Klin Onkol 2009; 22(4): 139-153.
Summary
Primary pulmonary sarcomas are rare diseases unlike lung carcinomas. The occurence of these sarcomas is between 0.013–0.40% of all malignant lung tumours. There are malignant mesenchymal tumours. They are flowing from the soft tissue of lung. The pulmonary sarcomas are heterogenic
group with various biological behaviour. Their morfologic structure does not digger from the sarcomas of soft tissue. The primary pulmonary sarcomas occur more often in childhood and in young people unlike lung carcinomas. Radiation and some toxic substances are noted risk factors.
Some gene mutations, infectious pathoghens and contraception have a possible impact on the origin of some types of the sarcomas. The current
hypothesisis, that most of the sarcomas, if not all sarcomas, stem from primitive multipotent mesenchymal cell by malignant transformation in
one or more lines. The diagnostic standard is biopsy from tumour with histologic and immunohistochemistry examination of a sample. The basic
diagnostic problem is exclusion of a secondary origin of sarcomatic cells in the lung, because pulmonary metastasis of extrapulmonary sarcomas
are more often than the primary pulmonary involvement. The optimal treatment is a resection of the tumour. The other therapeutic modalities are
radiotherapy and chemotherapy, but results of these modalities are unsatisfactory. There are various chemotherapeutic regimes, monotherapy or combination regimes. The basic cytostatics are doxorubicine, iphosphamide, dacarbazine. Problems of the chemotherapy are high toxicity and
relatively low curative effect about 20%. The first studies with biological treatment of the sarcomas of soft tissue have been published recently. This types of drugs could be a part of the complex management of these primary pulmonary tumours in the future. The primary pulmonary sarcomas
have mostly aggresive course and often recur. Their prognosis is usually not very good. The survival median is 48 months and 5- years survival ranges between 38 and 48%. Prognostic factors are the size of tumour, histological type, grading, clinical stage and measure of a surgery major.
