British Journal of Radiology (2004) 77, 623-625
© 2004 British Institute of Radiology
doi: 10.1259/bjr/52485284
Pulmonary haemangiosarcoma with main pulmonary artery thrombosis imitating subacute pulmonary embolism with infarction
C Engelke, MD
1
M Riedel, MD, FESC
2
E J Rummeny, MD
1 and
K Marten, MD
1
1 Department of Radiology, Klinikum der TU München, Ismaningerstr 22, 81675 Munich and 2 Department of Cardiology, Deutsches Herzzentrum München, TU München, Lazarettstr 36, 80636 Munich, Germany
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Abstract
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We report a patient with subacute pulmonary hypertension caused by unilateral massive pulmonary artery thrombosis due to a pulmonary haemangiosarcoma of the lower lobe with pulmonary arterial and bronchial invasion. The patient was misdiagnosed as having subacute pulmonary embolism and underwent thrombolytic therapy complicated by severe pulmonary haemorrhage. The imaging features of pulmonary artery thrombosis with underlying malignancy and their differential diagnosis are discussed.
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Introduction
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In situ pulmonary artery thrombosis (PAT) in adults is a potentially life-threatening disorder rarely recognized ante mortem. The English literature pertaining to PAT is limited to isolated clinical case reports or small descriptive pathological series stating a close association with concurrent morbidity such as thrombophilia, pneumonia or pulmonary arterial flow disturbance [1–5]. The purpose of this case report is to familiarize radiologists with this entity, because CT angiography (CTA) can be instrumental in reaching the diagnosis in these patients who are commonly referred with clinical suspicion of pulmonary thromboembolism.
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Case report
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A 71-year-old Caucasian woman presented with a 1-week history of progressive dyspnoea, sudden-onset left-sided chest pain, cough, haemoptysis and fever. She had suffered an anterior myocardial infarction 15 years ago but had otherwise been well in the past. There was no past medical history of venous thromboembolism or chronic pulmonary disease. On admission she was in respiratory distress with cyanosis, tachypnoea (22 min–1) and tachycardia (125 beats min–1), but her blood pressure was stable (120/75 mmHg). Under mask-inhalation of 2 l of oxygen per minute arterial blood gas analysis showed global respiratory insufficiency (PaO2 55.2 mmHg, PaCO2 49.1 mmHg) with metabolic and respiratory acidosis (pH 7.237, base deficit 6.9 mmol l–1). Her fibrinogen, C-reactive protein and cardiac troponin T (0.18 ng ml–1) levels were elevated but other routine biochemical parameters were within normal limits. Electrocardiogram (ECG) showed atrial fibrillation, a SIQIIITIII-pattern, but no signs of acute myocardial ischaemia. Transthoracic echocardiography was indicative of pulmonary hypertension, revealing massive right ventricular dilatation and a systolic pressure gradient across the tricuspid valve of 45 mmHg. No right heart thrombi were found. An initial chest radiograph showed a prominent main pulmonary artery and revealed left-sided retrocardiac pulmonary opacity (Figure 1
).
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Figure 1. Chest radiograph displaying a prominent pulmonary artery left retrocardiac lower lobe consolidation or mass and a right mid zone infiltrate found to be consistent with pulmonary haemorrhage on subsequent CT.
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Lung scintigraphy for suspected pulmonary embolism (PE) showed absent perfusion of the left lung and bilateral diffuse ventilation inhomogeneity with a complete defect in the left lower lobe, interpreted as high probability for PE. Subsequently she received 100 mg recombinant tissue plasminogen activator (rt-PA) intravenously over 2 h followed by therapeutic heparinization. The following day pulmonary CTA, performed for continuing minor haemoptysis, confirmed total occlusion of the left main pulmonary artery believed to be related to massive unilateral PE with a left lower lobe "consolidation" (Figure 2a) thought to represent post-infarction pneumonia, supported by pneumococci-positive sputum. The patient received a repeat dose of 50 mg rt-PA and antibiotics were commenced. However, haemoptysis increased dramatically and subsequent bronchoscopy revealed an exophytic mass at the left anterobasal segment bronchus origin. Histopathological examination including immunochemistry of a bronchial biopsy specimen of the lesion was diagnostic for respiratory mucosal haemangiosarcoma (grade 2).
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Figure 2. (a) Initial pulmonary CT angiography (CTA) in the early arterial phase showing pulmonary arterial hypertension with a main pulmonary artery diameter exceeding the ascending aortic width and left pulmonary artery thrombosis with "smooth-margined" proximal total occlusion at its origin. Note right hilar subcarinal and paratracheal lymphadenopathy and the left lower lobe-consolidating mass (*), which was initially interpreted as pulmonary infarction. (b) The late subsequent late arterial CTA 1 week later shows bright tumour enhancement within the pulmonary mass (arrowheads). Axial CT section below the carina displays the main tumour portion within the left lower pulmonary lobe (arrowheads). The patient had new small bilateral pleural effusions.
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Repeat late arterial phase pulmonary CTA after 1 week showed unchanged complete occlusion of the left pulmonary artery system by non-enhancing material with a smooth, "streamline-" or "sediment-like"-shaped margin at the main pulmonary artery bifurcation consistent with PAT but atypical for acute or subacute PE. The left lower lobe consolidating mass displayed substantial peripheral contrast enhancement and had direct contact to the occluded left lower lobe artery. Bilateral mediastinal lymphadenopathy was noted (Figure 2b
). In addition there was bilateral pulmonary lobular ground glass opacity in view of the haemoptysis consistent with pulmonary haemorrhage. Cranial, cervical and abdominal staging-CTs were unremarkable and lower limb duplex ultrasound was negative for deep venous thrombosis. In view of the mediastinal lymphadenopathy, the advanced left pulmonary tumour, was considered irresectable. After stabilization the patient underwent palliative chemotherapy with partial local and mediastinal tumour remission. Since that time there has been local tumour progression, severe chronic pulmonary hypertension and hemiparesis from cerebral metastasis.
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Discussion
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In situ PAT in adults has been reported sporadically in isolated clinical cases or descriptive pathological series in close association with drug-induced or intrinsic thrombophilia, thrombophilia with underlying systemic disease such as nephrotic syndrome, with pulmonary arterial occlusion by tumours such as primary pulmonary artery sarcoma, and with various severe pulmonary parenchymal disorders including mycotic or caseous tuberculous pneumonia [1, 2]. PAT with underlying pulmonary parenchymal disease is usually minor and causes few clinical symptoms. Other causes such as thrombophilia or tumour invasion of the pulmonary artery can cause distally or proximally propagating PAT. The course of acute massive PAT is serious and may result in sudden death. PAT may also cause subacute cor pulmonale or have a more chronic fatal course in cases with missed diagnosis [1, 3–5]. Unfortunately due to its presumably inconstant and non-specific clinical symptoms and similarities with thromboembolism on CTA, the ante mortem diagnosis is difficult and the true incidence of this underdiagnosed disorder is unknown. On CTA the morphology of the thrombus will in many cases be indistinguishable from PE. However, PAT is commonly unilateral, whereas massive pulmonary thromboembolism with unilateral proximal main pulmonary artery occlusion, total absence of emboli on the contralateral side is rare. Furthermore, a convex-margined total main pulmonary artery occlusion at its origin is not a feature of acute or subacute PE and has typically been reported in PAT [3, 6]. Another important differential diagnosis, primary sarcoma of the pulmonary artery, shares common clinical features with PAT. In fact pulmonary artery sarcoma is complicated by recurrent minor episodes of post-occlusive PAT containing tumour cells and by distal embolism as a slowly progressive disease process, rarely with an acute clinical onset [7, 8]. On late arterial CTA a hypervascular intraluminal tumour mass of pulmonary artery sarcoma will display contrast enhancement, whereas intra-arterial thrombus will remain low density. In addition, expansile intraluminal growth is detectable on CT by the convex shape of the proximal tumour margin and by tuberous vessel expansion best seen at the segmental artery level. In our case, the absence of thrombus enhancement – while having a massively enhancing tumour – and the streamline-shaped concave proximal margin made proximal intra-arterial tumour thrombus and therefore the presence of a primary pulmonary artery sarcoma very unlikely.
The clinical workup of patients with PAT due to primary pulmonary malignancy is difficult for two reasons: firstly, once a working diagnosis of venous thromboembolism is entertained the diagnosis of underlying pulmonary neoplasia with vascular involvement contraindicating thrombolytic therapy may be significantly delayed. Thrombolysis or therapeutic anticoagulation, which is required in non-malignant PAT as well as in PE to prevent distal propagation of thrombotic material and recurrent embolism is usually contraindicated in patients with underlying pulmonary malignancy to prevent serious bleeding complications. Secondly, biopsy material from bronchoscopy frequently does not allow differentiation between primary parenchymal and pulmonary arterial sarcomatous tissues, and surgical or post mortem confirmation may be required for final diagnosis. Due to the irresectable status in our case the final diagnosis would not have affected patient management, as haemorrhage may occur in both entities with vascular invasion.
Received for publication June 27, 2003.
Revision received September 15, 2003.
Accepted for publication November 19, 2003.
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References
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Abstract
Introduction
