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• W2064124743 abstract "Malignant giant solitary fibrous tumor (SFT) of the mediastinum is a rare neoplasm derived from mesenchymal tissue. Owing to its large size, a complete resection of the tumor can present many challenges, particularly given its proximity to vital neighboring structures. We report a successful en-bloc resection of a massive mediastinal SFT, which was compressing the inferior trachea and heart, by means of a median sternotomy and an anterior left thoracotomy. We emphasize the rarity of this uncommon mediastinal mass. Key points of mediastinal SFT are discussed. Malignant giant solitary fibrous tumor (SFT) of the mediastinum is a rare neoplasm derived from mesenchymal tissue. Owing to its large size, a complete resection of the tumor can present many challenges, particularly given its proximity to vital neighboring structures. We report a successful en-bloc resection of a massive mediastinal SFT, which was compressing the inferior trachea and heart, by means of a median sternotomy and an anterior left thoracotomy. We emphasize the rarity of this uncommon mediastinal mass. Key points of mediastinal SFT are discussed. A 64-year-old man presenting with a mediastinal mass was referred to our hospital. Subsequent to a chest x-ray and computed tomography (CT), a diagnostic work-up revealed a large anterior mediastinal mass (142 × 106 mm) extending from the confluence of the brachiocephalic veins to the diaphragm. It significantly displaced both the carina and the heart posteriorly. Moreover, the mass displayed a marked heterogenicity suggestive of necrosis (Figure 1). No pleural effusion or significant lymph nodes were noticeable. Transthoracic echocardiography showed a mass that extrinsically compressed the right ventricle outflow tract, causing pulmonal valve pseudo-stenosis (maximal gradient of 31 mmHg). Positron emission tomography (PET) with [18fluorine]fluorodeoxyglucose (FDG) showed a large heterogenic hypermetabolic area in the anterior mediastinum. A CT-guided transcutaneous aspiration biopsy was preoperatively performed and was compatible with a tumor of mesenchymal origin. The histologic and immunohistochemical profile suggested that the mass was a solitary fibrous tumor (SFT). The patient underwent surgical resection, using combined median sternotomy and anterior left thoracotomy (Figure 2). The tumor appeared to arise from the mediastinal pleura on the left side. There was no invasion of vital neighboring structures or strong adherence to other mediastinal structures. The excised specimen measured 17.5 cm × 14 cm × 10 cm.FIGURE 2Combined median sternotomy and left anterior thoracotomy for removal of a giant mediastinal solitary fibrous tumor.View Large Image Figure ViewerDownload (PPT) Immunohistochemical analysis of the tumor cells showed consistent positive staining for smooth muscle actin, Bcl-2, cluster of differentiation 99 (CD99), and CD34; negative staining of the tumor cells was shown for low molecular weight cytokeratin (CAM 5.2), cytokeratin (KL1), S100, desmin, (CD31), chromogranin, and synaptophysin. However, an unusual histologic pattern of SFT was noted (i.e., tissue necrosis, focal increased mitotic activity of more than 4 mitoses per 10 high power field, high cellularity, large tumor size (175 mm), and no strong pleiomorphic tumor cells), which led to the classification of the tumor as a malignant giant SFT of the mediastinum (FIGURE 3, FIGURE 4). Focally, the tumor reached the surgical margin of the mediastinal pleura on the left side. Fluorescence in situ hybridization analysis showed an absence of the t(X;18) translocation, which ruled out the presence of synovial sarcoma.FIGURE 4Light photomicrograph of a malignant giant solitary fibrous tumor of the mediastinum showing focal increased mitotic activity (>4 mitoses/10 HPF) (arrows).View Large Image Figure ViewerDownload (PPT) After multidisciplinary deliberation, our patient underwent adjuvant radiotherapy (a cumulative dosage of 60 Gy in 30 fractions of 2 Gy), given that the tumor focally reached the surgical margin. The patient is doing well 1 year after surgery, with a stable body weight. Semi-annual radiologic follow-ups (CT scan and FDG-PET) have shown no signs of recurrence. SFTs (so-called fibrous mesotheliomas) are rare primary neoplasms in adults. They typically present as intrathoracic pleural-based lesions. Approximately 1 to 8% of these intrathoracic tumors have been reported to occur in the mediastinum. The frequency of mediastinal localization is identical in both men and women. The tumor generally develops between the fifth and seventh decades. Fortuitous discovery governs the success of treatment. Benign forms of the tumor are three- to four-times more common than the malignant forms. Although the pathogenesis is obscure, the currently accepted theory is a derivation from submesothelial mesenchymal cells with fibroblastic differentiation.1Engeland DM Hochholzer L McCarthy MJ Localized benign and malignant fibrous tumours of the pleura. A clinicopathologic review of 223 cases.Am J Surg Pathol. 1989; 13: 640-658Crossref PubMed Scopus (1065) Google Scholar, 2Briselli M Mark EJ Dickersin GR Solitary fibrous tumours of the pleura: eight new cases and review of 360 cases in the literature.Cancer. 1981; 47: 2678-2689Crossref PubMed Scopus (586) Google Scholar, 3Within GB Rosai J Solitary fibrous tumour of the mediastinum. A report of 14 cases.Am J Surg Pathol. 1989; 13: 547-557Crossref PubMed Scopus (232) Google Scholar The histologic features of SFTs mainly demonstrate 2 patterns (i.e., patternless and prominent vascularity). Immunohistochemically, SFTs commonly express CD34, CD99, and Bcl-2; epithelial membrane antigen and smooth muscle actin may also be expressed. They are usually negative for S-100 protein, desmin, and cytokeratins. These findings support the currently favored view that SFTs are composed of submesothelial mesemchymal cells. Pathologic criteria of malignancy include a large tumor size (more than 50 mm), infiltrative margins, high cellularity, nuclear pleiomorphism, an area of tissue necrosis, and an increased mitotic index (more than 4 mitoses in 10 high power field)1Engeland DM Hochholzer L McCarthy MJ Localized benign and malignant fibrous tumours of the pleura. A clinicopathologic review of 223 cases.Am J Surg Pathol. 1989; 13: 640-658Crossref PubMed Scopus (1065) Google Scholar, 2Briselli M Mark EJ Dickersin GR Solitary fibrous tumours of the pleura: eight new cases and review of 360 cases in the literature.Cancer. 1981; 47: 2678-2689Crossref PubMed Scopus (586) Google Scholar, 3Within GB Rosai J Solitary fibrous tumour of the mediastinum. A report of 14 cases.Am J Surg Pathol. 1989; 13: 547-557Crossref PubMed Scopus (232) Google Scholar, 4Gold JS Antonescu CR Hajdu C et al.Clinicopathologic correlates of solitary fibrous tumour.Cancer. 2002; 94: 1057-1068Crossref PubMed Scopus (473) Google Scholar; these criteria were observed in the tumor from our patient. Differential diagnoses of SFTs are extensive and consist of epithelioid sarcoma, schwannoma, leiomyosarcoma, malignant peripheral nerve sheath tumor, hemangiopericytoma, and synovial sarcoma, among others.5Fletcher CD The evolving classification of soft tissue tumours: an update based on the new WHO classification.Histopathology. 2006; 48: 3-12Crossref PubMed Scopus (409) Google Scholar The clinical behavior of this tumor is unpredictable. Signs and symptoms, if any, are usually associated with larger tumors and occur more frequently in malignant subtypes. Symptoms are the result of either local compression/invasion of adjacent thoracic structures (e.g., dyspnea, angina, and/or cough) or paraneoplastic syndromes (e.g., hypoglycemia, weight loss, and/or pulmonary osteoarthropathy).1Engeland DM Hochholzer L McCarthy MJ Localized benign and malignant fibrous tumours of the pleura. A clinicopathologic review of 223 cases.Am J Surg Pathol. 1989; 13: 640-658Crossref PubMed Scopus (1065) Google Scholar, 2Briselli M Mark EJ Dickersin GR Solitary fibrous tumours of the pleura: eight new cases and review of 360 cases in the literature.Cancer. 1981; 47: 2678-2689Crossref PubMed Scopus (586) Google Scholar The diagnostic and prognostic criteria for mediastinal tumors are identical to those for localized fibrous tumors of the pleura. Tissue is required for diagnosis. The accurate diagnosis of SFT is aided by ancillary techniques such as immunohistochemical staining. A preoperative diagnosis of SFT based on radiologic imaging can be difficult, and differentiation between malignant and benign forms is often impossible by CT or magnetic resonance imaging. The diagnostic accuracy of a CT-guided aspiration biopsy has been shown to be poor and may not be helpful in making the decision about the need for surgery. A CT-guided aspiration biopsy may be helpful in cases where the diagnosis of SFT is uncertain. To date, there are few reports of a FDG-PET performed in patients with these tumors. FDG-PET could possibly predict or preoperatively rule out malignancy in a SFT and may reveal metastatic recurrence, an event rarely observed in these tumors. Large SFTs with increased FDG-uptake have a likelihood for malignancy, but the role of a FDG-PET in clinical practice must be further defined.1Engeland DM Hochholzer L McCarthy MJ Localized benign and malignant fibrous tumours of the pleura. A clinicopathologic review of 223 cases.Am J Surg Pathol. 1989; 13: 640-658Crossref PubMed Scopus (1065) Google Scholar, 6Magdeleinat P Alifano M Petino A et al.Solitary fibrous tumours of the pleura: clinical characteristics, surgical treatment and outcome.Eur J Cardiothorac Surg. 2002; 21: 1087-1093Crossref PubMed Scopus (182) Google Scholar The treatment of choice for SFT is extensive surgical resection. Surgical excision is curative for most benign lesions; however, malignant lesions will often recur and progress despite adjuvant radiation or chemotherapy. The most important indicator of the clinical outcome is whether or not the tumor can be totally excised in the initial operation. In the case of a recurrence, a surgical resection offers the best chance for a complete cure. Few cases using adjuvant radiotherapy or chemotherapy in malignant SFT have been reported, and the effectiveness of radiation and chemotherapy has not been proven.1Engeland DM Hochholzer L McCarthy MJ Localized benign and malignant fibrous tumours of the pleura. A clinicopathologic review of 223 cases.Am J Surg Pathol. 1989; 13: 640-658Crossref PubMed Scopus (1065) Google Scholar, 2Briselli M Mark EJ Dickersin GR Solitary fibrous tumours of the pleura: eight new cases and review of 360 cases in the literature.Cancer. 1981; 47: 2678-2689Crossref PubMed Scopus (586) Google Scholar, 6Magdeleinat P Alifano M Petino A et al.Solitary fibrous tumours of the pleura: clinical characteristics, surgical treatment and outcome.Eur J Cardiothorac Surg. 2002; 21: 1087-1093Crossref PubMed Scopus (182) Google Scholar In conclusion, a long-term clinical follow-up is recommended for all patients with a SFT because of its potential adverse biologic behavior that could lead to repeated recurrences and/or malignant transformation. The behavior of a SFT is unpredictable, and the relationship between morphology and outcome is poor. For example, some “malignant” tumors behave as if benign, whereas morphologically “benign” lesions sometimes are aggressive in nature. In addition to the presence of histologic criteria of malignancy, an absence of sclerotic-hypocellular areas and a tumor size of more than 10 mm have been considered predictors of a poor outcome. The local recurrence or onset of metastases depends on histologic parameters. In cases of a benign classification (60–80%), the recurrence rate was only 2% after surgical excision. Approximately half of patients with a malignant SFT can be cured, whereas the rest often develop recurrences and metastases. In the latter cases, emphasis should be placed on continual follow-up examinations. If recurrence occurs in benign lesions, it may be due to incomplete resection, an unrecognized malignancy, or the growth of an unrelated second SFT. A follow-up plan after resection of a malignant SFT should include semi-annual radiologic controls by CT in the first 2 years, and this should be performed on a yearly basis thereafter. A long-term follow-up is mandatory in the case of malignant lesions because of possible late recurrences; these can be locally aggressive and may lead to death through local invasion and compression.1Engeland DM Hochholzer L McCarthy MJ Localized benign and malignant fibrous tumours of the pleura. A clinicopathologic review of 223 cases.Am J Surg Pathol. 1989; 13: 640-658Crossref PubMed Scopus (1065) Google Scholar, 4Gold JS Antonescu CR Hajdu C et al.Clinicopathologic correlates of solitary fibrous tumour.Cancer. 2002; 94: 1057-1068Crossref PubMed Scopus (473) Google Scholar, 7Baliga M Flowers R Heard K Siddiqi A Akhtar I Solitary fibrous tumour of the lung: a case report with a study of the aspiration biopsy, histopathology, immunohistochemistry, and autopsy findings.Diagn Cytopathol. 2007; 35: 239-244Crossref PubMed Scopus (28) Google Scholar, 8Kohler M Clarenbach CF Kestenholz P et al.Diagnosis, treatment and long-term outcome of solitary fibrous tumours of the pleura.Eur J Cardiothorac Surg. 2007; 32: 403-408Crossref PubMed Scopus (51) Google Scholar" @default.
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