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Mitral valve myxosarcoma Vivek Jawali, M.Ch., Murali Ramanujan Chakravarthy, M.Ch., Srinivasan Kolar, M.Ch., Murali Manohar, M.Ch., Tammanagowda Ainangowda Patil, DA, Jayant Kumar Das, M.Ch., Gilbert Joseph M.Ch., Jayaprakash Krishnamoorthy, MD, Shivananda Nadiminti, DNB Institute: Wockhardt Heart Institute, Bangalore, Karnataka Introduction Myxosarcomas are rare and incidence fewer than 3-5% has been quoted1,2 and these tumors arising from the posterior mitral leaflet (PML) is yet to be reported. The myxosarcoma was seen arising from the PML prolapsing in the aorta, causing a gradient of 70 mmHg across the aortic valve. In order to excise the tumor completely, the mitral valve had to be excised and replaced. Despite an echocardiographic confirmation of complete excision of the tumor, the tumor recurred. The histopathological examination showed both benign and malignant feature in the tumor. Case Report A 62-year-old lady was investigated for dysphagia, dyspnea and pain in the right lower limb. Clinical examination revealed a short systolic murmur in the aortic area. Transthoracic echocardiogram and transesophageal echocardiogram (TEE) showed an intra-cardiac mass measuring 24X34X49 mm. The mass was attached to the posterior mitral leaflet (PML) and freely prolapsing into the aorta causing a peak gradient of 70-mm-Hg (Figure 1). Doppler examination revealed a ‘cut off’ of the right iliac artery, possibly due to a tumor embolus. Preoperative blood investigations were unremarkable, X ray chest showed bilateral pleural effusion. Address for correspondence: Dr. Murali Chakravarthy, MD, DA, DNB Chief Consultant Anesthesiologist, Wockhardt Heart Institute 14, Cunningham Road, Bangalore 560052 Karnataka Telephone- Office: 91 080 51994444 Residence: 91 080 6721378, 91 080 6722796 Fax: 91 080 2281149 E-mail : [email protected]
@IJTCVS 097091342110305/055 Received -11/11/03; Review Completed - 16/12/04; Accepted - 17/12/04.
Normothermic cardiopulmonary bypass (CPB) was established using aortic and bicaval cannulation. Cardiac arrest was achieved by warm potassium rich blood cardioplegia. Small left atrium and thickened mitral leaflets were noted on opening the left atrium. There was a tumor found to be infiltrating in to the left ventricle but the main attachment of the tumor was on PML posteriorly near the commissure. In order to completely excise the tumor, PML had to be excised; mitral valve replacement was performed with a 25 mm tissue valve (Medtronic Hancock® , Medtronic Inc. 710, Medtronic Parkway NE, Minneapolis, MN 55432-5604) and CPB was disconnected easily. Postoperative TEE did not show any residual mass; embolectomy of the right iliac artery was performed successfully. The histological examination of the tumor (Fig 2,3,4) revealed two different patterns. Firstly areas of myxoid and collagen deposits without mitosis (benign); secondly cells exhibiting great degree of polymorphism having dense larger hyperchromatic nuclei and occasional mitosis (malignant). Myxomatous changes and inflammatory infiltrates were seen in the specimen of PML and features of benign myxoma were present in the embolus. The patient made uneventful recovery and was discharged on the 10th day. 40 days later the patient re-admitted with worsening heart failure and TEE showed a recurrent tumor measuring 43X36X74 mm arising from the free wall of the left ventricle (figure 1). The patient suddenly
Fig. 1. Preoperative and postoperative echocardiogram showing recurrent tumor (RT)
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collapsed and died on the 42nd day. The relatives of the deceased did not permit us to perform the postmortem. Discussion Primary malignant cardiac tumors occur with an estimated incidence of 0.002-0.3% of autopsies3, myxoma arising from the mitral leaflet is rare, malignant myxosarcoma of the heart even rarer4 and a few reports of myxomas of the mitral valve are available and are cited to extremely rare5. Cardiac myxomas can cause obstruction to the blood flow in the chambers of the heart, float across heart valves causing obstruction and can embolize6 and all these were seen in the current report. At present, conventional transthoracic and transesophageal echocardiography are the best means of establishing diagnosis and assess the need for surgery in most cases. Cardiac malignant lesions can mimic atrial myxomas at echocardiography; therefore a thorough preoperative investigation should be accomplished, best by magnetic resonance imaging or by total body computed tomography3. Most of the times the intra-cardiac tumors are diagnosed as benign and the malignancy is an unexpected finding at histopathological examination. If malignancy is suspected preoperatively, the option of not performing the surgery can be offered to the patient, because as of now the surgical and non surgical treatment of malignant myxosarcoma is unclear. Radical excision is preferred if malignancy is suspected; it may not be possible to excise the tumor completely without sacrificing important native structures, like mitral valve in this case and such events are described 3 . Unfortunately, most cardiac malignancies are detected too late for curative resection, and palliation remains the mainstay of therapy7. Currently the accepted method
Fig. 2. Microphotograph showing benign features of the tumor
Fig. 3. Shows predominantly pleomorphic bizarre nuclei with indistinct cell borders
Fig. 4. Shows myxomatous stroma
of treatment of intracardiac maliganancies is surgery, combined chemotherapy, peripheral blood stem cell collection transplantation and operations for systemic metastases in the brain, skeletal muscle and lung8. To date the only good medium and long-term results in the therapeutic management of heart sarcomas have been achieved by transplantation: the probable explanation is that criteria of surgical radicality should be those followed for soft tissue tumors located3. Rapidly progressive clinical course, multiple tumor growth and non-septal attachment of the tumor, undifferentiated nature of the cells from the tumor, recurrence from a different location all suggest a malignant profile of the primary cardiac tumors and similar behavior was seen in this case also. Most benign tumors can be resected completely; a few, because of their large size cannot be, and only tumor
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debulking may be possible. Heart transplantation should be considered for these patients. Many of the malignant tumors cannot be resected completely, either because of the extent of local spread and invasion or because of the frequent distant metastases. Transplantation may also be an option for those with extensive local disease. The long-term results for resected benign tumors are excellent; but very poor for malignant ones, and there are few survivors. For patients with unresectable sarcomas, radiation and chemotherapy may be used, but without great successful results4. Johansson and coworkers report a patient who survived 40 months after surgery, and this surprisingly long period of survival may well reflect both the effectiveness of the combined radiation and cytostatic treatment 9 . Non-surgical treatment of malignant tumors of the heart remains unclear. References
5. 6. 7. 8.
immunohistochemistry. Asian Cardiovasc Thorac Ann 2002; 10: 8-11. Basso C, Valente M, Poletti A, Casarotto D, Thiene G. Surgical pathology of primary cardiac and pericardial tumors. Eur J Cardiothorac Surg 1997; 12: 730-37. Donatelli F, Pocar M, Moneta A, Mariani MA, Pelenghi S, Triggiani M, Santoro F, Grossi A. Primary cardiac malignancy presenting as left atrial myxoma. Clinical and surgical considerations. Minerva Chir 1996; 51: 585-88. Samal AK, Ventura HO, Berman A, Okereke C, Gilliland YE, Willis GW J Myxosarcoma: a rare primary cardiac tumor. La State Med Soc 2002; 154: 308-12. Murphy DP, Glazier DB, Krause TJ. Mitral valve myxoma. Ann Thorac Surg 1997; 64: 1169-70. Vander Salm TJ. Unusual primary tumors of the heart. Semin Thorac Cardiovasc Surg 2000; 12: 89-100. Harris GJ, Tio FO, Grover FL.Primary left atrial myxosarcoma. Ann Thorac Surg 1993; 56: 564-66. Roh MS, Huh GY, Jeong JS, Lee GD, Hong SH. Left atrial myxosarcoma with systemic metastasis: a case report. J Korean Med Sci 2001; 16: 111-14. Johansson L, Kugelberg J, Thulin L. Myxofibrosarcoma in the left atrium originally presented as a cardiac myxoma with chondroid differentiation. A clinico-pathological report. APMIS 1989; 97: 833-38.
1. Liu S, Wang Z, Chen AQ, Zhou GH, Jiang ZB, Xiao MD. Cardiac myxoma and myxosarcoma: clinical experience and
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