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Cardiac Computed Tomography Guided Treatment of Metastatic Leiomyosarcoma to the Right Lung with Direct Extension into the Left Atrium of the Heart Islam Abudayyeh MD, Axel Joob MD, Edward Passen MD Islam Abudayyeh MD, Axel Joob MD, Edward Passen MD Division of Cardiology, Advocate Lutheran General Hospital Division of Cardiology, Advocate Lutheran General Hospital Introduction: Cardiac leiomyosarcoma (LMS) is usually a metastatic rather than primary tumor. LMS rarely involves multiple heart chambers through hematogenious spread. Its intra-cardiac occurrence usually portends a poor outcome. LMS is often resistant to chemotherapy or radiation and best treated with surgical resection if the tumor is small and localized in-situ. Methods and Significance: Cardiac CT angiography (CTA) was requested to evaluate the left atrial mass and assess for coronary artery disease prior to palliative cardiac surgery. The right lung mass was confined to the upper lobe, extending into and confined within the pulmonary vein and contiguous with the left atrial mass. Cardiac CTA demonstrated a homogeneous well-circumscribed avascular 21 by 40 mm left atrial mass without attachment to the atrial wall, extending only from the right upper pulmonary vein which separately enters the left atrium. Coronary CTA showed normal coronary arteries Conclusions: Recent advances in imaging with cardiac and coronary CT angiography provides clear images of cardiac and coronary anatomy as well as adjacent structures. To take full advantage of the technology requires additional training and an advanced level of competency. Moreover, image analysis from multiple planes by the cardiologist or cardiac surgeon guides management of complex cardiac conditions. In this case: Cardiac CTA permits assessment of cardiac and extra- cardiac structures as well as coronary arteries in any plane. A multiplanar approach allowed better visualization of the mass and its extension showing that it was a single tumor rather than multiple metastasis. Cardiac CTA may improve diagnosis and treatment options resulting in a real difference in clinical outcome. Advanced imaging in our patient demonstrated a single mass which was resected en-block, resulting in survival due to a curative surgical approach rather than palliative management. 1. Cardiac metastasis from uterine leio- myosarcoma. Moreno Anton F, Casado Herraez A, Puente Vazquez J, etal. Clin Transl Oncol 2006;8:375-8 2. Extensive Cardiac Metastases Secondary to Uterine Leiomyosarcoma. Anna M. Calleja, Clinton V. Wellnitz, etal. J Am Soc Echocardiogr 2009;22:1419.e5-1419.e7 3. Complete resection of a leiomyosarcoma of the left atrium invading the mitral anterior leaflet and obstructing the mitral orifice. Turkyilmaz E, Yilmaz F, Ozkan A, etal. Eur J Echocardiogr , Jan, 1 2008; 9(1):123-5 4. Intravenous Leioma Extending into the Right Ventricle. Hose A. Rocha-Filho , Leonid D. Shturman, David R. Okada, Suhny Abbara, Wilfred Mamuya. J Cardiovascular Computed Tomography, Jan, 2010 5. Noncardiac findings on cardiac CT part I: Pros and cons . Killeen RP, Doss JD, Cury RC. J Cardiovasc Comput Tomogr. 2009 Sep-Oct;3(5):293-9. Epub 2009 May 13. 6. Leiomyosarcoma of the right ventricle extending into the pulmonary trunk . Heart 2001;86:e2 doi:10.1136/heart.86.1.e2 7. Cardiac leiomyosarcoma: primary or secondary? A G Nicol and G M McAndrew. Br Heart J. 1968 May; 30(3): 432–435. 8. Primary Chest Wall Tumors. Asad A Shah, MD, Thomas A D’Amico. J Am Coll Surg. Vol. 210, No. 3, March 2010 9. Cardiac Epithelioid Leiomyosarcoma and the Role of Cardiac Imaging in the Differentiation of Intracardiac Masses. Christine Jellis, Joseph Doyle, Tom Sutherland. Etal. Clin. Cardiol. 33, 6, E6 – E9 (2010). 10. Intravenous leiomyomatosis extending into the right ventricle after subtotal hysterectomy . Mehmet Sah Topcuoglu, Hafize Yaliniz, Hakan Poyrazoglu. Etal. Ann Thorac Surg 2004;78:330-332 11. Magnetic Resonance Imaging of Pericardial Disease and Cardiac Masses . John D. Grizzard, Gregory B. Ang. Cardiol Clin 25 (2007) 111–140. Outcome: The patient underwent a block resection of the right upper lobe with its pulmonary vein and left atrial mass. Pathologic analysis demonstrated leiomyosarcoma. Follow-up over three years demonstrated no lung or cardiac masses. Figure 1. Cardiac CTA axial, coronal, and sagittal views of the cardiac left atrial mass Figure 4. Multiplanar cardiac CTA showing the direct extension from the right upper lobe through the pulmonary vein to the left atrium (standard axes shown on right) Figure 3. Multiplanar cardiac CTA showing the direct extension from the right upper lobe through the pulmonary vein to the left atrium (standard axes shown on right) A 64-year-old woman had uterine leiomyosarcoma treated with hysterectomy and metastatic pulmonary nodules treated with left lower lobectomy and right lower lobe wedge resection. Two years later she developed an enlarging right mid-lung mass and then a new left atrial mass which appeared separate from the lung mass on surveillance chest CTs. Patient Case: References: Figure 2. Cardiac CTA axial, coronal and sagittal views of the right lung mass Figure 9. 3 year follow-up cardiac CTA of right middle and lower pulmonary veins entering the left atrium, right upper pulmonary vein and artery surgical clips and right upper lobectomy suture line Figure 7. 3 year follow-up cardiac CTA axial, coronal, and sagittal views of the cardiac left atrium Figure 8. 3 year follow-up cardiac CTA axial, coronal, and sagittal views of the right lung Figure 6. Cardiac CTA showing normal coronary arteries Figure 5. Separate right upper pulmonary vein with mass

Cardiac Computed Tomography Guided Treatment of Metastatic Leiomyosarcoma to the Right Lung with Direct Extension into the Left Atrium of the Heart Islam

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Cardiac Computed Tomography Guided Treatment ofMetastatic Leiomyosarcoma to the Right Lung withDirect Extension into the Left Atrium of the Heart

Islam Abudayyeh MD, Axel Joob MD, Edward Passen MDIslam Abudayyeh MD, Axel Joob MD, Edward Passen MDDivision of Cardiology, Advocate Lutheran General HospitalDivision of Cardiology, Advocate Lutheran General Hospital

Cardiac Computed Tomography Guided Treatment ofMetastatic Leiomyosarcoma to the Right Lung withDirect Extension into the Left Atrium of the Heart

Islam Abudayyeh MD, Axel Joob MD, Edward Passen MDIslam Abudayyeh MD, Axel Joob MD, Edward Passen MDDivision of Cardiology, Advocate Lutheran General HospitalDivision of Cardiology, Advocate Lutheran General Hospital

Introduction:Cardiac leiomyosarcoma (LMS) is usually a metastatic rather than primary tumor. LMS rarely involves multiple heart chambers through hematogenious spread. Its intra-cardiac occurrence usually portends a poor outcome. LMS is often resistant to chemotherapy or radiation and best treated with surgical resection if the tumor is small and localized in-situ.

Methods and Significance:Cardiac CT angiography (CTA) was requested to evaluate the left atrial mass and assess for coronary artery disease prior to palliative cardiac surgery.

The right lung mass was confined to the upper lobe, extending into and confined within the pulmonary vein and contiguous with the left atrial mass. Cardiac CTA demonstrated a homogeneous well-circumscribed avascular 21 by 40 mm left atrial mass without attachment to the atrial wall, extending only from the right upper pulmonary vein which separately enters the left atrium. Coronary CTA showed normal coronary arteries

Conclusions:Recent advances in imaging with cardiac and coronary CT

angiography provides clear images of cardiac and coronary anatomy as well as adjacent structures. To take full advantage of the technology requires additional training and an advanced level of competency.Moreover, image analysis from multiple planes by the cardiologist or cardiac surgeon guides management of complex cardiac conditions.

In this case:

Cardiac CTA permits assessment of cardiac and extra-cardiac structures as

well as coronary arteries in any plane. A multiplanar approach allowed better visualization of the mass and its extension showing that it was a single tumor rather than multiple metastasis.

Cardiac CTA may improve diagnosis and treatment options resulting in a real

difference in clinical outcome. Advanced imaging in our patient demonstrated a single mass which was resected en-block, resulting in survival due to a curative surgical approach rather than palliative management.

1. Cardiac metastasis from uterine leio- myosarcoma. Moreno Anton F, Casado Herraez A, Puente Vazquez J, etal. Clin Transl Oncol 2006;8:375-8

2. Extensive Cardiac Metastases Secondary to Uterine Leiomyosarcoma. Anna M. Calleja, Clinton V. Wellnitz, etal. J Am Soc Echocardiogr 2009;22:1419.e5-1419.e7

3. Complete resection of a leiomyosarcoma of the left atrium invading the mitral anterior leaflet and obstructing the mitral orifice. Turkyilmaz E, Yilmaz F, Ozkan A, etal. Eur J Echocardiogr , Jan, 1 2008; 9(1):123-5

4. Intravenous Leioma Extending into the Right Ventricle. Hose A. Rocha-Filho , Leonid D. Shturman, David R. Okada, Suhny Abbara, Wilfred Mamuya. J Cardiovascular Computed Tomography, Jan, 2010

5. Noncardiac findings on cardiac CT part I: Pros and cons. Killeen RP, Doss JD, Cury RC. J Cardiovasc Comput Tomogr. 2009 Sep-Oct;3(5):293-9. Epub 2009 May 13.

6. Leiomyosarcoma of the right ventricle extending into the pulmonary trunk. Heart 2001;86:e2 doi:10.1136/heart.86.1.e2

7. Cardiac leiomyosarcoma: primary or secondary? A G Nicol and G M McAndrew. Br Heart J. 1968 May; 30(3): 432–435.

8. Primary Chest Wall Tumors. Asad A Shah, MD, Thomas A D’Amico. J Am Coll Surg. Vol. 210, No. 3, March 2010

9. Cardiac Epithelioid Leiomyosarcoma and the Role of Cardiac Imaging in the Differentiation of Intracardiac Masses. Christine Jellis, Joseph Doyle, Tom Sutherland. Etal. Clin. Cardiol. 33, 6, E6 – E9 (2010).

10. Intravenous leiomyomatosis extending into the right ventricle after subtotal hysterectomy . Mehmet Sah Topcuoglu, Hafize Yaliniz, Hakan Poyrazoglu. Etal. Ann Thorac Surg 2004;78:330-332

11. Magnetic Resonance Imaging of Pericardial Disease and Cardiac Masses. John D. Grizzard, Gregory B. Ang. Cardiol Clin 25 (2007) 111–140.

Outcome:The patient underwent a block resection of the right upper lobe with its pulmonary vein and left atrial mass. Pathologic analysis demonstrated leiomyosarcoma. Follow-up over three years demonstrated no lung or cardiac masses.

Figure 1. Cardiac CTA axial, coronal, and sagittal views of the cardiac left atrial mass

Figure 4. Multiplanar cardiac CTA showing the direct extension from the right upper lobe through the pulmonary vein to the left atrium (standard axes shown on right)

Figure 3. Multiplanar cardiac CTA showing the direct extension from the right upper lobe through the pulmonary vein to the left atrium (standard axes shown on right)

A 64-year-old woman had uterine leiomyosarcoma treated with hysterectomy and metastatic pulmonary nodules treated with left lower lobectomy and right lower lobe wedge resection. Two years later she developed an enlarging right mid-lung mass and then a new left atrial mass which appeared separate from the lung mass on surveillance chest CTs.

Patient Case:

References:

Figure 2.Cardiac CTA axial, coronal and sagittal views of the right lung mass

Figure 9. 3 year follow-up cardiac CTA of right middle and lower pulmonary veins entering the left atrium, right upper pulmonary vein and artery surgical clips and right upper lobectomy suture line

Figure 7.3 year follow-up cardiac CTA axial,

coronal, and sagittal views of the cardiac left atrium

Figure 8. 3 year follow-up cardiac CTA axial, coronal, and sagittal views of the right lung

Figure 6. Cardiac CTA showing normal coronary arteries

Figure 5. Separate right upper pulmonary vein with mass