INTERESTING IMAGE
Pulmonary Artery Angiosarcoma on 18F-FDG PET/CT Masquerading as Pulmonary Embolism Alexander Kessler, MD, and Hongju Son, MD Abstract: A 53-year old woman suspicious for having pulmonary embolism on enhanced chest CT was proven to have metabolically active tumor in her pulmonary arteries on 18F-FDG PET/CT. Through biopsy and surgery, this turned out to be a pulmonary artery angiosarcoma. This case demonstrates that PET/CT is very useful in differentiating an aggressive malignant tumor from a bland pulmonary embolus in the pulmonary vasculature. Key Words: pulmonary embolism, pulmonary artery, angiosarcoma, pulmonary artery angiosarcoma, PET/CT (Clin Nucl Med 2015;40: 82–84)
Received for publication January 02, 2014; revision accepted September 23, 2014. From the Department of Imaging Sciences, University of Rochester Medical Center, Rochester, NY. Conflicts of interest and sources of funding: none declared. Reprints: Hongju Son, MD, Department of Imaging Sciences, University of Rochester Medical Center 601 Elmwood Avenue Box 648, Rochester, NY 14642-8648. E-mail: [email protected]. Copyright © 2014 by Lippincott Williams & Wilkins ISSN: 0363-9762/15/4001–0082
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REFERENCES 1. Bhagwat K, Hallam J, Antippa P, et al. Diagnostic enigma: primary pulmonary artery sarcoma. Interact Cardiovasc Thorac Surg. 2012;14:342–344. 2. Kondo Y, Muto A, Nishibe T, et al. Primary pulmonary artery sarcoma: difficult differential diagnosis from chronic pulmonary thromboembolism. Ann Vasc Surg. 2007;21:505–507. 3. Weissferdt A, Moran CA. Primary vascular tumors of the lungs: a review. Ann Diagn Pathol. 2010;14:296–308. 4. Mayer E, Kriegsmann J, Gaumann A, et al. Surgical treatment of pulmonary artery sarcoma. J Thorac Cardiovasc Surg. 2001;121:77–82. 5. Kojima K, Okamoto I, Ushijima S, et al. Successful treatment of primary pulmonary angiosarcoma. Chest. 2003;124:2397–2400. 6. Widera E, Sulica R. Pulmonary artery sarcoma misdiagnosed as chronic thromboembolic pulmonary hypertension. Mt Sinai J Med. 2005;72:360–364. 7. Sasaki R, Soejima T, Kishi K, et al. Angiosarcoma treated with radiotherapy: impact of tumor type and size on outcome. Int J Radiat Oncol Biol Phys. 2002;52: 1032–1040. 8. Choong CK, Lawton JS, Moon MR, et al. Failure of medical therapy for pulmonary “thromboembolic” disease: beware the unsuspected primary sarcoma of the pulmonary artery. J Thorac Cardiovasc Surg. 2004;128:763–765. 9. Blackmon S, Rice D, Correa A, et al. Management of primary pulmonary artery sarcomas. Ann Thorac Surg. 2009;87:977–984. 10. Wittram C, Scot JA. 18F-FDG PET of pulmonary embolism. AJR Am J Roentgenol. 2007;189:171–176. 11. Chong S, Kim TS, Kim BT, et al. Pulmonary artery sarcoma mimicking pulmonary thromboembolism: integrated FDG PET/CT. AJR Am J Roentgenol. 2007; 188:1691–1693. 12. Suman KCS, Sharma P, Singh H, et al. Primary rhabdomyosarcoma of pulmonary artery: 18F-FDG PET/CT for detecting recurrence in a rare tumor. Clin Nucl Med. 2013;38:e155–e156.
Clinical Nuclear Medicine • Volume 40, Number 1, January 2015
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Clinical Nuclear Medicine • Volume 40, Number 1, January 2015
Pulmonary Artery Angiosarcoma
FIGURE 1. CTA of the chest with axial images at the level of the main pulmonary trunk (A) and right main pulmonary artery (B). A 53-year old woman developed progressive cough, pleuritic chest pain, and dyspnea over the span of several months. CT revealed extensive left main, lobar, and segmental pulmonary artery filling defects that were thought to be pulmonary emboli (arrows). She was started on anticoagulation but after 2 months of therapy, her symptoms continued to worsen.
FIGURE 2. Follow-up CTA of the chest (A and B) and corresponding axial PET images (C and D). CT now reveals significant progression of the filling defects into the more distal left pulmonary arteries (short solid arrows) and right main pulmonary artery (dashed arrows). These lesions show moderate FDG uptake (SUVmax, 6.8) raising suspicion for an aggressive tumor rather than a bland pulmonary embolism. A consolidative lesion with very mild uptake in the posterior left upper lobe (long solid arrows) may represent focal pulmonary infarct or inflammatory lesion. © 2014 Lippincott Williams & Wilkins
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Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
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Clinical Nuclear Medicine • Volume 40, Number 1, January 2015
Kessler and Son
FIGURE 3. Follow-up axial/coronal CTA images (A and C) and corresponding axial PET image (B) 7 months after left pneumonectomy and right main pulmonary endarterectomy. Surgical pathology revealed a pulmonary artery sarcoma, and there is now progression of disease in the right lung involving multiple lobar, segmental, and subsegmental pulmonary arteries (solid arrows) despite postsurgical chemotherapy. The tumor demonstrates intense FDG uptake (SUVmax, 8.4). The left lung shows postsurgical change, and surgical material is partially seen (dashed arrows). Pulmonary artery angiosarcoma is an extremely rare highly malignant tumor that arises from the intima of the pulmonary trunk.1 Patients typically present with symptoms similar to thromboembolic disease, including dyspnea, chest pain, and hemoptysis.2 Characterized by an insidious growth pattern, there is often extensive local invasion and hematogenous metastases such that the malignancy is quite advanced at the time of presentation.3 As the tumor grows, it embolizes to the peripheral pulmonary vasculature, increasing right ventricular afterload and resulting in progressive right heart failure.4 Prognosis is quite dismal, with the majority of patients dying within months of initial presentation despite radical surgical resection and radiochemotherapy.5–7 These tumors present nearly identical to thromboembolic disease and are therefore extremely difficult to diagnose.8 Both FDG PET/CT and Gadolinium-enhanced MRI have both shown the ability to differentiate these entities before surgical biopsy.9 With PET/CT, the bland thrombus seen with acute pulmonary embolus produces an inflammatory response of mild FDG uptake (SUVmean, 1.65).10 Conversely, pulmonary angiosarcoma has been shown to demonstrate high FDG uptake (SUVmean, 7.0).11 This suggests that PET/CT can be particularly sensitive in distinguishing tumor from bland thrombus. In addition, its ability to monitor response to therapy and tumor surveillance after completion of treatment makes it a very useful modality in the workup and management of this disease process.12 Although uncommon, this case demonstrates the importance of keeping a high suspicion for primary pulmonary artery angiosarcoma in patients diagnosed as having thromboembolic disease whose symptoms are not improved with anticoagulation.
84
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© 2014 Lippincott Williams & Wilkins
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Pulmonary Artery Angiosarcoma on 18F-FDG PET/CT Masquerading as Pulmonary Embolism Alexander Kessler, MD, and Hongju Son, MD Abstract: A 53-year old woman suspicious for having pulmonary embolism on enhanced chest CT was proven to have metabolically active tumor in her pulmonary arteries on 18F-FDG PET/CT. Through biopsy and surgery, this turned out to be a pulmonary artery angiosarcoma. This case demonstrates that PET/CT is very useful in differentiating an aggressive malignant tumor from a bland pulmonary embolus in the pulmonary vasculature. Key Words: pulmonary embolism, pulmonary artery, angiosarcoma, pulmonary artery angiosarcoma, PET/CT (Clin Nucl Med 2015;40: 82–84)
Received for publication January 02, 2014; revision accepted September 23, 2014. From the Department of Imaging Sciences, University of Rochester Medical Center, Rochester, NY. Conflicts of interest and sources of funding: none declared. Reprints: Hongju Son, MD, Department of Imaging Sciences, University of Rochester Medical Center 601 Elmwood Avenue Box 648, Rochester, NY 14642-8648. E-mail: [email protected]. Copyright © 2014 by Lippincott Williams & Wilkins ISSN: 0363-9762/15/4001–0082
82
www.nuclearmed.com
REFERENCES 1. Bhagwat K, Hallam J, Antippa P, et al. Diagnostic enigma: primary pulmonary artery sarcoma. Interact Cardiovasc Thorac Surg. 2012;14:342–344. 2. Kondo Y, Muto A, Nishibe T, et al. Primary pulmonary artery sarcoma: difficult differential diagnosis from chronic pulmonary thromboembolism. Ann Vasc Surg. 2007;21:505–507. 3. Weissferdt A, Moran CA. Primary vascular tumors of the lungs: a review. Ann Diagn Pathol. 2010;14:296–308. 4. Mayer E, Kriegsmann J, Gaumann A, et al. Surgical treatment of pulmonary artery sarcoma. J Thorac Cardiovasc Surg. 2001;121:77–82. 5. Kojima K, Okamoto I, Ushijima S, et al. Successful treatment of primary pulmonary angiosarcoma. Chest. 2003;124:2397–2400. 6. Widera E, Sulica R. Pulmonary artery sarcoma misdiagnosed as chronic thromboembolic pulmonary hypertension. Mt Sinai J Med. 2005;72:360–364. 7. Sasaki R, Soejima T, Kishi K, et al. Angiosarcoma treated with radiotherapy: impact of tumor type and size on outcome. Int J Radiat Oncol Biol Phys. 2002;52: 1032–1040. 8. Choong CK, Lawton JS, Moon MR, et al. Failure of medical therapy for pulmonary “thromboembolic” disease: beware the unsuspected primary sarcoma of the pulmonary artery. J Thorac Cardiovasc Surg. 2004;128:763–765. 9. Blackmon S, Rice D, Correa A, et al. Management of primary pulmonary artery sarcomas. Ann Thorac Surg. 2009;87:977–984. 10. Wittram C, Scot JA. 18F-FDG PET of pulmonary embolism. AJR Am J Roentgenol. 2007;189:171–176. 11. Chong S, Kim TS, Kim BT, et al. Pulmonary artery sarcoma mimicking pulmonary thromboembolism: integrated FDG PET/CT. AJR Am J Roentgenol. 2007; 188:1691–1693. 12. Suman KCS, Sharma P, Singh H, et al. Primary rhabdomyosarcoma of pulmonary artery: 18F-FDG PET/CT for detecting recurrence in a rare tumor. Clin Nucl Med. 2013;38:e155–e156.
Clinical Nuclear Medicine • Volume 40, Number 1, January 2015
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Clinical Nuclear Medicine • Volume 40, Number 1, January 2015
Pulmonary Artery Angiosarcoma
FIGURE 1. CTA of the chest with axial images at the level of the main pulmonary trunk (A) and right main pulmonary artery (B). A 53-year old woman developed progressive cough, pleuritic chest pain, and dyspnea over the span of several months. CT revealed extensive left main, lobar, and segmental pulmonary artery filling defects that were thought to be pulmonary emboli (arrows). She was started on anticoagulation but after 2 months of therapy, her symptoms continued to worsen.
FIGURE 2. Follow-up CTA of the chest (A and B) and corresponding axial PET images (C and D). CT now reveals significant progression of the filling defects into the more distal left pulmonary arteries (short solid arrows) and right main pulmonary artery (dashed arrows). These lesions show moderate FDG uptake (SUVmax, 6.8) raising suspicion for an aggressive tumor rather than a bland pulmonary embolism. A consolidative lesion with very mild uptake in the posterior left upper lobe (long solid arrows) may represent focal pulmonary infarct or inflammatory lesion. © 2014 Lippincott Williams & Wilkins
www.nuclearmed.com
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
83
Clinical Nuclear Medicine • Volume 40, Number 1, January 2015
Kessler and Son
FIGURE 3. Follow-up axial/coronal CTA images (A and C) and corresponding axial PET image (B) 7 months after left pneumonectomy and right main pulmonary endarterectomy. Surgical pathology revealed a pulmonary artery sarcoma, and there is now progression of disease in the right lung involving multiple lobar, segmental, and subsegmental pulmonary arteries (solid arrows) despite postsurgical chemotherapy. The tumor demonstrates intense FDG uptake (SUVmax, 8.4). The left lung shows postsurgical change, and surgical material is partially seen (dashed arrows). Pulmonary artery angiosarcoma is an extremely rare highly malignant tumor that arises from the intima of the pulmonary trunk.1 Patients typically present with symptoms similar to thromboembolic disease, including dyspnea, chest pain, and hemoptysis.2 Characterized by an insidious growth pattern, there is often extensive local invasion and hematogenous metastases such that the malignancy is quite advanced at the time of presentation.3 As the tumor grows, it embolizes to the peripheral pulmonary vasculature, increasing right ventricular afterload and resulting in progressive right heart failure.4 Prognosis is quite dismal, with the majority of patients dying within months of initial presentation despite radical surgical resection and radiochemotherapy.5–7 These tumors present nearly identical to thromboembolic disease and are therefore extremely difficult to diagnose.8 Both FDG PET/CT and Gadolinium-enhanced MRI have both shown the ability to differentiate these entities before surgical biopsy.9 With PET/CT, the bland thrombus seen with acute pulmonary embolus produces an inflammatory response of mild FDG uptake (SUVmean, 1.65).10 Conversely, pulmonary angiosarcoma has been shown to demonstrate high FDG uptake (SUVmean, 7.0).11 This suggests that PET/CT can be particularly sensitive in distinguishing tumor from bland thrombus. In addition, its ability to monitor response to therapy and tumor surveillance after completion of treatment makes it a very useful modality in the workup and management of this disease process.12 Although uncommon, this case demonstrates the importance of keeping a high suspicion for primary pulmonary artery angiosarcoma in patients diagnosed as having thromboembolic disease whose symptoms are not improved with anticoagulation.
84
www.nuclearmed.com
© 2014 Lippincott Williams & Wilkins
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
