Unique Case of Primary Malignant Fibrous Histiocytoma of the Right Ventricle with Moderator Band Involvement
AbstractMalignant primary tumors of the heart are very rare and in most cases are located in the left side of the heart, but involvement of the moderator band of the right heart has not yet been described in the literature. We report the case of a 22-year-old woman who presented after an episode of transient sudden loss of consciousness. The transthoracic echocardiography (TTE) and transesophageal echocardiography evaluations showed an echogenic mass that originated in the right ventricle and protruded into the tricuspid valve during systole. While operating on the patient, we found a sessile tumor that originated exclusively from the moderator band of the right ventricle. The tumor was completely resected through the tricuspid valve. An immunohistochemistry examination of the tumor confirmed the diagnosis of a malignant fibrous histiocytoma (MFH) (undifferentiated pleomorphic sarcoma). To our knowledge, this case is the first of moderator band involvement among the 50 cases of primary MFH of the heart that have been reported in the literature. After a year and a half, TTE, computed tomography (CT), and positron emission tomography CT (PET-CT) showed the absence of cardiac tumor, a normal tricuspid function, and no metastasis. The completeness of the surgical resection and the absence of local recurrence >1 year after surgery are encouraging signs for the patient's outcome.
Alam M, Rosman HS, Grullon C. 1995. Transesophageal echocardiography in evaluation of atrial masses. Angiology 46:123-8.nBear PA, Moodie DS. 1987. Malignant primary cardiac tumors. The Cleveland Clinic experience, 1956 to 1986. Chest 92:860-2.nBurke AP, Cowan D, Virmani R. 1992. Primary sarcomas of the heart. Cancer 69:3857-95.nBurke AP, Virmani R. 1995. Tumor of the heart and great vessels. In: Atlas of tumor pathology. 3rd series. Fascicle 16. Washington, DC: Armed Forces Institute of Pathology. p 1-11.nBurke AP, Tazelaar H, Butany JW, et al. 2004. Cardiac sarcomas. In: Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC, eds. Pathology and genetics of tumours of the lung, pleura, thymus and heart. Lyon, France: IARC Press. p 276-7.nDonsbeck AV, Ranchere D, Coindre JM, Le Gall F, Cordier J, Loire R. 1999. Primary cardiac sarcomas: an immunohistochemical and grading study with long-term follow-up of 24 cases. Histopathology 34:295-304.nEdwards LC 3rd, Louie EK. 1994. Transthoracic and transesophageal echocardiography for the evaluation of cardiac tumors, thrombi, and valvular vegetations. Am J Card Imaging 8:45-58.nFujio A, Kitano M, Asakura S, Matsui T. 1986. A case of malignant histiocytoma of the epicardium [in Japanese with English abstract]. Nippon Kyobu Geka Gakkai Zasshi 34:267-71.nKern SE, Cowen ME, Abrams GD. 1985. Malignant fibrous histiocytoma of the heart presenting as unilateral pulmonary thromboembolism and infarct. Hum Pathol 16:1279-81.nKim J, Baek W, Kim K, Yoon Y, Kim D, Lim H. 2003. A primary cardiac sarcoma preoperatively presented as a benign left atrial myxoma. Chest 44:530-3.nLam KY, Dickens P, Chan AC. 1993. Tumors of the heart: a 20-year experience with a review of 12,485 consecutive autopsies. Arch Pathol Lab Med 117:1027-31.nLlombart-Cussac A, Pivot X, Contesso G, et al. 1998. Adjuvant chemotherapy for primary cardiac sarcomas: the IGR experience. Br J Cancer 78:1624-8.nMatsukuma S, Yamaguchi H, Hamawaki M, Ito M, Matsuoka Y. 2008. Primary pericardial malignant fibrous histiocytoma causing cardiac tamponade. Ann Thorac Surg 86:646-9.nMeng Q, Lai H, Lima J, Tong W, Qian Y, Lai S. 2002. Echocardiographic and pathologic characteristics of primary cardiac tumors: a study of 149 cases. Int J Cardiol 84:69-75.nMilicic D, Juretic A, Bulum J, et al. 2007. Primary malignant fibrous histiocytoma of the heart with skeletal muscles metastases. J Card Surg 22:513-6.nNawa S, Tamesue K, Yamamoto H. 1997. Fistula formation from neovascularity developing in malignant fibrous histiocytoma of the heart. Br J Radiol 70:1066-7.nOkamoto K, Kato S, Katsuki S, et al. 2001. Malignant fibrous histiocytoma of the heart: case report and review of 46 cases in the literature. Int Med 40:1222-6.nOzawa A, Iwasaki T, Miyake F, et al. 1997. A case of primary malignant fibrous histiocytoma of the heart with a left-to-right atrial shunt. Jpn Circ J 61:943-6.nPutnam JB, Sweeny MS, Colon R, Lanza LA, Frazier OH, Cooley DA. 1991. Primary cardiac sarcomas. Ann Thorac Surg 51:906-10.nReynen K. 1996. Frequency of primary tumors of the heart. Am J Cardiol 77:107.nSilverman NA. 1980. Primary cardiac tumors. Ann Surg 191:127-38.nTanprasert P, Vidhayasarronyut S, Dharamadhach A, Suchatlampong V. 1981. A rare case of pericardial malignant fibrous histiocytoma. J Med Assoc Thai 64:473-9.nWilk W, Janicki K. 1998. Primary intrapericardial malignant fibrous histiocytoma. A case report. Pol J Pathol 49:43-6.n
How to Cite
Author Disclosure & Copyright Transfer Agreement
In order to publish the original work of another person(s), The Heart Surgery Forum® must receive an acknowledgment of the Author Agreement and Copyright Transfer Statement transferring to Forum Multimedia Publishing, L.L.C., a subsidiary of Carden Jennings Publishing Co., Ltd. the exclusive rights to print and distribute the author(s) work in all media forms. Failure to check Copyright Transfer agreement box below will delay publication of the manuscript.
A current form follows:
The author(s) hereby transfer(s), assign(s), or otherwise convey(s) all copyright ownership of the manuscript submitted to Forum Multimedia Publishing, LLC (Publisher). The copyright transfer covers the exclusive rights to reproduce and distribute the article and the material contained therein throughout the world in all languages and in all media of expression now known or later developed, including but not limited to reprints, photographic reproduction, microfilm, electronic data processing (including programming, storage, and transmission to other electronic data record(s), or any other reproductions of similar nature), and translations.
However, Publisher grants back to the author(s) the following:
- The right to make and distribute copies of all or part of this work for use of the author(s) in teaching;
- The right to use, after publication in The Heart Surgery Forum, all or part of the material from this work in a book by the author(s), or in a collection of work by the author(s);
- The royalty-free right to make copies of this work for internal distribution within the institution/company that employs the author(s) subject to the provisions below for a work-made-for-hire;
- The right to use figures and tables from this work, and up to 250 words of text, for any purpose;
- The right to make oral presentations of material from this work.
Publisher reserves the right to grant or refuse permission to third parties to republish all or part of the article or translations thereof. To republish, such third parties must obtain written permission from the Publisher. (This is in accordance with the Copyright Statute, United States Code, Title 17. Exception: If all authors were bona fide officers or employees of the U.S. Government at the time the paper was prepared, the work is a “work of the US Government” (prepared by an officer or employee of the US Government as part of official duties), and therefore is not subject to US copyright; such exception should be indicated on signature lines. If this work was prepared under US Government contract or grant, the US Government may reproduce, royalty-free, all or portions of this work and may authorize others to do so, for official US Government purposes only, if the US Government contract or grant so requires.
I have participated in the conception and design of this work and in the writing of the manuscript and take public responsibility for it. Neither this manuscript nor one with substantially similar content under my authorship has been published, has been submitted for publication elsewhere, or will be submitted for publication elsewhere while under consideration by The Heart Surgery Forum, except as described in an attachment. I have reviewed this manuscript (original version) and approve its submission. If I am listed above as corresponding author, I will provide all authors with information regarding this manuscript and will obtain their approval before submitting any revision. I attest to the validity, accuracy, and legitimacy of the content of the manuscript and understand that Publisher assumes no responsibility for the validity, accuracy, and legitimacy of its content. I warrant that this manuscript is original with me and that I have full power to make this Agreement. I warrant that it contains no matter that is libelous or otherwise unlawful or that invades individual privacy or infringes any copyright or other proprietary right. I agree to indemnify and hold Publisher harmless of and from any claim made against Publisher that relates to or arises out of the publication of the manuscript and agree that this indemnification shall include payment of all costs and expenses relating to the defense of any such claim, including all reasonable attorney’s fees.
I warrant that I have no financial interest in the drugs, devices, or procedures described in the manuscript (except as disclosed in the attached statement).
I state that the institutional Human Subjects Committee and/or the Ethics Committee approved the clinical protocol reported in this manuscript for the use of experimental techniques, drugs, or devices in human subjects and appropriate informed consent documents were utilized.
Furthermore, I state that any and all animals used for experimental purposes received humane care in USDA registered facilities in compliance with the “Principles of Laboratory Animal Care” formulated by the National Society for Medical Research and the “Guide for the Care and Use of Laboratory Animals” prepared by the Institute of Laboratory Animal Resources and published by the National Institutes of Health (NIH Publication No. 85-23, revised 1985).