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Pulmonary Artery Sarcoma Masquerading as Saddle Pulmonary Embolism
CASE REPORTS
Case Reports
Pulmonary Artery Sarcoma Masquerading as Saddle Pulmonary Embolism Somsupha Kanjanauthai, MD a,∗ , Tony Kanluen, MD b , Cynthia Ray, MD c and a
Department of Internal Medicine, Henry Ford Hospital, 2699 West Grand Boulevard, CFP-1, Detroit, MI 48202, United States b Department of Emergency Medicine, Henry Ford Hospital, United States c Division of Pulmonary and Critical Care, Henry Ford Hospital, United States
Pulmonary artery sarcoma is a highly malignant tumour. Therefore, making the diagnosis is very important. We describe a case which presented with dyspnea on exertion and was initially diagnosed as saddle pulmonary embolism per CT thorax with contrast. Despite adequate anticoagulation, symptoms still progressed. Follow-up CT thorax showed an extension of the presumed filling defect or clots into the left main pulmonary artery with new lung nodules. This prompted suspicion that this may not be a pulmonary embolism. Biopsy of the lung nodule revealed high grade soft tissue sarcoma with primary source from the pulmonary artery. Our case highlights that pulmonary artery sarcoma should always be included in the differential diagnosis of pulmonary embolism especially, if symptoms still progress while on adequate anticoagulation, or any pulmonary nodules develop on follow-up exam. (Heart, Lung and Circulation 2008;17:417–436) Published by Elsevier Inc on behalf of Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Keywords. Pulmonary artery sarcoma; Pulmonary angiosarcoma; Pulmonary embolism; Sarcoma
Introduction
P
ulmonary artery sarcoma is a very rare tumour of the cardiovascular system with only a few cases reported in recent literature.1–4 The disease was first described at autopsy by Mandelstamm.5 Most of the subsequent reported cases have been diagnosed at autopsy. Making a clinical diagnosis of pulmonary artery sarcoma is very difficult and one needs close follow-up to make a definite diagnosis. It can present as dyspnea, chest pain, cough, or haemoptysis. We describe a case that initially was misdiagnosed as pulmonary embolism per computed tomography (CT) thorax findings suggesting a filling defect or clot in the pulmonary arteries. Subsequently, the diagnosis was made by follow-up CT thorax revealing a lung nodule that was biopsied.
Case A 56-year-old male with past medical history of hypothyroidism and hepatitis C presented to the emergency room (ER) with several weeks of progressive exertional dyspnea. He denied chest pain, paroxysmal nocturnal dyspnea, orthopnea, fever, chills, and cough. Initial chest X-ray was normal. CT thorax with contrast was performed and revealed what appeared to be a large saddle embolism in Received 30 July 2007; received in revised form 3 August 2007; accepted 15 August 2007; available online 2 October 2007 ∗
Corresponding author. Tel.: +1 313 916 3829. E-mail address: [email protected] (S. Kanjanauthai).
the pulmonary trunk and right main pulmonary artery. He was admitted for anticoagulation and discharged home uneventfully. Two months later, his shortness of breath progressively worsened and he developed right-sided pleuritic chest pain. Follow-up CT thorax with contrast (Fig. 1) demonstrated an extension of the saddle embolus into a central portion of the left main pulmonary artery and new nodules in the right upper, middle (Fig. 2), and lower lobe. Two dimensional (2D) echocardiography showed normal ejection fraction and no sign of right ventricular strain. Evaluation for inherited coagulation disorders was negative. Due to a high suspicion of non-thromboembolic process such as neoplasm, positron emission tomography (PET) scan was done and demonstrated increased metabolic activity in the small lung nodules. Therefore, CT-guided biopsy was performed and revealed high grade soft tissue sarcoma (Fig. 3) with the primary source most likely from the pulmonary artery. The patient subsequently underwent chemotherapy with initial improvement. Due to the high grade sarcoma and presence of metastases at the initial diagnosis, the disease continued to progress despite aggressive chemotherapy and the patient expired one and a half years later.
Discussion Pulmonary artery sarcoma is a rare tumour of the cardiovascular system, and very few cases have been reported in recent literature.1–4 Most pulmonary artery sarcomas arise from the dorsal area of the pulmonary trunk,6–8
Published by Elsevier Inc on behalf of Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand.
1443-9506/04/$30.00 doi:10.1016/j.hlc.2007.08.003
418
Case Reports
CASE REPORTS
Heart, Lung and Circulation 2008;17:417–436
Figure 1. CT thorax with contrast revealed a large filling defect in pulmonary trunk, right, and left main pulmonary arteries (arrow).
Figure 2. CT thorax with contrast demonstrated lung nodules in right middle lobe (arrow).
although the tumours also may arise from the right and left pulmonary arteries, the pulmonary valve, and the right ventricular outflow tract. Because of its rarity and insidious growth characteristics, pulmonary artery sarcoma is often misdiagnosed as pulmonary embolism,9,10 leading to inappropriate therapy such as prolonged anticoagulation or thrombolysis. Symptoms and signs such as weight loss, fever, anaemia,11,12 and digital clubbing13,14 may be subtle clues to diagnosis. Other characteristics, such as the absence of risk factors for deep vein thrombosis, high sedimentation rate, nodular parenchymal infiltrates on CT scans, unilateral absence of blood flow on perfusion scan, and lack of response to anticoagulation should raise the suspicion of a process other than pulmonary embolism. In our case, the preliminary diagnosis was made by lack
Figure 3. Histopathologic appearance of the atypical high grade spindle cell sarcoma.
of response to adequate anticoagulation and developing new pulmonary nodules. CT-guided lung nodule biopsy confirmed the diagnosis of pulmonary artery sarcoma. Our case highlights that pulmonary artery sarcoma should always be included in the differential diagnosis of pulmonary embolism especially if symptoms progress while on adequate anticoagulation, or any pulmonary nodules develop on follow-up exam.
Conclusion Although rare, pulmonary artery sarcoma should be kept on the differential diagnosis when the symptoms progress despite adequate anticoagulation for a pulmonary embolism or new pulmonary nodules develop.
References 1. Chong S, Kim TS, Kim BT, Cho EY, Kim J. Pulmonary artery sarcoma mimicking pulmonary artery thromboembolism: integrated FDG PET/CT. AJR Am J Roentgenol 2007;188:1691–3. 2. Alsoufi B, Slater M, Smith PP, Karamlou T, Mansoor A, Ravichandran P. Pulmonary artery sarcoma mimicking massive pulmonary embolus: a case report. Asian Cardiovasc Thorac Ann 2006;14:e71–3. 3. Levy E, Korach A, Amir G, Milgalter E. Undifferentiated sarcoma of the pulmonary artery mimicking pulmonary thromboembolic disease. Heart Lung Circ 2006;15:62–3. 4. Allen S, Todd J, Copley S, Al-Nahhas A. F-18 FDG uptake in bilateral pulmonary artery leiomyosarcoma, one mimicking a pulmonary embolus. Clin Nucl Med 2005;30:418–9. 5. Mandelstamm M. Uber primare Neubildugen des Herzens. Virchows Arch Pathol Anat 1923;245:43–54. 6. Fox JE, Chiles C, Aquino SL, et al. Pulmonary artery sarcomas: a review of clinical and radiological features. J Comput Assist Tomogr 1997;21:750–5. 7. Burke AP, Virmani R. Sarcomas of the great vessels: a clinicopathological study. Cancer 1993;71:1761–3. 8. Hohbach C, Mall W. Chondrosarcoma of the pulmonary artery. Beitr pathol 1977;160:298–307.
Case Reports
9. Delaney SG, Doyle TCA, Burton RW, et al. Pulmonary artery sarcoma mimicking pulmonary embolism. Chest 1993;103:1631–3. 10. Akomea AC, Dussek JE, Anderson DR, et al. Pulmonary artery sarcoma mimicking Pulmonary embolism: successful surgical intervention. Ann Thorac Surg 1996;61:1536–8. 11. Altman NH, Shelley WM. Primary intimal sarcoma of the pulmonary artery. Johns Hopkins Med J 1973;133:214–22.
419
12. Wackers FJ, Van Der Schoot JB, Hampe JF. Sarcoma of the pulmonary trunk associated with hemorrhagic tendency. Cancer 1969;23:339–51. 13. Hopwood D, McNeil G. Spindle cell sarcoma of the pulmonary trunk: a case report with histochemistry and electron microscopy. J Pathol 1979;128:71–7. 14. Jose SL, Fedullo PF, Franco P, et al. Digital clubbing associated with pulmonary artery sarcoma. Chest 1996;109:1651–3.
Concealed Neo-Aortic Incompetence and New Right Ventricular Outflow Tract Obstruction After Arterial Switch Operation in a Late Presenter Sameh I. Sersar, MD ∗ , Ghassan M. Baslaim, MD, Division of Cardiothoracic Surgery, King Faisal Specialist Hospital & Research Centre, Jeddah 21499, Saudi Arabia.
We report a 9 year old boy who presented late with cyanosis as a case of dextro-transposition of great arteries, intact ventricular septum and left ventricular outflow tract obstruction (D-TGA/IVS/LVOTO). Arterial switch operation (ASO) with resection of sub-neo aortic membrane and repair of mitral valve were done for the naturally trained LV. On the second postoperative day, the newly discovered right ventricular outflow tract obstruction (RVOTO) was relieved and mitral valve replacement (MVR) was done for significant mixed stenotic/regurgitant mitral valve disease, and intraoperative extra-corporeal membrane oxygenation (ECMO) support was instituted for pulmonary dysfunction for 4 days. Failure of extubation warranted further assessment that revealed significant aortic incompetence (AI) during cardiac catheterisation study, which was underestimated by echocardiography. Aortic valve replacement (AVR) was done on the 11th postoperative day and he was then extubated and had uneventful hospital course in spite of two emergency procedures: drainage of sub-dural haematoma and appendectomy. (Heart, Lung and Circulation 2008;17:417–436) © 2007 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved. Keywords. Neo-aortic incompetence; Late presenter
D
espite the fact that potential postoperative problems of the arterial switch operation (ASO) still remain mostly unknown for considerably late presenters with dextro-transposition of great arteries, intact ventricular septum and left ventricular outflow tract obstruction (DTGA/IVS/LVOTO), this operation remains the procedure of choice for patients with transposition. This operation can be performed in certain subsets of patients with DTGA/IVS/LVOTO with early and late survival similar to that of matched patients with normal pulmonary valve and LVOT.1 The development of right ventricular outflow tract obstruction (RVOTO) and neo-aortic incompetence (AI) Received 24 February 2007; received in revised form 6 August 2007; accepted 8 August 2007; available online 4 October 2007 ∗ Corresponding author at: Division of Cardiothoracic Surgery, King Faisal Specialist Hospital & Research Centre, MBC-J 16, PO Box 40047, Jeddah 21499, Saudi Arabia. Tel.: +966 26 677 777; fax: +966 26 639 581. E-mail address: [email protected] (S.I. Sersar).
after ASO are known potential problems during intermediate-to-late follow-up.2,3 However, immediate postoperative RVOTO and neo-AI requiring intervention are rare. Hereby, we describe the challenge of diagnosis and treatment of these two complications in a late presenter for ASO.
Case Report The patient is a 9-year-old boy referred as a late presenter with chronic cyanosis secondary to D-TGA/IVS/LVOTO. Trans-thoracic echocardiography (TTE) showed a subpulmonic membrane with a gradient of 70 mmHg across the LVOT, large atrial septal defect (ASD), mild pulmonary incompetence, MV regurgitation and well-balanced ventricles with preserved function (Fig. 1a). The patient underwent ASO, Lecompte manoeuvre for antero-posteriorly related great arteries with the main pulmonary artery slightly larger than the aorta, buttonhole transfer technique for the usual coronary pattern, mitral valve repair for a significantly thickened and dys-
© 2007 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved.
1443-9506/04/$30.00 doi:10.1016/j.hlc.2007.08.001
CASE REPORTS
Heart, Lung and Circulation 2008;17:417–436
Case Reports
Pulmonary Artery Sarcoma Masquerading as Saddle Pulmonary Embolism Somsupha Kanjanauthai, MD a,∗ , Tony Kanluen, MD b , Cynthia Ray, MD c and a
Department of Internal Medicine, Henry Ford Hospital, 2699 West Grand Boulevard, CFP-1, Detroit, MI 48202, United States b Department of Emergency Medicine, Henry Ford Hospital, United States c Division of Pulmonary and Critical Care, Henry Ford Hospital, United States
Pulmonary artery sarcoma is a highly malignant tumour. Therefore, making the diagnosis is very important. We describe a case which presented with dyspnea on exertion and was initially diagnosed as saddle pulmonary embolism per CT thorax with contrast. Despite adequate anticoagulation, symptoms still progressed. Follow-up CT thorax showed an extension of the presumed filling defect or clots into the left main pulmonary artery with new lung nodules. This prompted suspicion that this may not be a pulmonary embolism. Biopsy of the lung nodule revealed high grade soft tissue sarcoma with primary source from the pulmonary artery. Our case highlights that pulmonary artery sarcoma should always be included in the differential diagnosis of pulmonary embolism especially, if symptoms still progress while on adequate anticoagulation, or any pulmonary nodules develop on follow-up exam. (Heart, Lung and Circulation 2008;17:417–436) Published by Elsevier Inc on behalf of Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Keywords. Pulmonary artery sarcoma; Pulmonary angiosarcoma; Pulmonary embolism; Sarcoma
Introduction
P
ulmonary artery sarcoma is a very rare tumour of the cardiovascular system with only a few cases reported in recent literature.1–4 The disease was first described at autopsy by Mandelstamm.5 Most of the subsequent reported cases have been diagnosed at autopsy. Making a clinical diagnosis of pulmonary artery sarcoma is very difficult and one needs close follow-up to make a definite diagnosis. It can present as dyspnea, chest pain, cough, or haemoptysis. We describe a case that initially was misdiagnosed as pulmonary embolism per computed tomography (CT) thorax findings suggesting a filling defect or clot in the pulmonary arteries. Subsequently, the diagnosis was made by follow-up CT thorax revealing a lung nodule that was biopsied.
Case A 56-year-old male with past medical history of hypothyroidism and hepatitis C presented to the emergency room (ER) with several weeks of progressive exertional dyspnea. He denied chest pain, paroxysmal nocturnal dyspnea, orthopnea, fever, chills, and cough. Initial chest X-ray was normal. CT thorax with contrast was performed and revealed what appeared to be a large saddle embolism in Received 30 July 2007; received in revised form 3 August 2007; accepted 15 August 2007; available online 2 October 2007 ∗
Corresponding author. Tel.: +1 313 916 3829. E-mail address: [email protected] (S. Kanjanauthai).
the pulmonary trunk and right main pulmonary artery. He was admitted for anticoagulation and discharged home uneventfully. Two months later, his shortness of breath progressively worsened and he developed right-sided pleuritic chest pain. Follow-up CT thorax with contrast (Fig. 1) demonstrated an extension of the saddle embolus into a central portion of the left main pulmonary artery and new nodules in the right upper, middle (Fig. 2), and lower lobe. Two dimensional (2D) echocardiography showed normal ejection fraction and no sign of right ventricular strain. Evaluation for inherited coagulation disorders was negative. Due to a high suspicion of non-thromboembolic process such as neoplasm, positron emission tomography (PET) scan was done and demonstrated increased metabolic activity in the small lung nodules. Therefore, CT-guided biopsy was performed and revealed high grade soft tissue sarcoma (Fig. 3) with the primary source most likely from the pulmonary artery. The patient subsequently underwent chemotherapy with initial improvement. Due to the high grade sarcoma and presence of metastases at the initial diagnosis, the disease continued to progress despite aggressive chemotherapy and the patient expired one and a half years later.
Discussion Pulmonary artery sarcoma is a rare tumour of the cardiovascular system, and very few cases have been reported in recent literature.1–4 Most pulmonary artery sarcomas arise from the dorsal area of the pulmonary trunk,6–8
Published by Elsevier Inc on behalf of Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand.
1443-9506/04/$30.00 doi:10.1016/j.hlc.2007.08.003
418
Case Reports
CASE REPORTS
Heart, Lung and Circulation 2008;17:417–436
Figure 1. CT thorax with contrast revealed a large filling defect in pulmonary trunk, right, and left main pulmonary arteries (arrow).
Figure 2. CT thorax with contrast demonstrated lung nodules in right middle lobe (arrow).
although the tumours also may arise from the right and left pulmonary arteries, the pulmonary valve, and the right ventricular outflow tract. Because of its rarity and insidious growth characteristics, pulmonary artery sarcoma is often misdiagnosed as pulmonary embolism,9,10 leading to inappropriate therapy such as prolonged anticoagulation or thrombolysis. Symptoms and signs such as weight loss, fever, anaemia,11,12 and digital clubbing13,14 may be subtle clues to diagnosis. Other characteristics, such as the absence of risk factors for deep vein thrombosis, high sedimentation rate, nodular parenchymal infiltrates on CT scans, unilateral absence of blood flow on perfusion scan, and lack of response to anticoagulation should raise the suspicion of a process other than pulmonary embolism. In our case, the preliminary diagnosis was made by lack
Figure 3. Histopathologic appearance of the atypical high grade spindle cell sarcoma.
of response to adequate anticoagulation and developing new pulmonary nodules. CT-guided lung nodule biopsy confirmed the diagnosis of pulmonary artery sarcoma. Our case highlights that pulmonary artery sarcoma should always be included in the differential diagnosis of pulmonary embolism especially if symptoms progress while on adequate anticoagulation, or any pulmonary nodules develop on follow-up exam.
Conclusion Although rare, pulmonary artery sarcoma should be kept on the differential diagnosis when the symptoms progress despite adequate anticoagulation for a pulmonary embolism or new pulmonary nodules develop.
References 1. Chong S, Kim TS, Kim BT, Cho EY, Kim J. Pulmonary artery sarcoma mimicking pulmonary artery thromboembolism: integrated FDG PET/CT. AJR Am J Roentgenol 2007;188:1691–3. 2. Alsoufi B, Slater M, Smith PP, Karamlou T, Mansoor A, Ravichandran P. Pulmonary artery sarcoma mimicking massive pulmonary embolus: a case report. Asian Cardiovasc Thorac Ann 2006;14:e71–3. 3. Levy E, Korach A, Amir G, Milgalter E. Undifferentiated sarcoma of the pulmonary artery mimicking pulmonary thromboembolic disease. Heart Lung Circ 2006;15:62–3. 4. Allen S, Todd J, Copley S, Al-Nahhas A. F-18 FDG uptake in bilateral pulmonary artery leiomyosarcoma, one mimicking a pulmonary embolus. Clin Nucl Med 2005;30:418–9. 5. Mandelstamm M. Uber primare Neubildugen des Herzens. Virchows Arch Pathol Anat 1923;245:43–54. 6. Fox JE, Chiles C, Aquino SL, et al. Pulmonary artery sarcomas: a review of clinical and radiological features. J Comput Assist Tomogr 1997;21:750–5. 7. Burke AP, Virmani R. Sarcomas of the great vessels: a clinicopathological study. Cancer 1993;71:1761–3. 8. Hohbach C, Mall W. Chondrosarcoma of the pulmonary artery. Beitr pathol 1977;160:298–307.
Case Reports
9. Delaney SG, Doyle TCA, Burton RW, et al. Pulmonary artery sarcoma mimicking pulmonary embolism. Chest 1993;103:1631–3. 10. Akomea AC, Dussek JE, Anderson DR, et al. Pulmonary artery sarcoma mimicking Pulmonary embolism: successful surgical intervention. Ann Thorac Surg 1996;61:1536–8. 11. Altman NH, Shelley WM. Primary intimal sarcoma of the pulmonary artery. Johns Hopkins Med J 1973;133:214–22.
419
12. Wackers FJ, Van Der Schoot JB, Hampe JF. Sarcoma of the pulmonary trunk associated with hemorrhagic tendency. Cancer 1969;23:339–51. 13. Hopwood D, McNeil G. Spindle cell sarcoma of the pulmonary trunk: a case report with histochemistry and electron microscopy. J Pathol 1979;128:71–7. 14. Jose SL, Fedullo PF, Franco P, et al. Digital clubbing associated with pulmonary artery sarcoma. Chest 1996;109:1651–3.
Concealed Neo-Aortic Incompetence and New Right Ventricular Outflow Tract Obstruction After Arterial Switch Operation in a Late Presenter Sameh I. Sersar, MD ∗ , Ghassan M. Baslaim, MD, Division of Cardiothoracic Surgery, King Faisal Specialist Hospital & Research Centre, Jeddah 21499, Saudi Arabia.
We report a 9 year old boy who presented late with cyanosis as a case of dextro-transposition of great arteries, intact ventricular septum and left ventricular outflow tract obstruction (D-TGA/IVS/LVOTO). Arterial switch operation (ASO) with resection of sub-neo aortic membrane and repair of mitral valve were done for the naturally trained LV. On the second postoperative day, the newly discovered right ventricular outflow tract obstruction (RVOTO) was relieved and mitral valve replacement (MVR) was done for significant mixed stenotic/regurgitant mitral valve disease, and intraoperative extra-corporeal membrane oxygenation (ECMO) support was instituted for pulmonary dysfunction for 4 days. Failure of extubation warranted further assessment that revealed significant aortic incompetence (AI) during cardiac catheterisation study, which was underestimated by echocardiography. Aortic valve replacement (AVR) was done on the 11th postoperative day and he was then extubated and had uneventful hospital course in spite of two emergency procedures: drainage of sub-dural haematoma and appendectomy. (Heart, Lung and Circulation 2008;17:417–436) © 2007 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved. Keywords. Neo-aortic incompetence; Late presenter
D
espite the fact that potential postoperative problems of the arterial switch operation (ASO) still remain mostly unknown for considerably late presenters with dextro-transposition of great arteries, intact ventricular septum and left ventricular outflow tract obstruction (DTGA/IVS/LVOTO), this operation remains the procedure of choice for patients with transposition. This operation can be performed in certain subsets of patients with DTGA/IVS/LVOTO with early and late survival similar to that of matched patients with normal pulmonary valve and LVOT.1 The development of right ventricular outflow tract obstruction (RVOTO) and neo-aortic incompetence (AI) Received 24 February 2007; received in revised form 6 August 2007; accepted 8 August 2007; available online 4 October 2007 ∗ Corresponding author at: Division of Cardiothoracic Surgery, King Faisal Specialist Hospital & Research Centre, MBC-J 16, PO Box 40047, Jeddah 21499, Saudi Arabia. Tel.: +966 26 677 777; fax: +966 26 639 581. E-mail address: [email protected] (S.I. Sersar).
after ASO are known potential problems during intermediate-to-late follow-up.2,3 However, immediate postoperative RVOTO and neo-AI requiring intervention are rare. Hereby, we describe the challenge of diagnosis and treatment of these two complications in a late presenter for ASO.
Case Report The patient is a 9-year-old boy referred as a late presenter with chronic cyanosis secondary to D-TGA/IVS/LVOTO. Trans-thoracic echocardiography (TTE) showed a subpulmonic membrane with a gradient of 70 mmHg across the LVOT, large atrial septal defect (ASD), mild pulmonary incompetence, MV regurgitation and well-balanced ventricles with preserved function (Fig. 1a). The patient underwent ASO, Lecompte manoeuvre for antero-posteriorly related great arteries with the main pulmonary artery slightly larger than the aorta, buttonhole transfer technique for the usual coronary pattern, mitral valve repair for a significantly thickened and dys-
© 2007 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved.
1443-9506/04/$30.00 doi:10.1016/j.hlc.2007.08.001
CASE REPORTS
Heart, Lung and Circulation 2008;17:417–436