The Importance of Differential Diagnosis: Intimal Sarcoma of the Pulmonary Artery

Keywords

Pulmonology; Pulmonary disease; Intimal sarcoma; Dyspnea

Case Description

Pulmonary artery intimal sarcoma is an extremely rare type of malignant tumour, which mimics pulmonary thromboembolism. A misdiagnosis of pulmonary thromboembolism is usually made because both pathologies have a similar clinical presentation, and a repletion defect in the pulmonary arteries is identified in the chest computed tomography.

Despite its low incidence, it is essential to suspect this entity, since therapeutic management is different from pulmonary thromboembolism and early diagnosis can increase the life expectancy of these patients. We present the case of a 58-year-old woman, with no personal history of interest who consults for left pleuritic pain, dyspnoea and dry cough of one month of evolution.

A CT pulmonary angiogram is performed, which is interpreted as a pulmonary thromboembolism, so anticoagulant treatment is scheduled. Two months later, she comes again for worsening dyspnea and pleuritic pain. The chest X- ray shows a newly appeared left pleural effusion.

After this, a diagnostic thoracocentesis is performed with a result compatible with exudate of lymphocytic predominance without complication data [1]. A new chest CT without intravenous contrast is requested in which the occupation of the light of the left main pulmonary artery persists (Figure 1).

Figure 1: A: Chest CT without intravenous contrast is requested in which the occupation of the light of the left main pulmonary artery persists. B: shows an increase in metabolism in the pleura in relation to pleural implants.

On this occasion, it can be seen that the material is calcium density, which may correspond to an endovascular involvement by an intimal sarcoma. Given these findings, the study is extended with a Positron Emission Tomography (PET-CT), which reinforces the suspicion and also shows an increase in metabolism in the pleura in relation to pleural implants.

An ultrasound-guided thick needle biopsy (BAG) of the left pleural calcified lesion was performed with anatomopathological result of osteoforming fusocellular sarcoma with endothelial phenotype (osteogenic variant) and immunohistochemical study [2]. After confirming the diagnosis of suspicion and in the presence of disseminated lymph node and pleuropulmonary disease, the patient is referred to the oncology service to assess the option of participating in a clinical trial given the impossibility of surgical option.

References

1. Jiang S, et al. Pulmonary artery intimal sarcoma misdiagnosed as pulmonary embolism: A case report. Oncol Lett. 2017;13:2713-2716.

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2. Bhagwat K, et al. Diagnostic enigma: primary pulmonary artery sarcoma. Interact Cardiovasc Thorac Surg. 2012;14:342-344.

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