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Case
A 70-year-old female patient with known Parkinson’s disease and arterial hypertension presented with a progressive exertional dyspnoea over the last couple of months. She also suffered from night sweats and weight loss of 5 kg which the patient claimed to be intentional. Angina, syncope, cough, fever and haemoptysis were denied. Chest X-ray was performed and a right paratracheal mass was observed. Therefore, a CT thorax was indicated and showed a 12.6×6.5 cm right central mass with infiltration of the pulmonary artery. Furthermore, intrapulmonary artery opacity was detected and initially interpreted as a saddle pulmonary thrombus.
The patient was referred to our centre for monitoring and anticoagulation therapy. Further examination showed no signs of acute right heart failure, and deep vein thrombosis was excluded. Re-evaluation with angio-CT of the thorax revealed a neoplasm growing along the right pulmonary artery subtotally occluding both pulmonary arteries (figure 1). Transthoracic echocardiography showed no signs of pulmonary hypertension, normal right ventricle measurements, mild tricuspid regurgitation and reduced right ventricle function on tricuspid annular plane systolic excursion. CT-guided biopsy of the tumour was performed parasternal under local anaesthesia (figure 2). Histopathological examination showed undifferentiated pleomorphic sarcoma (UPS). Tumour cells were negative for pan-cytokeratin, desmin, caldesmon, S-100, CD34, CD 31, CD 45 and actin on immunohistochemical analysis. Thirty per cent of the tumour cells were positive for Ki-67. Due to the unresectable extensive nature of the tumour, the patient was referred for systemic therapy with doxorubicin and ifosfamide.
(A) Axial CT slice of the chest showing the tumour outgrowing the pulmonary artery invading into the right lung. (B) Coronal reformation illustrating subtotal occlusion of the main pulmonary artery.
CT-guided biopsy of the tumour under local anaesthesia as it invades the right lung.
Comment
Pulmonary artery sarcoma is an extremely rare malignancy with no recognised staging system. It is sometimes misdiagnosed as acute or even chronic pulmonary thromboembolism.1 The prognosis of unresectable or metastatic disease is poor with a median survival of 1.5–3 months while surgical treatment could only prolong survival to 8–36 months.1 2 The histological pattern of UPS (previously known as pleomorphic malignant fibrous histiocytoma) is usually seen in cases where the tumour invades outside the vessel wall, into the adventitia and further or in metastatic disease.2
Because of its low incidence and poor prognosis, optimal surgical and non-surgical strategies remain unclear.1 Doxorubicin-based chemotherapeutic agents are commonly used for unresectable pulmonary artery sarcoma. It was observed that these agents usually do not result in a substantial volumetric response on CT scans and they could prolong survival when pulmonary hypertension is well-controlled.3
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Footnotes
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.