Lung sarcoma – extremely aggressive malignant tumor developing from unripe soyedinitelnotkanny elements of a lung. Symptoms of sarcoma of a lung are similar to displays of cancer of lung, but differ in more rough progressing. Subjective symptoms can include short wind, cough, swallowing violation, fever, strong fatigue, perspiration. Often the syndrome of the top hollow vein, pleurisy comes to light, perikardit, pneumonia, an osteoartropatiya. In the diagnostic purposes X-ray, KT and MPT of lungs, a bronkhoskopiya, a tonkoigolny biopsy, etc. is carried out. For treatment of sarcoma of a lung surgical methods (radical and palliative), chemotherapy, radiotheraphy are used.
Lung sarcoma – a high-malignant tumor of an easy mesodermal origin which source soyedinitelnotkanny structures of walls of bronchial tubes and interalveolar partitions usually are. Sarcoma belongs to rare types of malignant tumors of a lung, 1% of all types of cancer of lung falls to its share. Sarcoma of a lung differs from other malignant new growths in rapid growth and early metastasis. Strikes young men (20-40 years) more often, however can develop at persons of any gender and age. At children sarcoma of a lung proceeds especially aggressively that is connected with age activity of growth of connecting fabric. The oncology and pulmonology are engaged in search of optimum ways of treatment of sarcoma of a lung.
Lung sarcoma reasons
Development of primary sarcoma of a lung can be initiated by various factors. It is experimentally confirmed that in an etiology of sarcomas of various localizations into the forefront can act the ionizing radiation, ultra-violet radiation, chemicals, smoke and impurity of air.
The smoking patients enter into group of the increased danger on developing of sarcoma of a lung; the workers contacting to production carcinogens (asbestos, aniline dyes, pesticides, etc.); the persons living in the industrial cities with high content of exhaust gases and soot in air. Abuse of suntan as natural (under beams of the sun), and artificial is unsafe (in a sunbed). The role of the burdened heredity is proved: in the presence of family cases of sarcoma the risk of its manifestation at other blood relatives is several times higher.
Secondary damage of lungs can represent metastasises of osteogene sarcoma, sarcoma of a uterus, sarcoma of soft fabrics, a mammary gland, a sredosteniye, etc. In most cases sarcoma of a lung settles down in peripheral departments, affecting the top shares, but can affect also all lung; it is sometimes localized in large bronchial tubes in the form of polipovidny outgrowths. The tumor has an appearance of massive knot of a rounded or polycyclic shape, a softish consistence, white-pink color on a section. Sarcoma metastasis occurs in the hematogenic way more often.
Sarcoma of lungs is divided by the origin on primary (growing from own tissues of a lung) and secondary (metastatic, extending in lungs from the remote primary center – muscles, bones, genitals, etc.). In the histologic relation primary sarcomas of a lung can be high-differentiated (low degree of a zlokachestvennost – with low mitotic activity of cages, big specific weight of stromalny elements) and low-differentiated (high degree of a zlokachestvennost – with the high speed of cell fission, prevalence of tumoral elements which is well developed by vascular network, the necrosis centers).
The high-differentiated sarcomas of lungs differ depending on a source of blastomatozny process and can be presented by the following types (in decreasing order of frequency):
- the angiosarkoma – comes from walls of blood vessels
- the fibrosarkoma - is formed of bronchial and peribronkhialny connecting fabric
- the limfosarkoma – develops from lymphoid fabric
- neurosarcoma – affects soyedinitelnotkanny elements of nervous tissue
- the hondrosarkoma – comes from cartilaginous elements of bronchial tubes
- the liposarkoma – is formed of fatty tissue
- the leyomiosarkoma and rabdomiosarkoma – come respectively from gladkomyshechny and cross-striped muscular tissue
- the gemangioperitsitoma – arises from peritsit - the cages which are a part of walls of capillaries.
Undifferentiated sarcomas of lungs share on:
- polymorphic and cellular.
Stadirovany sarcomas of a lung it is based on degree of prevalence of a tumor and assumes allocation of 4 stages:
I - limited infiltrate or knot in a lung to 3 cm in the diameter with lack of metastasises;
II – a tumor of 3-6 cm in size in the diameter with existence of single metastasises in peribronkhialny lymph nodes and lymph nodes of a root of a lung;
III – a tumor more than 6 cm in size in the diameter with existence of metastasises in sredosteniye lymph nodes, a pleura invasion
IV - a tumor of any sizes in combination with the remote metastasises.
Lung sarcoma symptoms
The clinical picture of sarcoma of a lung reminds clinic of cancer of lung, but unlike the last develops more promptly. Concrete symptoms are defined by a stage, localization and a histologic structure of a tumor. At intrabronkhialny type of growth of a tumor there can be a bronchial tube obturation – in this case the clinical picture reminds clinic of the central cancer of lung.
In typical cases of patients the progressing short wind, breast pains, cough with a phlegm and streaks of blood, temperature increase of a body, perspiration disturbs. The general health suffers because of constant weakness, bystry fatigue, lack of appetite. At a tumoral invasion of a gullet the dysphagy develops, at a compression of venous trunks – a syndrome of the top hollow vein. Germination of a pleura is followed by development of hemorrhagic pleurisy, an external cover of heart - a hemorrhagic perikardit.
Sometimes sarcoma of a lung comes to light at detailed inspection concerning long or recurrent pneumonia. In certain cases in a clinical picture not signs of damage of lungs, but a paraneoplastic syndrome (a pulmonary osteoartropatiya) – periostita, arthritises, articulate pains, deformation of fingers of hands dominate. In late stages cancer intoxication, a cancer kakheksiya, anemia develops.
For confirmation of the diagnosis consultation of the oncologist and thoracic surgeon is held, data of the anamnesis are specified, tool inspections are appointed. It is possible to suspect lung sarcoma on bystry progressing of a tumor at persons of young age. In detection of sarcoma of lungs beam methods have the leading value (a X-ray analysis, KT of lungs). On roentgenograms the roundish or deckle-edged oval shadow which is more often localized in peripheral departments is defined. At dynamic control bystry increase in a tumor is noted. KT and MRT of lungs allow to analyse in more detail parameters of a tumor and to make a process stadirovaniye.
With an intrabronkhialny growth polipovidny sarcoma is well visualized during a bronkhoskopiya. It has an appearance of white-pink formation of irregular shape without capsule. The histologic diagnosis is specified by means of an endoscopic biopsy or a tonkoigolny transthoracic biopsy which is carried out under control of a computer tomography. At detection of ekssudativny pleurisy carrying out a pleural puncture with a cytologic research of the received material is expedient. In certain cases at sarcoma of a lung resort to performance of a diagnostic torakoskopiya or eksplorativny torakotomiya.
Differential diagnosis of primary sarcoma of a lung is performed with peripheral cancer of a lung, metastatic tumors, tuberkulomy, echinococcosis and not parasitic cysts of a lung, good-quality and malignant timomy.
Treatment of sarcoma of a lung
At the choice of rational medical tactics the lung sarcoma stage, its type and localization is considered. In operable cases lobectomy or a pulmonektomiya with a limfadenektomiya is carried out. In the preoperative period the polychemotherapy course is, as a rule, appointed. Radiation therapy is usually applied as a part of complex treatment (supplements surgical and chemotherapeutic treatment) as sarcoma of a lung is less sensitive to radiotheraphy. The innovative technology of selective internal radiotheraphy (SIRT) – introduction of radioisotopes through a catheter takes root into the lung vessels going to a tumor.
If the radical resection is impracticable, carrying out palliative operation for elimination of pathological symptomatology and increase in short-term survival of patients is possible. Besides, at inoperable tumors the chemotherapy separately or in combination with radiation therapy, immunochemotherapy can be appointed.
Sarcoma of lungs – an oncological disease with potentially adverse forecast. Distinctive feature is tendency to a retsidivirovaniye and metastasis of sarcoma of a lung that causes low interest of survival of patients with this diagnosis. Five-year survival at an initiation of treatment at the first stage makes 50%, on the third – 20%. At detection of primary sarcoma of a lung at late stages or secondary damage of a lung patients perish within several months.