Pulmonary Artery Angiosarcoma
Dr. João Sakuray Pais | Orientador: Dr. Eduardo Kaiser Ururahy Nunes Fonseca
Differential Diagnosis
Pulmonary Thromboembolism
- Partial or complete embolic occlusion of one or more pulmonary arteries
- Usually thrombi deposit in vessels bifurcation
- Main imaging characteristics are filling defects within the pulmonary vasculature with acute pulmonary emboli
Myxoma
- More common in the left atrium (LA), especially in the interatrial septum originating from fossa ovalis (78%)
- Most common primary cardiac neoplasm (50%)
- Signs and symptoms of central nervous system or peripheral embolization, which may be caused by tumor fragments or accumulated thrombus
- Main imaging characteristics are the presence of a narrow stalk, which is normally seen in echocardiogram, and tumor mobility. Also, usually shows intense high signal in Triple IR (cardiac MRI). Mild or no 18-FDG uptake
Papillary Fibroelastoma
- Collection of avascular fronds of dense connective tissue lined by endothelium
- 10% of all primary cardiac tumors and the second most common benign primary cardiac neoplasm after myxoma
- 90% occur on valve surfaces, making them by far the most common primary cardiac tumor to occur on valves
- Main imaging features: small, mobile masses attached to valves by a short pedicle. Also shows a marked homogeneous and progressive late gadolinium enhancement compared to perfusion / early enhancement. Mild or no 18-FDG uptake
Sarcoma
- Rare and aggressive, due to infiltrative nature and early spread
- 25% of all primary malignant cardiac tumors
- Wide contact with the surface (vessel wall or myocardium), intense 18-FDG uptake and late heterogeneous gadolinium enhancement
Surgery
- Bilateral thromboendarterectomy type 1
- Pulmonary valve replacement PBio 21
- Enlargement of the right ventricular outflow tract
- Correction with pericardial patch of the pulmonary valve
- Excision of tumor originating from the pulmonary valve
Anatomopathology
- Intimal sarcoma of the pulmonary artery
- Branches of the pulmonary artery involved by the neoplasm
- Surgical margin compromised by the neoplasm
- Immunohistochemistry results: positive for smooth muscle actin; negative for CD31 and CD34
Definitive Anatomopathological Diagnosis
Anatomopathological-Imaging Correlation
Follow up
Patient referred to São Paulo Cancer Institute (ICESP)
Chemotherapy:
- Literature review
- Few cases treated with Antracicline, Vinorelbine and Platin
- High risk of recurrence and death
- Referred to radiotherapy
- Chemotherapy if recurrence
Radiotherapy:
- Adjuvant radiotherapy
- 30 x 200Gy in heart
- 30 sessions
- Actinic pneumonitis – improvement with corticosteroids
Follow up – 12 months
Local Recurrence: elongated filling defect, apparently originating near the valve prosthesis and extending into the right ventricle
Metastasis: nodular opacity in the middle lobe (0.8 cm) suspected of secondary involvement, associated with atelectasia
References
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Cardiac Neoplasms with Echocardiographic Correlation. RadioGraphics. 2000 Sep;20(5):1303–19.
- Colak E, Kitamura FC, Hobbs SB, Wu CC, Lungren MP, Prevedello LM, et al. The RSNA Pulmonary
Embolism CT Dataset. Radiology: Artificial Intelligence. 2021 Mar 1;3(2):e200254.
- Wittram C, Maher MM, Yoo AJ, Kalra MK, Shepard JAO, McLoud TC. CT Angiography of Pulmonary
Embolism: Diagnostic Criteria and Causes of Misdiagnosis. RadioGraphics. 2004 Sep;24(5):1219–38.
- Cervilla-Muñoz E, Galeano‐Valle F, Del-Toro-Cervera J, Calleja-Cartón E, Demelo‐Rodríguez P.
Differential diagnosis and treatment approach to pulmonary artery sarcoma: a case report and literature
review. ERJ Open Research. 2020 Jul 1;6(3):00124-2020.
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and Lung. Seminars in Ultrasound, CT and MRI. 2011 Oct;32(5):365–76.
- Chong S, Kim TS, Kim BT, Cho EY, Kim J. Pulmonary Artery Sarcoma Mimicking Pulmonary
Thromboembolism: Integrated FDG PET/CT. American Journal of Roentgenology. 2007 Jun;188(6):1691–3.
- Kim C, Kim MY, Kang JW, Song JS, Lee KY, Kim SS. Pulmonary Artery Intimal Sarcoma versus Pulmonary
Artery Thromboembolism: CT and Clinical Findings. Korean Journal of Radiology [Internet]. 2018;19(4):792.
Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6005959/
