n=103) with resected primary solitary fibrous tumor were identified (excluding meningeal tumors). The most common sites of occurrence were abdomen and pleura; these tumors were larger than those occurring in the extremities, head and neck or trunk, but did not demonstrate significant outcome differences. Overall 5- and 10-year metastasis-free rates were 74 and 55%, respectively, while 5- and 10-year disease-specific survival rates were 89 and 73%. Patient age, tumor size, and mitotic index predicted both time to metastasis and disease-specific mortality, while necrosis predicted metastasis only. A risk stratification model based on age, size, and mitotic index clearly delineated patients at high risk for poor outcomes. While small tumors with low mitotic rates are highly unlikely to metastasize, large tumors ≥ 15 cm, which occur in patients ≥ 55 years, with mitotic figures ≥ 4/10 high-power fields require close follow-up and have a high risk of both metastasis and death.
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                Author and article information

                Journal
                Case Rep Oncol
                Case Rep Oncol
                CRO
                CRO
                Case Reports in Oncology
                S. Karger AG (Basel, Switzerland )
                1662-6575
                3 July 2023
                Jan-Dec 2023
                3 July 2023
                : 16
                : 1
                : 465-473
                Affiliations
                [a ]Department of Otolaryngology, Head and Neck Surgery, Kansai Medical University, Hirakata, Osaka, Japan
                [b ]Department of Pathology, Kansai Medical University, Hirakata, Osaka, Japan
                [c ]Department of Pathology, Shizuoka General Hospital, Shizuoka, Japan
                Author notes
                Correspondence to: Kensuke Suzuki, suzukken@ 123456hirakata.kmu.ac.jp
                Article
                531067
                10.1159/000531067
                10359685
                37485015
                18371a5d-1253-4301-9a4a-a7898b711f14
                © 2023 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
                : 28 December 2022
                : 5 May 2023
                : 2023
                Page count
                Figures: 5, References: 37, Pages: 9
                Funding
                This project was supported by funding from the Academic Society for Research in Otolaryngology, Kansai Medical University. The funding source participated in the study design, collection, analysis, and interpretation of data as well as in writing the manuscript.
                Categories
                Case Report

                Oncology & Radiotherapy
                solitary fibrous tumor,head and neck,parotid gland,buccal space,stat6
                Oncology & Radiotherapy
                solitary fibrous tumor, head and neck, parotid gland, buccal space, stat6

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