Metastatic Malignant Solitary Fibrous Tumor to the Right Ventricular Cavity: A Case Report and Literature Review

Metastatic Malignant Solitary Fibrous Tumor to the Right Ventricular Cavity: A Case Report and Literature Review | Authorea try { document.documentElement.classList.add('js'); } catch (e) { } var _gaq = _gaq || []; _gaq.push(['_setAccount', 'G-8VDV14Y67G']); _gaq.push(['_trackPageview']); (function() { var ga = document.createElement('script'); ga.type = 'text/javascript'; ga.async = true; ga.src = ('https:' == document.location.protocol ? 'https://ssl' : 'http://www') + '.google-analytics.com/ga.js'; var s = document.getElementsByTagName('script')[0]; s.parentNode.insertBefore(ga, s); })(); Skip to main content Preprints Collections Wiley Open Research IET Open Research Ecological Society of Japan All Collections About About Authorea FAQs Contact Us Quick Search anywhere Search for preprint articles, keywords, etc. Search Search ADVANCED SEARCH SCROLL This is a preprint and has not been peer reviewed. Data may be preliminary. 26 August 2025 V1 Latest version Share on Metastatic Malignant Solitary Fibrous Tumor to the Right Ventricular Cavity: A Case Report and Literature Review Authors : Jyothik Varun Inampudi , Andrew Gregory 0000-0001-8260-6193 , John Gallagher 0009-0002-3570-8730 , John Dawdy 0000-0002-5827-1757 , Awadelkarim Abdalaziz , and Luis Afonso [email protected] Authors Info & Affiliations https://doi.org/10.22541/au.175621356.67106392/v1 149 views 96 downloads Contents Abstract Information & Authors Metrics & Citations View Options References Figures Tables Media Share Abstract Solitary fibrous tumors (SFTs) are rare mesenchymal spindle-cell neoplasms that are typically found in the pleura. We present the case of a 74-year-old woman with metastatic colonic cancer who arrived to the emergency department with nausea, fatigue, and lower extremity swelling. Echocardiography revealed a 2.9 cm x 2.9 cm right ventricular mass extending into the pulmonary outflow tract, which was confirmed with a CT scan of the chest. A prior cardiac MRI confirmed a large intracardiac tumor causing right-sided heart dysfunction. The patient expired shortly afterwards. This case highlights the unusual presentation of a SFT within the right ventricle. Introduction Solitary fibrous tumors (SFTs) are rare subtypes of tumors derived from mesenchymal stem cells that form spindles. 1 The most common site for these tumors to develop is within the pleura, 2 with cardiac primary SFTs being exceedingly rare. Although only approximately 20% of SFTs are malignant, 3 benign tumors have the possibility of malignant transformation. The risk of SFT metastasis ranges from 35% 4 to nearly 50% 5 5-10 years after initial diagnosis, with 5- and 10-year disease specific survival rates of approximately 89% and 73%, respectively. 5 Metastasis of SFTs commonly occurs to the lungs, pleura, and liver 4 but metastasis to the heart has been reported in a few cases, primarily to the left atrium. 6 We describe a case of a metastatic SFT within the right ventricle, a finding which has not been reported elsewhere in the literature. Case Presentation A 74-year-old female presented to the emergency department (ED) with worsening nausea, vomiting, fatigue, and bilateral lower extremity swelling and pain for several days. Her past medical history was significant for hypertension, hyperlipidemia, and biopsy-confirmed solitary colonic fibrous sarcoma ( Figure 1) with metastases to the lungs, inguinal lymph nodes, and pelvic bones partially treated with abdominoperineal resection, a colostomy tube, and chemotherapy. Due to tachycardia (118 beats per minute) on presentation to the ED, an electrocardiogram was obtained and showed sinus tachycardia with probable left atrial enlargement and right axis deviation. A chest X-ray revealed a moderate-sized right-sided pleural effusion and a smaller-sized left-sided pleural effusion with an enlarged cardiomediastinal silhouette. A computed tomography (CT) scan of the chest revealed cardiomegaly and an infiltrative lobulated cardiac mass measuring 2.7 x 4.6 x 4.1 x 4.2 x 4.3 cm in the right ventricle with extension to the pulmonary artery outflow tract ( Figure 2 ). Additionally, bilateral pleural effusions with right > left involvement were noted. A transthoracic echocardiogram (TTE) was ordered and revealed a small atrial and left ventricular cavity with hyperdynamic systolic function with a preserved ejection fraction (>80%). Both the right atrium and ventricle (RV) were severely dilated. The RV was hypokinetic with a prominent moderator band and a large intracardiac mass within RV measuring 2.9 cm x 2.9 cm ( Figure 3 ). Mitral and aortic valve regurgitation were trace, but the tricuspid valve (TV) annulus was dilated with severe TV regurgitation with an estimated peak right ventricular systolic pressure of 80-85 mmHg (normal pressure <40 mmHg). The results of a cardiac magnetic resonance imaging (cMRI) scan at an outside facility two months prior to the current presentation were also reviewed. The cMRI showed an irregular multilobulated mass measuring 29 x 18 mm that was adherent to the RV free wall with near total obstruction of the RV outflow tract ( Figure 4). The mass is hyperintense on T2 sequence with heterogeneous uptake on delayed gadolinium hyperenhancement postcontrast images. These findings are consistent with intracardiac metastatic disease in the setting of a known history of malignancy. Perfusion imaging of the mass showed minimal contract enhancement and post flash replenishment on myocardial contrast echocardiography, with reduced vascularity relative to the septal myocardium ( Video 1 ). Given these findings, the patient was evaluated by cardiothoracic surgery and palliative care. Due to her diffuse metastatic disease, she was not deemed to be a surgical candidate. She was discharged to an inpatient hospice facility, where she died within 48 hours due to complications of her metastatic cancer. Discussion In the present case, we describe a 74-year-old woman with metastatic colorectal sigmoid cancer that presented with an intracardiac right ventricular SFT. SFTs arise from the mesenchyme, more specifically from fibroblasts, and can occur in any location within the body but were first characterized in the pleura. 7 On histology, SFTs exhibit ”patternless” architecture with alternating hypercellular and hypocellular areas. 1 The hallmark genetic alteration in SFTs is a NAB2-STAT6 gene fusion, resulting from a paracentric inversion on chromosome 12q13, which leads to downstream activation of cell growth and subsequent tumorigenesis. 8 While most SFTs remain benign, there is a possibility of malignant progression with up to 30% of these tumors displaying such behavior. 9-11 The incidence of SFTs is around 1 new case per million people per year. 1,12,13 SFTs typically present in the 5th or 6th decades of life. 5,14 Primary sites have been reported in multiple organs, including the pleura, distal extremities, trunk, abdominal cavity, head and neck, as well as the meninges. 14,15 When primary SFTs involve the heart, most arise within the pericardial tissue. 16-18 A CT scan of the chest of SFTs within the pericardium usually reveals an anterior mediastinal mass with possible displacement of nearby structures, including the esophagus, great vessels, and trachea. SFTs involving the endocardium are exceptionally rare, with only 1 case being reported in the literature. Suzuki et al. discuss the case of a 24-year-old woman who had a resection of an SFT in her right forearm and was subsequently found to have an SFT in the left atrial endocardium. 19 Eventually, her cardiac SFT was also surgically removed, and both SFTs were found to be malignant. While not as rare as those in the endocardium, SFTs in the epicardium have been described as well, in a total of three patients. 20-22 These three patients ranged from 49-68 years old, with two of them later having the epicardial masses resected. 20-22 The third patient received a heart transplant and died of a post-operative infection, at which point the epicardial SFT was discovered. 21 To date, only 1 other case of an RV SFT has been reported. This patient presented with a tumor that grew within the pericardial cavity of the RV wall, and which was ultimately surgically resected along with a portion of the ventricular wall. 18 Our case contributes to the literature for two important reasons. First, we demonstrated a rare presentation of SFT in the heart as a result of metastasis from a distant organ. While metastasis of SFTs may occur most commonly to the lungs, pleura, and liver, 4 metastasis to the heart has only been reported in a few cases, primarily to the left atrium. 6 Second, intraventricular involvement of an SFT within the RV from distant metastasis is a finding which has never been reported elsewhere in the literature. In SFTs, cardiac MRIs reveal hypointensity and variable intensity on T1 and T2-weighted imaging, respectively, which are related to collagen content and vascularity within the tumor. 23 Chest CT scans can also be helpful, especially in SFTs within the thoracic or abdominal cavities, where variable enhancement of intravenous contrast may be revealed. 24 TTE with perfusion imaging may also be used and shows minimal contrast enhancement with post flash replenishment on TTE due to vascularity as in our patient. While imaging strongly suggests the diagnosis, the definitive diagnosis requires histologic analysis and STAT6 immunohistochemical staining following tissue biopsy. 25 Prompt diagnosis and treatment are important, as the 5-year overall survival rate of SFT is estimated to be 84%, with 5-year local recurrence free survival and 5-year metastasis-free survival of 71% and 66%, respectively. 26 With metastasis, survival rates fall significantly, with 3-year and 5-year rates of 73% and 49%, respectively. 27 The gold standard of treatment for SFTs remains surgical resection, which when complete offers good prognosis especially in cases of localized disease. 26,28 For cases where operative removal is not recommended, chemotherapy and radiotherapy may be considered though with less favorable outcomes. 26,28,29 Conclusion In conclusion, we report a case of a colonic SFT with metastasis to the right ventricular cavity, a location of SFT metastasis which has not previously been reported in the literature. SFTs are a rare entity, and though most are benign, malignant transformation with metastasis may occur. Such possibilities should be considered, and this report shows that even cardiac involvement is possible for an SFT, with the reported finding of an intracardiac mass in the RV of our patient. Monitoring patients with SFT for metastasis is crucial to prevent serious complications and ensure timely referral for treatment, such as surgical resection. Figure 1. Histology image showing the patient’s solitary fibrous tumor of the sigmoid colon which was positive for the NAB2-STAT6 gene fusion. Characteristic collagen bundles (black arrows), hypervascularity (blue arrows), and fibroblastic-like mesenchymal cells (white arrows) are observed. Figure 2. Computed tomography (CT) scan showing cardiomegaly and an infiltrative lobulated cardiac mass measuring approximately 2.7 x 4.6 x 4.1 x 4.2 x 4.3 cm (see asterisk) in the right ventricle with extension to the pulmonary artery outflow tract. Pleural effusions are also observed, worse on the right than left. Figure 3. A) TTE demonstrating a severely dilated and hypokinetic right ventricle with an intracardiac mass measuring about 2.9 x 2.9 cm (white arrows) with resultant right ventricular outflow tract obstruction. B) Relatively avascular mass on perfusion imaging. The mass is indicated by a white asterisk in both images. Abbreviations: LA = left ventricle; RA = right atrium; LV = left ventricle; Ao = Aorta; TTE = transthoracic echocardiography. Figure 4. Cardiac magnetic resonance imaging (MRI) scan showing an irregular multilobulated 29 x 18 mm mass adherent to the right ventricular free wall at the beginning of the ventricular outflow tract. The mass is hyperintense on T2 sequence with heterogeneous uptake on delayed gadolinium hyperenhancement postcontrast images. These findings are consistent with intracardiac metastatic disease in the setting of a known history of malignancy. Video 1 . Perfusion imaging of the mass showed minimal contract enhancement and post flash replenishment on myocardial contrast echocardiography, with reduced vascularity relative to the septal myocardium. References 1. Martin-Broto J, Mondaza-Hernandez JL, Moura DS, Hindi N. A comprehensive review on solitary fibrous tumor: New insights for new horizons. Cancers . 2021;13(12):2913. doi:10.3390/cancers13122913 2. Ren C, D’Amato G, Hornicek FJ, Tao H, Duan Z. Advances in the molecular biology of the solitary fibrous tumor and potential impact on clinical applications. Cancer Metastasis Rev . 2024;43(4):1337-1352. doi:10.1007/s10555-024-10204-8 3. Robinson LA. Solitary fibrous tumor of the pleura. Cancer Control . 2006;13(4):264-269. doi:10.1177/107327480601300403 4. O’Neill AC, Tirumani SH, Do WS, et al. Metastatic patterns of solitary fibrous tumors: A single-institution experience. Am J Roentgenol . 2017;208(1):2-9. doi:10.2214/ajr.16.16662 5. Demicco EG, Park MS, Araujo DM, et al. 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Keywords echocardiography magnetic resonance imaging metastasis right ventricle solitary fibrous tumor Authors Affiliations Jyothik Varun Inampudi Wayne State University Department of Internal Medicine View all articles by this author Andrew Gregory 0000-0001-8260-6193 Wayne State University School of Medicine View all articles by this author John Gallagher 0009-0002-3570-8730 Wayne State University School of Medicine View all articles by this author John Dawdy 0000-0002-5827-1757 Henry Ford Hospital View all articles by this author Awadelkarim Abdalaziz Wayne State University Department of Internal Medicine View all articles by this author Luis Afonso [email protected] Wayne State University Department of Internal Medicine View all articles by this author Metrics & Citations Metrics Article Usage 149 views 96 downloads .FvxKWukQNSOunydq8rnd { width: 100px; } Citations Download citation Jyothik Varun Inampudi, Andrew Gregory, John Gallagher, et al. Metastatic Malignant Solitary Fibrous Tumor to the Right Ventricular Cavity: A Case Report and Literature Review. Authorea . 26 August 2025. DOI: https://doi.org/10.22541/au.175621356.67106392/v1 If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. Simply select your manager software from the list below and click Download. For more information or tips please see 'Downloading to a citation manager' in the Help menu . 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