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Huo X, Zhao W, Liu X, Zhang W, Xu L, Zhang B, Chen J. Ultrasonography in the assessment of primary myxofibrosarcoma in the left atrium: a case report. BMC Cardiovasc Disord 2022; 22:553. [PMID: 36536277 PMCID: PMC9764716 DOI: 10.1186/s12872-022-03009-6] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/07/2022] [Accepted: 12/12/2022] [Indexed: 12/24/2022] Open
Abstract
BACKGROUND Cardiac myxofibrosarcoma is a rare cardiac malignant tumor, whose diagnosis is challenging due to its rare and non-specific manifestations. Ultrasound is the most important tool for detecting cardiac tumors. Yet, its diagnostic value in cardiac myxoidfibrosarcoma is rarely reported. Herein, we summarized the ultrasonic manifestations of myxofibrosarcoma in a 72-year-old Han woman. CASE PRESENTATION The patient presented with crushing chest pain without obvious inducement, lasting 3-5 min each time, which would be relieved after rest, accompanied by palpitation, chest tightness, shortness of breath, dizziness, and syncope. The electrocardiogram (ECG) suggested atrial fibrillation. Ultrasound found two moderate echogenic masses in the left atrium; one was about 48 × 31 mm in size, and the other was about 25 × 24 mm in size. The clinical diagnosis was atrial mass and atrial fibrillation. The patient underwent the operation of left atrial tumor resection + mitral valvuloplasty + atrial fibrillation radiofrequency ablation + left atrial appendectomy. The tumor was completely removed, and the patient did not receive radiotherapy or chemotherapy after surgery. The patient was reexamined by ultrasound at 6, 42, and 91 days after surgery, and no obvious abnormalities were found. On day 115, moderate echoic mass was detected on the posterior wall of the left atrium, nearing the mitral valve ring, with a size of about 28 × 23 mm. Currently, the patient is under follow-up care. CONCLUSION As the most important examination method for cardiac tumors, cardiac ultrasound has good diagnostic and differential diagnosis value and can be used regularly due to its simplicity and safety. The diagnosis rate of cardiac myxofibrosarcoma can be greatly improved by summarizing the ultrasonographic manifestations of cardiac myxofibrosarcoma and differentiating them from other lesions.
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Affiliation(s)
- Xiaoguang Huo
- grid.477019.cDepartment of Ultrasound, Zibo Central Hospital, No. 54 West Communist Youth League Road, Zhangdian District, Zibo, 255036 Shandong People’s Republic of China
| | - Wei Zhao
- grid.477019.cDepartment of Ultrasound, Zibo Central Hospital, No. 54 West Communist Youth League Road, Zhangdian District, Zibo, 255036 Shandong People’s Republic of China
| | - Xiao Liu
- grid.477019.cDepartment of Ultrasound, Zibo Central Hospital, No. 54 West Communist Youth League Road, Zhangdian District, Zibo, 255036 Shandong People’s Republic of China
| | - Wenzhong Zhang
- grid.477019.cDepartment of Ultrasound, Zibo Central Hospital, No. 54 West Communist Youth League Road, Zhangdian District, Zibo, 255036 Shandong People’s Republic of China
| | - Li Xu
- grid.477019.cDepartment of Ultrasound, Zibo Central Hospital, No. 54 West Communist Youth League Road, Zhangdian District, Zibo, 255036 Shandong People’s Republic of China
| | - Baohua Zhang
- grid.477019.cDepartment of Pathology, Zibo Central Hospital, No. 54 West Communist Youth League Road, Zhangdian District, Zibo, 255036 Shandong People’s Republic of China
| | - Ju Chen
- grid.477019.cDepartment of Ultrasound, Zibo Central Hospital, No. 54 West Communist Youth League Road, Zhangdian District, Zibo, 255036 Shandong People’s Republic of China
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Reddy KVC, Kumar P, Sanzgiri P, George AM. Primary cardiac myxofibrosarcoma with osteoid differentiation mimicking a left atrial myxoma: A rare entity. J Cardiol Cases 2020; 22:253-256. [PMID: 33133322 DOI: 10.1016/j.jccase.2020.07.013] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/18/2020] [Revised: 06/22/2020] [Accepted: 07/02/2020] [Indexed: 11/16/2022] Open
Abstract
Cardiac myxofibrosarcoma (MFS) is an uncommon entity. It is among the most challenging conditions to diagnose due to its rarity, high variability, and non-specific findings. These tumors often simulate left atrial myxoma or mitral stenosis at clinical presentation. Although, the definitive diagnosis of cardiac tumors depends on histopathological examination, various imaging techniques are also useful to study tumor characteristics to plan an appropriate treatment strategy. Here we highlight a case of primary cardiac MFS of left atrium (LA) showing areas of transition to undifferentiated pleomorphic sarcoma (UPS) with bone or osteoid formation, which is extremely rare and not well described. <Learning objective: Primary cardiac myxofibrosarcoma (MFS) is a rare and aggressive cardiac tumor. It is often confused with benign myxoma, leading to a delay in initiation of treatment. This delay can often lead to poor clinical outcomes. Our study will guide clinicians in early diagnosis, treatment, and counseling of patients with this rare entity. Echocardiography, together with magnetic resonance imaging, histology, and immunohistochemistry are essential in the diagnosis of MFS.>.
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Affiliation(s)
- K V Charan Reddy
- Department of Clinical and Interventional Cardiology, Lilavati Hospital and Research Center, Mumbai, India
| | - P Kumar
- Department of Cardiovascular and Thoracic Surgery, Lilavati Hospital and Research Center, Mumbai, India
| | - P Sanzgiri
- Department of Clinical and Interventional Cardiology, Lilavati Hospital and Research Center, Mumbai, India
| | - A M George
- Department of Histopathology, Lilavati Hospital and Research Center, Mumbai, India
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Saponara M, Indio V, Pizzi C, Serban ED, Urbini M, Astolfi A, Paolisso P, Suarez SM, Nannini M, Pacini D, Agostini V, Leone O, Ambrosini V, Tarantino G, Fanti S, Niro F, Buia F, Attinà D, Pantaleo MA. Successful multidisciplinary clinical approach and molecular characterization by whole transcriptome sequencing of a cardiac myxofibrosarcoma: A case report. World J Clin Cases 2019; 7:3018-3026. [PMID: 31624749 PMCID: PMC6795718 DOI: 10.12998/wjcc.v7.i19.3018] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/26/2019] [Revised: 07/25/2019] [Accepted: 07/27/2019] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Cardiac tumors are rare and complex entities. Surgery represents the cornerstone of therapy, while the role of adjuvant treatment remains unclear and, in case of relapse or metastatic disease, the prognosis is very poor. Lack of prospective, randomized clinical trials hinders the generation of high level evidence for the optimal diagnostic workup and multimodal treatment of cardiac sarcomas. Herein, we describe the multidisciplinary clinical management and molecular characterization of a rare case of cardiac myxofibrosarcoma in an elderly woman.
CASE SUMMARY A 73-year-old woman presented signs and symptoms of acute left-sided heart failure. Imaging examination revealed a large, left atrial mass. With suspicion of a myxoma, she underwent surgery, and symptoms were promptly relieved. Histology showed a cardiac myxofibrosarcoma, a rare histotype of cardiac sarcoma. Eight months later, disease unfortunately relapsed, and after a multidisciplinary discussion, a chemotherapy with doxorubicin and then gemcitabine was started, achieving partial radiologic and complete metabolic response, which was maintained up to 2 years and is still present. This report is focused on the entire clinical path of our patient from diagnosis to follow-up, through surgery and strategies adopted at relapse. Moreover, due to their rarity, very little is known about the molecular landscape of myxofibrosarcomas. Thus, we also performed and described preliminary genome analysis of the tumor tissue to get further insight on mechanisms involved in tumor growth, and to possibly unveil new clinically actionable targets.
CONCLUSION We report a case of cardiac myxofibrosarcoma that achieved a very good prognosis due to an integrated surgical, cardiac and oncologic treatment strategy.
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Affiliation(s)
- Maristella Saponara
- Department of Specialized, Experimental and Diagnostic Medicine, Medical Oncology Unit, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Valentina Indio
- “Giorgio Prodi” Cancer Research Center, University of Bologna, Bologna 40138, Italy
| | - Carmine Pizzi
- Department of Specialized, Experimental and Diagnostic Medicine, Cardiology and Transplantation Unit, Sant’Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Elena-Daniela Serban
- Department of Pathology, Cardiovascular Pathology Unit, Sant’Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Milena Urbini
- “Giorgio Prodi” Cancer Research Center, University of Bologna, Bologna 40138, Italy
| | - Annalisa Astolfi
- “Giorgio Prodi” Cancer Research Center, University of Bologna, Bologna 40138, Italy
| | - Pasquale Paolisso
- Department of Specialized, Experimental and Diagnostic Medicine, Cardiology and Transplantation Unit, Sant’Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Sofia Martin Suarez
- Departments of Cardiovascular Surgery and Transplantation, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Margherita Nannini
- Department of Specialized, Experimental and Diagnostic Medicine, Medical Oncology Unit, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Davide Pacini
- Departments of Cardiovascular Surgery and Transplantation, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Valentina Agostini
- Department of Pathology, Cardiovascular Pathology Unit, Sant’Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Ornella Leone
- Department of Pathology, Cardiovascular Pathology Unit, Sant’Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Valentina Ambrosini
- Department of Specialized, Experimental and Diagnostic Medicine, Nuclear Medicine Unit, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Giuseppe Tarantino
- “Giorgio Prodi” Cancer Research Center, University of Bologna, Bologna 40138, Italy
| | - Stefano Fanti
- Department of Specialized, Experimental and Diagnostic Medicine, Nuclear Medicine Unit, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Fabio Niro
- Department of Specialized, Experimental and Diagnostic Medicine, Radiology Unit, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Francesco Buia
- Department of Specialized, Experimental and Diagnostic Medicine, Radiology Unit, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Domenico Attinà
- Department of Specialized, Experimental and Diagnostic Medicine, Radiology Unit, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Maria Abbondanza Pantaleo
- Department of Specialized, Experimental and Diagnostic Medicine, Medical Oncology Unit, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
- “Giorgio Prodi” Cancer Research Center, University of Bologna, Bologna 40138, Italy
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Ujihira K, Yamada A, Nishioka N, Iba Y, Maruyama R, Nakanishi K, Shimizu A, Hatanaka KC, Mitsuhashi T, Shinohara T, Ueda HI. A case report of primary cardiac myxofibrosarcoma presenting with severe congestive heart failure. J Cardiothorac Surg 2016; 11:95. [PMID: 27387491 PMCID: PMC4936254 DOI: 10.1186/s13019-016-0490-3] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/18/2016] [Accepted: 06/24/2016] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Primary cardiac sarcomas are extremely rare. Furthermore, the myxofibrosarcomas are one of the rarest forms of cardiac sarcomas, and its prognosis is known to be quite poor. CASE PRESENTATION This is a case of a 23-year-old man who presented with acute severe congestive heart failure caused by almost complete obstruction of the mitral valve due to a large left atrial tumor. The patient required endotracheal intubation before his arrival to the hospital, and underwent an emergent surgical excision of the tumor. The tumor had a complex shape and originated from the orifice of the right upper pulmonary vein. Because the tumor seemed to extend over most of the surface of the left atrium, it seemed impossible to reconstruct the left atrium had we done a complete transmural resection. Instead, we carefully peeled the tumor leaving the outer layer of the left atrial wall. We applied cryoablation to the attached site, in order to prevent a recurrence of the tumor. The pathology report revealed that the tumor was a myxofibrosarcoma, and it seemed to originate from the heart. The patient received radiation therapy after the surgery and continues to be alive and well after 1-year, without apparent recurrence. CONCLUSIONS Cardiac myxofibrosarcoma can cause acute, severe left-sided heart failure. Non-transmural atrial wall resection with cryoablation might be effective for patients with cardiac myxofibrosarcomas with extensive atrial attachment.
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Affiliation(s)
- Kosuke Ujihira
- Department of Cardiovascular Surgery, Teine Keijinkai Hospital, 1-12 Maeda Teine-ku, Sapporo, 006-0811, Japan.
| | - Akira Yamada
- Department of Cardiovascular Surgery, Teine Keijinkai Hospital, 1-12 Maeda Teine-ku, Sapporo, 006-0811, Japan
| | - Naritomo Nishioka
- Department of Cardiovascular Surgery, Teine Keijinkai Hospital, 1-12 Maeda Teine-ku, Sapporo, 006-0811, Japan
| | - Yutaka Iba
- Department of Cardiovascular Surgery, Teine Keijinkai Hospital, 1-12 Maeda Teine-ku, Sapporo, 006-0811, Japan
| | - Ryushi Maruyama
- Department of Cardiovascular Surgery, Teine Keijinkai Hospital, 1-12 Maeda Teine-ku, Sapporo, 006-0811, Japan
| | - Katsuhiko Nakanishi
- Department of Cardiovascular Surgery, Teine Keijinkai Hospital, 1-12 Maeda Teine-ku, Sapporo, 006-0811, Japan
| | - Ai Shimizu
- Department of Surgical Pathology, Hokkaido University Hospital, N14W5 Kita-ku, Sapporo, 060-8648, Japan
| | - Kanako C Hatanaka
- Department of Surgical Pathology, Hokkaido University Hospital, N14W5 Kita-ku, Sapporo, 060-8648, Japan
| | - Tomoko Mitsuhashi
- Department of Surgical Pathology, Hokkaido University Hospital, N14W5 Kita-ku, Sapporo, 060-8648, Japan
| | - Toshiya Shinohara
- Department of Pathology, Teine Keijinkai Hospital, 1-12 Maeda Teine-ku, Sapporo, 006-0811, Japan
| | - Hatsue Ishibashi Ueda
- Department of Pathology and Biobank, National Cerebral and Cardiovascular Center, 5-7-1 Fujishirodai, Suita, 565-8565, Japan
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Malignant perivascular epithelioid cardiac sarcomas: a case report and a review of the literature. Case Rep Cardiol 2015; 2015:258140. [PMID: 25977823 PMCID: PMC4421031 DOI: 10.1155/2015/258140] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/24/2015] [Accepted: 04/05/2015] [Indexed: 12/02/2022] Open
Abstract
Cardiac tumors, either benign or malignant, are difficult to diagnose due to their rarity, variety, and nonspecific presentation. Since primary cardiac sarcoma remains an unusual diagnosis, the literature on its presentation, diagnosis, and optimal management remains scarce. To our knowledge the following case of cardiac perivascular epithelioid cell tumor is the fourth reported case found in the literature. Although complete surgical resection remains the gold standard for cardiac sarcomas, our case demonstrates that not all of them can be completely resected.
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