EComment: Pulmonary artery sarcoma: a challenging diagnostic dilemma

Abstract

Bhagwat and colleagues have reported on the successful surgical treatment of a patient with primary pulmonary artery sarcoma [1]. We would like to share our experience relating to a 62 year female and to add a brief comment as well [2]. We reported on a patient with primary pulmonary artery sarcoma presenting initially with weight loss, shortness of breath, palpitations, and dysphagia. The diagnostic approach consisted of echocardiography, CT scan, MRI and coronary angiogram, and the differential diagnosis included pulmonary emboli, chronic thromboembolic disease and pulmonary artery tumour. The patient underwent exploratory median sternotomy under cardiopulmonary bypass (CPB), logistic EuroSCORE 6%, and the entire tumour was resected with an extensive tumour endarterectomy. However, as a fibrotic area in the right ventricle was left, this was considered to be a palliative procedure and the patient was referred for chemotherapy and radiotherapy [2]. Pulmonary artery sarcoma presents a diagnostic challenge, as the main presenting complaint of patients is progressive dyspnoea. Dyspnoea is a quite common symptom in diseases of the heart and lungs and therefore a high index of suspicion is necessary to make the diagnosis early, and to proceed with surgery and radiotherapy and/or chemotherapy [2]. Since 1923, when the first case was described, until 2006, no more than 210-220 cases have been reported [2]. Pulmonary artery sarcomas arise from pluripotent intimal cells. They are most frequently located within the pulmonary trunk, spreading into the proximal and distal pulmonary arteries. Typical onset age is 45-55 years, with reported age ranging from 13 to 86 years and a female to male ratio equal to 2:1. Due to the rarity of these tumours, they are often initially misdiagnosed as chronic thromboembolic disease leading to unnecessary anticoagulation of affected patients [2]. Preoperative histological diagnosis is generally not possible although, rarely, biopsy may be obtained via CT-guided transthoracic aspiration or, alternatively, via transvenous catheter suction biopsy with pulmonary angioscopy, as well as by transbronchial biopsy [2]. Endarterectomy via median sternotomy and CPB, with dissection of the intima and most of the media, may result in complete tumour removal and is the most commonly used and widely acceptable surgical strategy. Although in unilateral disease it is possible to attempt a pneumonectomy, in bilateral disease it seems to be of limited value [2]. Alternative surgical methods include total excision of pulmonary trunk with reconstruction, and heart and lung transplantation for unresectable tumours [3,4]. Palliative treatment includes debulking of the tumour, endovascular stent or bypass procedure [2,5]. The prognosis is poor: without surgery survival is 1.5 months. The median survival has been significantly improved with radical surgical resection and multimodality treatment [1,2]. Surgical treatment remains the cornerstone of management, offering definite diagnosis, improvement of symptoms and the best outcome. However, if curative resection is not possible, palliative resection still provides good symptom relief and can improve survival [2]. A high index of suspicion for early diagnosis is the key to successful treatment and prolonged survival for these patients presenting with no specific symptoms. Conflict of Interest: None declared