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      Incidental finding of solitary fibrous tumor of male breast: case report and review of literature

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          Abstract

          Solitary fibrous tumor (SFT) of the breast is a rare mesenchymal tumor composed of spindle-shaped tumor cells with collagen and large blood vessels in the shape of a “staghorn”. It is discovered anywhere in the human body, usually incidentally or through nonspecific symptoms. A combination of clinical, histological, and immunohistochemical features is required to establish a diagnosis. There are no proper guidelines for the treatment of SFTs because of their rarity; however, wide surgical excision remains the “gold standard”. A multidisciplinary team approach is recommended. They are mostly benign with a 5-year survival rate of 89%. Following a PubMed-indexed English literature review, only six publications presenting nine cases of breast SFT in a male patient were found. The following is the case of a 73-year-old man who presented with dry cough. A SFT in the right breast was discovered incidentally during the investigative work up, and the patient was referred to our Breast Clinic at the Jules Bordet Institute, Brussels, Belgium, for appropriate treatment. The patient’s presentation, imaging, and histological sample all supported the diagnosis, and he underwent uneventful surgical resection. Here, we present the first case of an incidental finding of a SFT of the male breast, with its diagnosis and therapeutic challenges.

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          Most cited references20

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          The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior

          Background Solitary Fibrous Tumor (SFT) is a distinct soft tissue neoplasm associated with NAB2-STAT6 gene fusion. It can involve a number of anatomic sites and exhibits a wide spectrum of histological features. Main body Apart from diversity in morphological features seen even in conventional SFT, two histologic variants (fat-forming and giant cell-rich) are also recognized. In addition, a malignant form and dedifferentiation are well recognized. Owing to diverse histological features and involvement of diverse anatomic locations, SFT can mimic other soft tissue neoplasms of different lineages including schwannoma, spindle cell lipoma, dermatofibrosarcoma protuberans, liposarcoma, gastrointestinal stromal tumor (GIST), malignant peripheral nerve sheath tumor (MPNST), and synovial sarcoma. SFT is classified as an intermediate (rarely metastasizing) tumor according to World Health Organization Classification of Tumors of Soft tissue and Bone, 5th edition. The management and prognosis of SFT differs from its malignant mimics and correct diagnosis is therefore important. Although SFT expresses a distinct immunohistochemical (IHC) profile, the classic histomorphological and IHC profile is not seen in all cases and diagnosis can be challenging. NAB2-STAT6 gene fusion has recently emerged as a sensitive and specific molecular marker and its IHC surrogate marker signal transducer and activator of transcription 6 (STAT6) has also shown significant sensitivity and specificity. However, few recent studies have reported STAT6 expression in other soft tissue neoplasms. Conclusion This review will focus on describing the diversity of histological features of SFT, differential diagnoses and discussing the features helpful in distinguishing SFT from its histological mimics.
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            Spindle cell lipoma-like tumor, solitary fibrous tumor and myofibroblastoma of the breast: a clinico-pathological analysis of 13 cases in favor of a unifying histogenetic concept.

            We reviewed the clinico-pathological features of a series of 13 cases of benign spindle stromal tumors (BSSTs) of the breast relating to a basic common theme consisting of a well-circumscribed proliferation of vimentin+/CD34+/BCL-2+/CD99+ spindly to oval-epithelioid cells, variably arranged in haphazard to short fascicular growth pattern, with interspersed thick or thin collagen bands. Morphological variations included atypical mono- or multi-nucleated cells in five cases and a mature lipomatous tumor component, varying from focal to prominent, in eight cases. Based on morphological and immunophenotypical features, a distinction was made between two main subtypes of these tumors--fibroblastic and myofibroblastic. The former subtype included two cases respectively represented by a typical solitary fibrous tumor (SFT) and a neoplasm labeled "spindle-cell lipoma (SCL)-like tumor", closely reminiscent of soft tissue SCL. Both tumors had cells with fibroblastic-like appearance, haphazardly arranged and immunoreactive for vimentin, CD34, BCL-2, and CD99. The latter subtype, comprised nine cases exhibiting evidence of myofibroblastic differentiation (desmin and alpha-smooth muscle actin) which were classified as myofibroblastomas (MFBs). The remaining two cases were defined as "mixed BSSTs", having typical features of diverse neoplasms, respectively represented by a case of MFB with focal SFT and pleomorphic/SCL-like areas, and SFT with focal MFB-like component. The common basic morpho-immunophenotypical features, the possibility that both fibroblastic and myofibroblastic tumors may contain an additional mature lipomatous component, and the existence of hybrid stages (mixed BSSTs) strongly support the view that such tumors belong to the same category of lesions. We postulate that the precursor of all these neoplasms is the vimentin+/CD34+ cells of the mammary stroma, the well-known inherent plasticity of which to differentiate toward several mesenchymal lines, provides the explanation for the phenotypic heterogeneity of these neoplasms. Accordingly, the encompassing term "benign spindle stromal tumors of the breast" is advocated for such tumors.
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              Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond.

              SFT is an ultrarare mesenchymal ubiquitous tumor, with an incidence rate <1 case/million people/year. The fifth WHO classification published in April 2020 subdivided SFT into three categories: benign (locally aggressive), NOS (rarely metastasizing), and malignant. Recurrence can occur in up to 10-40% of localized SFTs, and several risk stratification models have been proposed to predict the individual risk of metastatic relapse. The Demicco model is the most widely used and is based on age at presentation, tumor size, and mitotic count. Total en bloc resection is the standard treatment of patients with a localized SFT; in case of advanced disease, the clinical efficacy of conventional chemotherapy remains poor. In this review, we discuss new insights into the biology and the treatment of patients with SFT. NAB2-STAT6 oncogenic fusion, which is the pathognomonic hallmark of SFT, is supposedly involved in the overexpression of vascular endothelial growth factor (VEGF). These specific biological features encouraged the successful assessment of antiangiogenic drugs. Overall, antiangiogenic therapies showed a significant activity toward SFT in the advanced/metastatic setting. Nevertheless, these promising results warrant additional investigation to be validated, including randomized phase III trials and biological translational analysis, to understand and predict mechanisms of efficacy and resistance. While the therapeutic potential of immunotherapy remains elusive, the use of antiangiogenics as first-line treatment should be considered.
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                Author and article information

                Journal
                Rom J Morphol Embryol
                Rom J Morphol Embryol
                RJME
                Romanian Journal of Morphology and Embryology
                Academy of Medical Sciences, Romanian Academy Publishing House, Bucharest
                1220-0522
                2066-8279
                Oct-Dec 2022
                31 December 2022
                : 63
                : 4
                : 653-657
                Affiliations
                [1 ] Department of Surgical Oncology, Jules Bordet Institute, Université Libre de Bruxelles, Brussels, Belgium
                [2 ] Department of Radiology, Jules Bordet Institute, Université Libre de Bruxelles, Brussels, Belgium
                [3 ] Department of Pathology, AZ Zeno, Knokke-Heist, Belgium
                [4 ] Department of Pathology, Jules Bordet Institute, Université Libre de Bruxelles, Brussels, Belgium
                [5 ] Department of Medical Oncology, Jules Bordet Institute, Université Libre de Bruxelles, Brussels, Belgium
                [6 ] Department of Radiology, Emergency County Hospital, Sibiu, Romania
                Author notes
                Corresponding Author: Florin Grosu, MD, PhD Department of Radiology Emergency County Hospital Sibiu 2–4 Corneliu Coposu Street 550245 Sibiu Romania + +40756–097 966 dr.floringrosu@ 123456gmail.com
                Corresponding Author: Cătălin Florin Pop, MD Department of Surgery Jules Bordet Institute, Université Libre de Bruxelles 90 Rue Meylemeersch 1070 Brussels Belgium + +3225–413 244 \ + +3225–413 132 catalin.florin.pop@ 123456hubruxelles.be
                Article
                630422653657
                10.47162/RJME.63.4.08
                10026915
                36808201
                cf9868b1-86ac-4faf-b58b-c7bee32359c7
                Copyright © 2022, Academy of Medical Sciences, Romanian Academy Publishing House, Bucharest

                This is an open-access article distributed under the terms of a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International Public License, which permits unrestricted use, adaptation, distribution and reproduction in any medium, non-commercially, provided the new creations are licensed under identical terms as the original work and the original work is properly cited.

                History
                : 21 November 2022
                : 06 February 2023
                Categories
                Case Report

                solitary fibrous tumor , breast tumors , male tumors , breast conserving surgery , mastectomy

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