A case of primary undifferentiated pleomorphic sarcoma of the heart complicated by multiple cerebral embolisms and literature review

A case of primary undifferentiated pleomorphic sarcoma of the heart complicated by multiple cerebral embolisms and literature review | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Research Article A case of primary undifferentiated pleomorphic sarcoma of the heart complicated by multiple cerebral embolisms and literature review Nana Tian, Xiaojie Dai, Hongxing Zhang, Yi Jia, Tao Wu, Bo Ning, and 9 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-8016932/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Primary cardiac undifferentiated pleomorphic sarcoma is extremely rare and associated with many complications and poor prognosis. This report hereby presents the diagnosis and treatment process of a patient with primary undifferentiated pleomorphic sarcoma of the heart complicated by multiple cerebral embolisms, with the aim of enhancing clinicians' understanding of primary undifferentiated pleomorphic sarcoma of the heart complicated by cerebral embolism. Primary undifferentiated pleomorphic sarcoma of the heart Cerebral embolism Figures Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 Figure 8 Case information A 56-year-old female was admitted to the hospital due to sudden weakness in the right limb and slurred speech for more than 2 hours. At symptom onset, the right limb was completely immobile. Physical examination: bilateral cervical lymph nodes were enlarged, multiple masses could be palpated in bilateral thyroid glands. The heart rhythm was regular, the first heart sound was normal, A2>P2, and lasag-like murmurs during systolic and diastolic periods could be heard in the mitral valve auscultation area. Neurological physical examination: Consciousness was clear, articulation was unclear, right-handed, right central facial and lingual palsy, bilateral pharyngeal reflexes were weakened, the right limb hemiplegia test was positive, the right finger-nose test and the calcineal-knee-tibial test was not stable and accurate, bilateral pathological signs were not elicited, and no positive localization signs were found in the remaining neurological physical examination. NIHSS score: 7 points. Past medical history One year ago, the patient experienced chest tightness and shortness of breath after activities. Color Doppler ultrasound examination of the heart before August indicated a space-occupying lesion in the left atrium (possibly myxoma), EF74% (see Fig 1a and1b).Subsequently, cardiac malignant tumor resection + left atrial thrombus evacuation surgery was performed. During the operation, there was a dark red irregular mass of 50mm×40mm×40mm in the left atrium(see Fig 1c). The pedicle of the mass was wide and located at the foramen ovale of the atrial septum. Histopathological examination confirmed an undifferentiated pleomorphic sarcoma of the left atrium (see Fig 2a and 2b). After the operation, warfarin sodium tablets were taken for anticoagulation treatment due to atrial fibrillation. Seven months ago, after cardioversion with electrical defibrillation, warfarin sodium tablets were discontinued for anticoagulation therapy. Four months before hospitalization, the patient once again presented with symptoms of chest tightness and shortness of breath. Whole-body PET-CT and contrast-enhanced CT revealed metastases in the pulmonary artery, pulmonary vein, pericardium, inferior vena cava, mediastinum, bilateral adrenal glands and renal vein. Three months before hospitalization, color Doppler ultrasound of the heart revealed tumor recurrence. Pembrolizumab injection + anlotinib were administered for treatment. After the treatment, the volume of the cardiac tumor gradually shrank, and the symptoms of chest tightness and shortness of breath gradually alleviated. Admission diagnosis 1. Cerebral infarction (TOAST classification: Cardiogenic embolic type); 2. Postoperative recurrence of undifferentiated pleomorphic sarcoma in the left atrium accompanied by multiple metastases. Auxiliary examinations: Blood routine, blood lipid, and fasting blood glucose were approximately normal. Coagulation: APTT 43.40 s,FIB 662.0mg/dl. No abnormalities were found in the remaining biochemical tests. Electrocardiogram: Sinus rhythm, myocardial injury; Color Doppler ultrasound of the heart: EF60%. A low echo of 20mm×15mm can be seen on the atrial septal side of the left atrium. It swings with the heart cycle. The left ventricular systolic function is normal. Mitral regurgitation (low to moderate).The head CT examination in the emergency department showed no bleeding. Due to the postoperative surgery for undifferentiated pleomorphic sarcoma of the left atrium, the residual metal substances in the body could not be used for magnetic resonance examination. Treatment plan Considering that the symptoms of the patient at the onset of the disease were that the right limb was at its most severe and completely immobile, the weakness of the right limb had been relieved by the time of admission, and the right limb paraplegia test was positive. Although the time from the onset to admission was within the time window of intravenous thrombolysis, the undifferentiated pleomorphic sarcoma in the left atrium of the patient recurred and had multiple metastases. Whether there was intracranial metastasis could not be ruled out (intracranial tumors are contraindications for intravenous thrombolysis). Therefore, intravenous thrombolytic therapy was not performed. Considering the patient's clinical symptoms and the absence of indications for emergency intracranial vascular intervention, and taking all the above into account, as well as informing the patient's family of the condition and having them agree and sign the informed consent form, 5,000 U of low molecular weight heparin sodium was administered each time, with anticoagulant therapy every 12 hours. Two days after admission, a re-examination of the head CT scan indicated a low-density shadow in the left basal ganglia region (see Figure 3). Three days after admission, warfarin sodium tablets were added for anticoagulation treatment, and low-molecular-weight heparin sodium was gradually discontinued. In the later stage, warfarin alone was used for anticoagulation treatment.Treatment plan for primary undifferentiated pleomorphic sarcoma of the heart: The cardiac surgery consultation suggests continuing targeted drug therapy and temporarily not considering surgical treatment. Changes in the condition and treatment plan On the 10th day after admission, the patient suddenly experienced weakness in the right limb accompanied by speech impairment again. Neurological examination: Clear consciousness, complete aphasia, both eyes staring to the left, the right nasolabial sulcus is shallower than the left one, tongue protruding to the right, right upper limb muscle strength grade 0, right lower limb muscle strength grade 2, positive right Babinski sign. The NIHSS score was 12 points (gaze 1 point + facial paralysis 1 point + right upper limb muscle strength 4 points + right lower limb muscle strength 3 points + speech 2 points + articulation 1 point). Urgent head CT examination: Cerebral infarction in the left basal ganglia region. Considering the recurrence of acute cerebral embolism, emergency general anesthesia was used to perform thrombectomy through the left middle cerebral artery catheter and thrombectomy with a left middle cerebral artery stent(see Fig 4a, b), and the postoperative blood flow grade of LMCA was 2B. The results of the re-examination of the head CT on the 12th day of admission: a new low-density shadow was added in the left frontal lobe (see Fig5). Neurological examination of the patient: Clear consciousness, poor comprehension, no speech, right central facial and lingual paralysis, right limb muscle strength grade 4, positive right Babinski sign. The NIHSS score is 7.On the 12th day after admission, the patient suddenly experienced consciousness disorders again, aphasia, staring to the right with both eyes, unequal pupils on both sides, 3.5mm on the left side and 3.0mm on the right side, sensitive response to light, no muscle strength of grade 0 in the left limb, muscle strength of grade 2 in the right limb, positive bilateral Babinski sign, and a NIHSS score of 16 points. Emergency head and neck CTA examination indicated occlusion of the right internal carotid artery (see Fig 6). Intracranial angioplasty was performed again. During the operation, it was found that the right internal carotid artery was completely occlusive beyond the ophthalmic artery, with a large embolus load. After thrombectomy using stent technology, the right middle cerebral artery beyond M1 was not recanalized, and the right anterior and posterior communicating arteries were partially recanalized. The postoperative blood flow grade of LMCA was 2a(see Fig 7)。Head CT on the 13th day: A low-density shadow was newly added in the right cerebral hemisphere (see Fig 8). Adjustment of the treatment plan Considering that the emboli of the patient's acute cerebral embolism originated from undifferentiated pleomorphic sarcoma tissue of the heart rather than thrombus, warfarin sodium tablets were discontinued for treatment. After the operation, the patient underwent tracheotomy and mechanical ventilation, presenting a dermal state, with unconscious eye opening. The bilateral pupils were of equal size and round, with a diameter of 2.5mm. The patient was sensitive to light reflex, and the pain stimulated flexure and adduction of both upper limbs and hyperextension of both lower limbs. Positive bilateral Babinski sign. The patient underwent tracheal intubation and ventilator-assisted ventilation after the operation. Ultimately, the patient died after being admitted to the hospital for more than three months due to repeated cerebral embolism, which led to limb paralysis and long-term bedridden status. Discussion Primary cardiac tumors are extremely rare, with a detection rate ranging from 0.0017% to 0.33%, and 90% of them are benign [1, 2]. Primary malignant cardiac tumors account for only 10% of primary cardiac tumors, among which undifferentiated pleomorphic sarcomas make up 10%[2]. The most malignant one is the undifferentiated sarcoma. Undifferentiated sarcoma refers to a general term for a group of sarcomas whose tissue types cannot be determined by gross, microscopic or immunohistochemical methods [1]. Patients with primary undifferentiated pleomorphic sarcoma of the heart have a rapid disease progression. Studies have shown that the local recurrence rate and metastasis rate are 13-42% and 31-35%, respectively [3], with a poor prognosis and an average survival period of only one year [1]. The main clinical symptoms depend on the size and location of the tumor, such as sudden death, embolism, obstruction and arrhythmia, etc. [2] Some patients may have no symptoms of the heart and first seek medical attention due to embolic events [4]. Auxiliary examinations include echocardiography, CT, cardiac MRI, PET-CT, etc. Cardiac MRI is superior to echocardiography and CT in differentiating the nature of soft tissues, distinguishing heart tissues, thrombosis, and benign/malignant lesions [5]. PET-CT is helpful in differentiating benign and malignant space-occupying lesions in the heart or pericardium. Literature reports that when the maximum SUV value is > 3.5, the sensitivity for diagnosing malignant cardiac tumors is 100%[5, 6]. Complete surgical resection is the main method for treating sarcoma. The completeness of tumor resection has a significant impact on prognosis. The survival rate of patients with negative tumor resection margins was significantly higher than that of patients with positive tumor resection margins. The combined treatment consisting of surgical resection and postoperative chemotherapy is associated with a better survival rate of patients [7]. Cardiogenic stroke is defined as the detachment of cardiogenic emboli, which leads to the blockage of the corresponding cerebral arteries and eventually results in ischemic stroke. It accounts for 14% - 30% of all ischemic strokes [8]. Cardiogenic stroke is closely related to various cardiovascular diseases. The most common high-risk factors include atrial fibrillation, recent (within 4 weeks) myocardial infarction, artificial mechanical valves, dilated cardiomyopathy, rheumatic mitral stenosis, etc., followed by infectious and non-infectious endocarditis, atrial myxoma, etc[8] .There are relatively few reports on cerebral embolism caused by primary undifferentiated pleomorphic sarcoma of the heart. Such cardiac embolic fragments can be substances of the tumor itself or thrombotic substances formed on the tumor surface when the tumor is exposed to blood flow [4]. There is no sufficient evidence to suggest that anticoagulation can reduce the incidence of embolic events in cardiac myxomas and other cardiac tumors. Therefore, there is currently no unified treatment opinion for patients with cerebral embolism caused by primary undifferentiated pleomorphic sarcoma of the heart [4]. Combined with the patient's clinical symptoms, medical history, auxiliary examinations, treatment plans, etc., finally, it was comprehensively considered that the TOAST classification of this patient was of the other cause type. The source of thrombi may be undifferentiated pleomorphic sarcoma tissue of the heart, thrombi caused by the rough surface of undifferentiated pleomorphic sarcoma of the heart, or it may be a hypercoagulable state of blood caused by tumors. Drug treatment: Warfarin anticoagulant therapy was ineffective and cerebral embolism recurred repeatedly. Three cerebral embolism events occurred in a short period of time, and two intracranial endovascular interventional treatments were performed. The patient's life was once saved, and the condition improved in the short term. However, in the end, the patient survived for more than three months due to multiple cerebral embolisms. This case indicates that intracranial endovascular interventional surgery is an effective treatment measure for patients with cardiac tumors after acute large vessel embolism events, but it cannot improve the prognosis of patients. The fundamental treatment measure still lies in the resection of cardiac tumors. As for how long after acute macrovascular events cardiac tumor surgery is performed, there are currently no literature reports. Considering that this patient experienced multiple cerebral embolism events in a short period of time, whether cardiac surgery can be performed earlier and when it can be done still requires more research. Declarations Data availability ： The case report materials are genuine and available. Acknowledggments We are grateful to all colleagues of Xi 'an Gaoxin Hospital. Author information Tian Nana drafted the manuscript as the first author. Dai Xiaojie was regarded as a co-first author and made the same contribution to this work. Zhang Hongxing also participated in this work and should be regarded as a co-second author. The corresponding authors, Wu Tao and Jia Yi, supervised this work. Consent to publish All participating authors agree to publish. Competing interests The author(s) declare no competing interests. Ethical Approval and accordance Ethical Review Statement for Case Report The article intended for publication by the corresponding author is a case report, titled：“A case of primary undifferentiated pleomorphic sarcoma of the heart complicated by multiple cerebral embolisms and literature review ”. I hereby pledge: When the author uses information related to clinical patients, they will strictly adhere to the World Medical Association's 'Declaration of Geneva: Physician's Oath,' the 'Declaration of Helsinki,' the 'International Code of Medical Ethics,' as well as China's 'Civil Code,' 'Personal Information Protection Law,' 'Basic Medical and Health Promotion Law,' and 'Law of the Practicing Physician,' respecting patients' wishes, protecting patient privacy, not disclosing patients' personal identity or genetic information, and safeguarding patients' rights and interests. All relevant information about the patient mentioned in this case report has been obtained with the patient's informed consent, and a consent form has been signed. The authors will make every effort to protect the patient's personal information and privacy in accordance with relevant laws and regulations. Corresponding author of the article: Wu Tao December 9, 2025 Review Comments: According to international ethical guidelines and the laws and regulations of our country, case reports do not constitute clinical research and do not require ethical review. Xi'an Gaoxin Hospital Medical Ethics Committee December 9, 2025 Funding statement This article has received no funding or any other form of financial support. Clinical trial number Not applicable. Consent to participate The clinical data involved in this article are all from deceased patients. Before publishing the article, the author confirmed that he had obtained the informed consent form (participation consent form and publication consent form) from the patient's son. Data availability statement All data generated or analysed during this study are included in this published article. References Liu Bo et al." A Case of Primary undifferentiated pleomorphic sarcoma of the heart with multiple metastases to the lungs." Chinese Journal of Circulation 000.012(2014):963-963. Yan Li, Liu Lili, Li Xingong. "WHO(2015) Introduction to the Histological Classification of Cardiac Tumors." Journal of Diagnostic Pathology 011(2016):023. Chen, Shiqi, et al. "Undifferentiated Pleomorphic Sarcoma: Long-Term Follow-Up from a Large Institution." Cancer Management and Research 11(2019). R. O. Escárcega, D. Bailey, and M. Defrain. "Multimodality Imaging for the Evaluation of an Undifferentiated Pleomorphic Sarcoma Presenting as Cardioembolic Stroke." Case Reports in Cardiology 2022(2022). Zhu Yuanyuan, et al." Analysis of Clinical and Imaging Features of Primary Cardiac Angiosarcoma." Chinese Journal of Cardiovascular Diseases 49.4(2021):6. 18F-FDG PET/CT in left atrial undifferentiated pleomorphic sarcoma with osteosarcomatous differentiation. Yoshida, Motoshi, et al. "A Case of Cardiac Undifferentiated Pleomorphic Sarcoma With Right Ventricular Outflow Tract Obstruction. " International heart journal 64 4(2023), 779-782. National Health Commission Stroke Prevention and Control Expert Committee, Atrial Fibrillation Stroke Prevention and Control Professional Committee, Chinese Society of Electrophysiology and Pacing, Chinese Medical Association, Arrhythmia Professional Committee, Chinese Medical Doctor Association." Chinese Guidelines for the Prevention and Control of Cardiogenic Stroke (2019)." Chinese Journal of Arrhythmia 23.6(2019):463-484. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-8016932","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":573467381,"identity":"7ae9d6cc-9d34-4003-8f43-508f7ecb0518","order_by":0,"name":"Nana Tian","email":"","orcid":"","institution":"Xi 'an Gaoxin Hospital","correspondingAuthor":false,"prefix":"","firstName":"Nana","middleName":"","lastName":"Tian","suffix":""},{"id":573467383,"identity":"404a0d48-4322-444b-aa27-2c23c32cd1a2","order_by":1,"name":"Xiaojie Dai","email":"","orcid":"","institution":"Xi 'an Gaoxin 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11:41:15","extension":"xml","order_by":31,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":41288,"visible":true,"origin":"","legend":"","description":"","filename":"826f8ed468b34d778a330c41893b6ddd1structuring.xml","url":"https://assets-eu.researchsquare.com/files/rs-8016932/v1/ada6527b16500c7d6a38b796.xml"},{"id":100396757,"identity":"0f5d22d2-959d-4a1e-91d0-341e3fe6680c","added_by":"auto","created_at":"2026-01-16 11:41:05","extension":"html","order_by":32,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":50770,"visible":true,"origin":"","legend":"","description":"","filename":"earlyproof.html","url":"https://assets-eu.researchsquare.com/files/rs-8016932/v1/27f1f3e03ec7a94317b7a131.html"},{"id":100406526,"identity":"fe863eaf-9f82-408a-884e-38637fdbf478","added_by":"auto","created_at":"2026-01-16 13:02:52","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":596411,"visible":true,"origin":"","legend":"\u003cp\u003ea：Cardiac ultrasound result: Space-occupying lesion in the left atrium (possible myxoma)\u003c/p\u003e\n\u003cp\u003eb：Cardiac ultrasound result: Space-occupying lesion in the left atrium (possible myxoma)\u003c/p\u003e\n\u003cp\u003ec: Pathological tissue of cardiac malignant tumor resection and left atrial thrombus removal\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-8016932/v1/75b50c9e12a4ae190ba4a51d.png"},{"id":100406570,"identity":"be1ba06e-cf86-4f9c-a104-ef28c651bba2","added_by":"auto","created_at":"2026-01-16 13:03:01","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":756716,"visible":true,"origin":"","legend":"\u003cp\u003ea:HE staining, magnification 10X4, pathological diagnosis: undifferentiated pleomorphic sarcoma.\u003c/p\u003e\n\u003cp\u003ePathological section F72718X10\u003c/p\u003e\n\u003cp\u003eb :HE staining, magnification 10X10, pathological diagnosis: undifferentiated pleomorphic sarcoma.\u003c/p\u003e\n\u003cp\u003ePathological section F72718X10\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-8016932/v1/dcfd0ccce68fb3f14d189493.png"},{"id":100396903,"identity":"82c4cf12-78eb-4234-8626-b25c59f868af","added_by":"auto","created_at":"2026-01-16 11:41:16","extension":"jpg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":20894,"visible":true,"origin":"","legend":"\u003cp\u003eHead CT: Low-density shadow in the left basal ganglia region\u003c/p\u003e","description":"","filename":"3.jpg","url":"https://assets-eu.researchsquare.com/files/rs-8016932/v1/35a73c70b67f6529f4c29c22.jpg"},{"id":100396657,"identity":"cddf05a3-a619-4ae9-84af-a80b486df6eb","added_by":"auto","created_at":"2026-01-16 11:40:56","extension":"png","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":289350,"visible":true,"origin":"","legend":"\u003cp\u003ea: Thrombectomy through the left middle cerebral artery catheter + thrombectomy with a left middle cerebral artery stent\u003c/p\u003e\n\u003cp\u003eb: Thrombectomy through the left middle cerebral artery catheter + thrombectomy with a left middle cerebral artery stent\u003c/p\u003e","description":"","filename":"4.png","url":"https://assets-eu.researchsquare.com/files/rs-8016932/v1/3b595d9d39372c37332ece60.png"},{"id":100396515,"identity":"e3403747-d552-4a7f-894c-16e05c75799a","added_by":"auto","created_at":"2026-01-16 11:40:46","extension":"jpg","order_by":5,"title":"Figure 5","display":"","copyAsset":false,"role":"figure","size":18661,"visible":true,"origin":"","legend":"\u003cp\u003eHead CT: A new low-density shadow is added in the left frontal lobe\u003c/p\u003e","description":"","filename":"5.jpg","url":"https://assets-eu.researchsquare.com/files/rs-8016932/v1/80045d0139ef030ae1909b6e.jpg"},{"id":100396030,"identity":"2697b097-0d1b-4a90-970b-1c47af5dd505","added_by":"auto","created_at":"2026-01-16 11:39:47","extension":"jpg","order_by":6,"title":"Figure 6","display":"","copyAsset":false,"role":"figure","size":23290,"visible":true,"origin":"","legend":"\u003cp\u003eHead CTA: Occlusion of the right internal carotid artery\u003c/p\u003e","description":"","filename":"6.jpg","url":"https://assets-eu.researchsquare.com/files/rs-8016932/v1/8db01e397d15bfa3af1c3f54.jpg"},{"id":100397129,"identity":"6d5db1bb-fdce-4b1f-879d-e2aa1639c225","added_by":"auto","created_at":"2026-01-16 11:41:34","extension":"png","order_by":7,"title":"Figure 7","display":"","copyAsset":false,"role":"figure","size":179119,"visible":true,"origin":"","legend":"\u003cp\u003ea：Intracranial vascular recanalization: The right internal carotid artery is completely occluded beyond the ophthalmic artery, with a large embolus load. After thrombectomy using stent technique, the right middle cerebral artery beyond M1 is not recanalized, while the right anterior and posterior communicating arteries are partially recanalized. The postoperative blood flow grade of the LMCA is 2a\u003c/p\u003e\n\u003cp\u003eb：Intracranial vascular recanalization: The right internal carotid artery is completely occluded beyond the ophthalmic artery, with a large embolus load. After thrombectomy using stent technique, the right middle cerebral artery beyond M1 is not recanalized, while the right anterior and posterior communicating arteries are partially recanalized. The postoperative blood flow grade of the LMCA is 2a\u003c/p\u003e\n\u003cp\u003ec：Intracranial vascular recanalization: The right internal carotid artery is completely occluded beyond the ophthalmic artery, with a large embolus load. After thrombectomy using stent technique, the right middle cerebral artery beyond M1 is not recanalized, while the right anterior and posterior communicating arteries are partially recanalized. The postoperative blood flow grade of the LMCA is 2a\u003c/p\u003e","description":"","filename":"7.png","url":"https://assets-eu.researchsquare.com/files/rs-8016932/v1/dca5112808a4657832d0d176.png"},{"id":100396604,"identity":"6ee44f2e-99d9-404a-b8c4-79278633ca47","added_by":"auto","created_at":"2026-01-16 11:40:54","extension":"jpg","order_by":8,"title":"Figure 8","display":"","copyAsset":false,"role":"figure","size":13278,"visible":true,"origin":"","legend":"\u003cp\u003eHead CT on the 13th day: A low-density shadow was newly added in the right cerebral hemisphere\u003c/p\u003e","description":"","filename":"8.jpg","url":"https://assets-eu.researchsquare.com/files/rs-8016932/v1/bab347aa01211d974491ca57.jpg"},{"id":105728067,"identity":"e1207b47-9f61-4bfa-8357-82f08ec6789a","added_by":"auto","created_at":"2026-03-30 11:09:03","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":2820966,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-8016932/v1/ac8f198d-c479-42db-a853-02708e2f8aa9.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"A case of primary undifferentiated pleomorphic sarcoma of the heart complicated by multiple cerebral embolisms and literature review","fulltext":[{"header":"Case information","content":"\u003cp\u003eA 56-year-old female was admitted to the hospital due to sudden weakness in the right limb and slurred speech for more than 2 hours. At symptom onset, the right limb was completely immobile. Physical examination: bilateral cervical lymph nodes were enlarged, multiple masses could be palpated in bilateral thyroid glands. The heart rhythm was regular, the first heart sound was normal, A2\u0026gt;P2, and lasag-like murmurs during systolic and diastolic periods could be heard in the mitral valve auscultation area. Neurological physical examination: Consciousness was clear, articulation was unclear, right-handed, right central facial and lingual palsy, bilateral pharyngeal reflexes were weakened, the right limb hemiplegia test was positive, the right finger-nose test and the calcineal-knee-tibial test was not stable and accurate, bilateral pathological signs were not elicited, and no positive localization signs were found in the remaining neurological physical examination. NIHSS score: 7 points.\u003c/p\u003e\n\u003cp\u003e\u003cbr\u003e\u003c/p\u003e"},{"header":"Past medical history","content":"\u003cp\u003eOne year ago, the patient experienced chest tightness and shortness of breath after activities. Color Doppler ultrasound examination of the heart before August indicated a space-occupying lesion in the left atrium (possibly myxoma), EF74% (see Fig 1a and1b).Subsequently, cardiac malignant tumor resection + left atrial thrombus evacuation surgery was performed. During the operation, there was a dark red irregular mass of 50mm×40mm×40mm in the left atrium(see Fig 1c). The pedicle of the mass was wide and located at the foramen ovale of the atrial septum. Histopathological examination confirmed an undifferentiated pleomorphic sarcoma of the left atrium (see Fig 2a and 2b). After the operation, warfarin sodium tablets were taken for anticoagulation treatment due to atrial fibrillation. Seven months ago, after cardioversion with electrical defibrillation, warfarin sodium tablets were discontinued for anticoagulation therapy. Four months before hospitalization, the patient once again presented with symptoms of chest tightness and shortness of breath. Whole-body PET-CT and contrast-enhanced CT revealed metastases in the pulmonary artery, pulmonary vein, pericardium, inferior vena cava, mediastinum, bilateral adrenal glands and renal vein. Three months before hospitalization, color Doppler ultrasound of the heart revealed tumor recurrence. Pembrolizumab injection + anlotinib were administered for treatment. After the treatment, the volume of the cardiac tumor gradually shrank, and the symptoms of chest tightness and shortness of breath gradually alleviated.\u003c/p\u003e\n\u003cp\u003e\u003cbr\u003e\u003c/p\u003e"},{"header":"Admission diagnosis","content":"\u003cp\u003e1. Cerebral infarction (TOAST classification: Cardiogenic embolic type); 2. Postoperative recurrence of undifferentiated pleomorphic sarcoma in the left atrium accompanied by multiple metastases. \u003cstrong\u003eAuxiliary examinations:\u003c/strong\u003e Blood routine, blood lipid, and fasting blood glucose were approximately normal. Coagulation: APTT 43.40 s,FIB 662.0mg/dl. No abnormalities were found in the remaining biochemical tests. Electrocardiogram: Sinus rhythm, myocardial injury; Color Doppler ultrasound of the heart: EF60%. A low echo of 20mm×15mm can be seen on the atrial septal side of the left atrium. It swings with the heart cycle. The left ventricular systolic function is normal. Mitral regurgitation (low to moderate).The head CT examination in the emergency department showed no bleeding. Due to the postoperative surgery for undifferentiated pleomorphic sarcoma of the left atrium, the residual metal substances in the body could not be used for magnetic resonance examination.\u0026nbsp;\u003c/p\u003e"},{"header":"Treatment plan","content":"\u003cp\u003eConsidering that the symptoms of the patient at the onset of the disease were that the right limb was at its most severe and completely immobile, the weakness of the right limb had been relieved by the time of admission, and the right limb paraplegia test was positive. Although the time from the onset to admission was within the time window of intravenous thrombolysis, the undifferentiated pleomorphic sarcoma in the left atrium of the patient recurred and had multiple metastases. Whether there was intracranial metastasis could not be ruled out (intracranial tumors are contraindications for intravenous thrombolysis). Therefore, intravenous thrombolytic therapy was not performed. Considering the patient's clinical symptoms and the absence of indications for emergency intracranial vascular intervention, and taking all the above into account, as well as informing the patient's family of the condition and having them agree and sign the informed consent form, 5,000 U of low molecular weight heparin sodium was administered each time, with anticoagulant therapy every 12 hours. Two days after admission, a re-examination of the head CT scan indicated a low-density shadow in the left basal ganglia region (see Figure 3). Three days after admission, warfarin sodium tablets were added for anticoagulation treatment, and low-molecular-weight heparin sodium was gradually discontinued. In the later stage, warfarin alone was used for anticoagulation treatment.Treatment plan for primary undifferentiated pleomorphic sarcoma of the heart: The cardiac surgery consultation suggests continuing targeted drug therapy and temporarily not considering surgical treatment.\u003c/p\u003e\n\u003cp\u003e\u003cbr\u003e\u003c/p\u003e"},{"header":"Changes in the condition and treatment plan","content":"\u003cp\u003eOn the 10th day after admission, the patient suddenly experienced weakness in the right limb accompanied by speech impairment again. Neurological examination: Clear consciousness, complete aphasia, both eyes staring to the left, the right nasolabial sulcus is shallower than the left one, tongue protruding to the right, right upper limb muscle strength grade 0, right lower limb muscle strength grade 2, positive right Babinski sign. The NIHSS score was 12 points (gaze 1 point + facial paralysis 1 point + right upper limb muscle strength 4 points + right lower limb muscle strength 3 points + speech 2 points + articulation 1 point). Urgent head CT examination: Cerebral infarction in the left basal ganglia region. Considering the recurrence of acute cerebral embolism, emergency general anesthesia was used to perform thrombectomy through the left middle cerebral artery catheter and thrombectomy with a left middle cerebral artery stent(see Fig 4a, b), and the postoperative blood flow grade of LMCA was 2B. The results of the re-examination of the head CT on the 12th day of admission: a new low-density shadow was added in the left frontal lobe (see Fig5). Neurological examination of the patient: Clear consciousness, poor comprehension, no speech, right central facial and lingual paralysis, right limb muscle strength grade 4, positive right Babinski sign. The NIHSS score is 7.On the 12th day after admission, the patient suddenly experienced consciousness disorders again, aphasia, staring to the right with both eyes, unequal pupils on both sides, 3.5mm on the left side and 3.0mm on the right side, sensitive response to light, no muscle strength of grade 0 in the left limb, muscle strength of grade 2 in the right limb, positive bilateral Babinski sign, and a NIHSS score of 16 points. Emergency head and neck CTA examination indicated occlusion of the right internal carotid artery (see Fig 6). Intracranial angioplasty was performed again. During the operation, it was found that the right internal carotid artery was completely occlusive beyond the ophthalmic artery, with a large embolus load. After thrombectomy using stent technology, the right middle cerebral artery beyond M1 was not recanalized, and the right anterior and posterior communicating arteries were partially recanalized. The postoperative blood flow grade of LMCA was 2a(see Fig 7)。Head CT on the 13th day: A low-density shadow was newly added in the right cerebral hemisphere (see Fig 8).\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cbr\u003e\u003c/p\u003e"},{"header":"Adjustment of the treatment plan","content":"\u003cp\u003eConsidering that the emboli of the patient's acute cerebral embolism originated from undifferentiated pleomorphic sarcoma tissue of the heart rather than thrombus, warfarin sodium tablets were discontinued for treatment. After the operation, the patient underwent tracheotomy and mechanical ventilation, presenting a dermal state, with unconscious eye opening. The bilateral pupils were of equal size and round, with a diameter of 2.5mm. The patient was sensitive to light reflex, and the pain stimulated flexure and adduction of both upper limbs and hyperextension of both lower limbs. Positive bilateral Babinski sign. The patient underwent tracheal intubation and ventilator-assisted ventilation after the operation. Ultimately, the patient died after being admitted to the hospital for more than three months due to repeated cerebral embolism, which led to limb paralysis and long-term bedridden status.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cbr\u003e\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003ePrimary cardiac tumors are extremely rare, with a detection rate ranging from 0.0017% to 0.33%, and 90% of them are benign [1, 2]. Primary malignant cardiac tumors account for only 10% of primary cardiac tumors, among which undifferentiated pleomorphic sarcomas make up 10%[2]. The most malignant one is the undifferentiated sarcoma. Undifferentiated sarcoma refers to a general term for a group of sarcomas whose tissue types cannot be determined by gross, microscopic or immunohistochemical methods [1].\u0026nbsp;Patients with primary undifferentiated pleomorphic sarcoma of the heart have a rapid disease progression. Studies have shown that the local recurrence rate and metastasis rate are 13-42% and 31-35%, respectively [3], with a poor prognosis and an average survival period of only one year [1].\u0026nbsp;The main clinical symptoms depend on the size and location of the tumor, such as sudden death, embolism, obstruction and arrhythmia, etc. [2] Some patients may have no symptoms of the heart and first seek medical attention due to embolic events [4].\u0026nbsp;Auxiliary examinations include echocardiography, CT, cardiac MRI, PET-CT, etc. Cardiac MRI is superior to echocardiography and CT in differentiating the nature of soft tissues, distinguishing heart tissues, thrombosis, and benign/malignant lesions [5]. PET-CT is helpful in differentiating benign and malignant space-occupying lesions in the heart or pericardium. Literature reports that when the maximum SUV value is \u0026gt; 3.5, the sensitivity for diagnosing malignant cardiac tumors is 100%[5, 6].\u0026nbsp;Complete surgical resection is the main method for treating sarcoma. The completeness of tumor resection has a significant impact on prognosis. The survival rate of patients with negative tumor resection margins was significantly higher than that of patients with positive tumor resection margins. The combined treatment consisting of surgical resection and postoperative chemotherapy is associated with a better survival rate of patients [7].\u0026nbsp;Cardiogenic stroke is defined as the detachment of cardiogenic emboli, which leads to the blockage of the corresponding cerebral arteries and eventually results in ischemic stroke. It accounts for 14% - 30% of all ischemic strokes [8].\u0026nbsp;Cardiogenic stroke is closely related to various cardiovascular diseases. The most common high-risk factors include atrial fibrillation, recent (within 4 weeks) myocardial infarction, artificial mechanical valves, dilated cardiomyopathy, rheumatic mitral stenosis, etc., followed by infectious and non-infectious endocarditis, atrial myxoma, etc[8]\u0026nbsp;.There are relatively few reports on cerebral embolism caused by primary undifferentiated pleomorphic sarcoma of the heart. Such cardiac embolic fragments can be substances of the tumor itself or thrombotic substances formed on the tumor surface when the tumor is exposed to blood flow [4].\u0026nbsp;There is no sufficient evidence to suggest that anticoagulation can reduce the incidence of embolic events in cardiac myxomas and other cardiac tumors. Therefore, there is currently no unified treatment opinion for patients with cerebral embolism caused by primary undifferentiated pleomorphic sarcoma of the heart [4].\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eCombined with the patient's clinical symptoms, medical history, auxiliary examinations, treatment plans, etc., finally, it was comprehensively considered that the TOAST classification of this patient was of the other cause type. The source of thrombi may be undifferentiated pleomorphic sarcoma tissue of the heart, thrombi caused by the rough surface of undifferentiated pleomorphic sarcoma of the heart, or it may be a hypercoagulable state of blood caused by tumors. Drug treatment: Warfarin anticoagulant therapy was ineffective and cerebral embolism recurred repeatedly. Three cerebral embolism events occurred in a short period of time, and two intracranial endovascular interventional treatments were performed. The patient's life was once saved, and the condition improved in the short term. However, in the end, the patient survived for more than three months due to multiple cerebral embolisms. This case indicates that intracranial endovascular interventional surgery is an effective treatment measure for patients with cardiac tumors after acute large vessel embolism events, but it cannot improve the prognosis of patients. The fundamental treatment measure still lies in the resection of cardiac tumors. As for how long after acute macrovascular events cardiac tumor surgery is performed, there are currently no literature reports. Considering that this patient experienced multiple cerebral embolism events in a short period of time, whether cardiac surgery can be performed earlier and when it can be done still requires more research.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eData availability\u003c/strong\u003e\u003cstrong\u003e：\u003c/strong\u003eThe case report materials are genuine and available.\u003c/p\u003e\u003cp\u003e\u003cstrong\u003eAcknowledggments\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWe are grateful to all colleagues of Xi 'an Gaoxin Hospital.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthor information\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eTian Nana drafted the manuscript as the first author. Dai Xiaojie was regarded as a co-first author and made the same contribution to this work. Zhang Hongxing also participated in this work and should be regarded as a co-second author. The corresponding authors, Wu Tao and Jia Yi, supervised this work.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent to publish\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll participating authors agree to publish.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe author(s) declare no competing interests.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthical Approval and accordance\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthical Review Statement for Case Report\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe article intended for publication by the corresponding author is a case report, titled：“A case of primary undifferentiated pleomorphic sarcoma of the heart complicated by multiple cerebral embolisms and literature review ”. I hereby pledge: When the author uses information related to clinical patients, they will strictly adhere to the World Medical Association's 'Declaration of Geneva: Physician's Oath,' the 'Declaration of Helsinki,' the 'International Code of Medical Ethics,' as well as China's 'Civil Code,' 'Personal Information Protection Law,' 'Basic Medical and Health Promotion Law,' and 'Law of the Practicing Physician,' respecting patients' wishes, protecting patient privacy, not disclosing patients' personal identity or genetic information, and safeguarding patients' rights and interests. All relevant information about the patient mentioned in this case report has been obtained with the patient's informed consent, and a consent form has been signed. The authors will make every effort to protect the patient's personal information and privacy in accordance with relevant laws and regulations.\u003c/p\u003e\n\u003cp\u003eCorresponding author of the article: Wu Tao\u003c/p\u003e\n\u003cp\u003eDecember 9, 2025\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eReview Comments:\u0026nbsp;\u003c/strong\u003eAccording to international ethical guidelines and the laws and regulations of our country, case reports do not constitute clinical research and do not require ethical review.\u003c/p\u003e\n\u003cp\u003eXi'an Gaoxin Hospital Medical Ethics Committee\u003c/p\u003e\n\u003cp\u003eDecember 9, 2025\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding statement\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis article has received no funding or any other form of financial support.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eClinical trial number\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent to participate\u003c/strong\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe clinical data involved in this article are all from deceased patients. Before publishing the article, the author confirmed that he had obtained the informed consent form (participation consent form and publication consent form) from the patient's son.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eData availability statement\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll data generated or analysed during this study are included in this published article.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003e Liu Bo et al.\" A Case of Primary undifferentiated pleomorphic sarcoma of the heart with multiple metastases to the lungs.\" Chinese Journal of Circulation 000.012(2014):963-963.\u003c/li\u003e\n\u003cli\u003e Yan Li, Liu Lili, Li Xingong. \"WHO(2015) Introduction to the Histological Classification of Cardiac Tumors.\" Journal of Diagnostic Pathology 011(2016):023.\u003c/li\u003e\n\u003cli\u003eChen, Shiqi, et al. \"Undifferentiated Pleomorphic Sarcoma: Long-Term Follow-Up from a Large Institution.\"\u0026nbsp;Cancer Management and Research\u0026nbsp;11(2019).\u003c/li\u003e\n\u003cli\u003eR. O. Esc\u0026aacute;rcega, D. Bailey, and M. Defrain. \"Multimodality Imaging for the Evaluation of an Undifferentiated Pleomorphic Sarcoma Presenting as Cardioembolic Stroke.\" Case Reports in Cardiology 2022(2022).\u003c/li\u003e\n\u003cli\u003e Zhu Yuanyuan, et al.\" Analysis of Clinical and Imaging Features of Primary Cardiac Angiosarcoma.\" Chinese Journal of Cardiovascular Diseases 49.4(2021):6.\u003c/li\u003e\n\u003cli\u003e 18F-FDG PET/CT in left atrial undifferentiated pleomorphic sarcoma with osteosarcomatous differentiation.\u003c/li\u003e\n\u003cli\u003eYoshida, Motoshi, et al. \"A Case of Cardiac Undifferentiated Pleomorphic Sarcoma With Right Ventricular Outflow Tract Obstruction. \"\u0026nbsp;International heart journal\u0026nbsp;64 4(2023), 779-782.\u003c/li\u003e\n\u003cli\u003eNational Health Commission Stroke Prevention and Control Expert Committee, Atrial Fibrillation Stroke Prevention and Control Professional Committee, Chinese Society of Electrophysiology and Pacing, Chinese Medical Association, Arrhythmia Professional Committee, Chinese Medical Doctor Association.\" Chinese Guidelines for the Prevention and Control of Cardiogenic Stroke (2019).\" Chinese Journal of Arrhythmia 23.6(2019):463-484.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Primary undifferentiated pleomorphic sarcoma of the heart, Cerebral embolism","lastPublishedDoi":"10.21203/rs.3.rs-8016932/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-8016932/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003ePrimary cardiac undifferentiated pleomorphic sarcoma is extremely rare and associated with many complications and poor prognosis. This report hereby presents the diagnosis and treatment process of a patient with primary undifferentiated pleomorphic sarcoma of the heart complicated by multiple cerebral embolisms, with the aim of enhancing clinicians' understanding of primary undifferentiated pleomorphic sarcoma of the heart complicated by cerebral embolism.\u003c/p\u003e","manuscriptTitle":"A case of primary undifferentiated pleomorphic sarcoma of the heart complicated by multiple cerebral embolisms and literature review","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2026-01-16 08:26:58","doi":"10.21203/rs.3.rs-8016932/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"f009f209-80b8-470f-9cf1-9dc76099ce50","owner":[],"postedDate":"January 16th, 2026","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2026-03-26T06:56:34+00:00","versionOfRecord":[],"versionCreatedAt":"2026-01-16 08:26:58","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-8016932","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-8016932","identity":"rs-8016932","version":["v1"]},"buildId":"XKTyCvWXoU3ODBz1xrDgd","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}