Introduction:
Primary pulmonary sarcomas (PPS) are rare types of non-epithelial malignant tumours of the lungs. PPS can originate from mesenchymal elements of bronchial wall, vessels or pulmonary stroma. Since the introduction of immunohistochemical and molecular diagnosis, a significant improvement in description and classification of sarcomas has been achieved.

Aim:
The present report was aimed at describing the current state of knowledge concerning diagnosis and treatment of PPS.

Material and methods:
A literature review was conducted in context of PPS.

Results and discussion:
The majority of available literature concerning PPS is limited to reports based on single cases or small series. PPS not only are devoid of typical clinical symptoms, but also their pathomorphological diagnosis is difficult. Identification and differentiation of sarcomas is increasingly based on molecular diagnosis. The most efficient method of treatment is a radical surgical resection. Neoadjuvant and adjuvant therapy is applied according to the soft-tissue sarcoma guidelines, however, due to the rarity of PPS cases, it is impossible to assess its impact on overall survival.

Conclusions:
Early diagnosis of PPS is difficult. Prognosis of PPS is poor, because surgery – the most efficient method of PPS treatment is not possible in many cases. Prognostic factors in PPS include the size of the tumour, histological grading of malignancy and clinical staging according to the lung cancer TNM.