Malignant neoplasm developing in the lung tissues and giving multiple metastases to other organs, half of the cases with a lethal outcome, is called sarcoma of the lungs( very fewer than one percent of the total number of lung cancers).
A distinctive feature of this disease( in comparison with other types of malignant tumors) is increased aggressiveness, extreme rapidity of development, early metastasis and frequent relapses.
In an actively growing children's body, affected by lung sarcoma, all these processes occur most intensively. This is due to the high rate of division of connective tissue cells.
People with different age categories are able to get sarcoma of the lungs, but more often white people over forty are exposed to it.
What is this disease?
Starting from the connective tissues that make up the walls of the bronchi and interalveolar septa, the lung sarcoma is a fairly large tumor node that can occupy part of the lobe( most often the upper lobe) or the entire lobe of the lung.
In some cases, the tumor can affect the lung entirely.
Sometimes a malignant formation has a special capsule that delimits it from surrounding lung tissue.
In some cases, the tumor infiltrates( fills its cells) with the parenchyma of the lung, and then sprouts into the tissues of the large bronchi.
The lung sarcoma can be:
- Primary. In this case, the tumor develops from the connective tissues of the most affected lung.
- Secondary. This pathology is a consequence of metastasis of the sarcoma that affects other organs. Secondary sarcomas occur much more often than primary and are detected much earlier.
Varieties of
Different levels of malignancy make it possible to distinguish the following types of lung sarcoma:
- Low grade malignancy. Tumors of this species originate from highly differentiated mature cells, the division of which occurs extremely slowly. They are characterized by a low content of cancer cells and a large number of unformed connective tissue( stroma).
- High grade malignancy. The impetus for the appearance of tumors of this species is provided by rapidly and frequently dividing low-grade cells. Such tumors contain a large number of malignant elements and quite a few stroma. Neoplasms of this species are well supplied by a developed vascular network. They are characterized by the presence of numerous necrotic foci.
Given the presence of connective tissue in a large number of elements and anatomical structures that make up the lung, each of which can give rise to the development of the tumor process, the lung sarcoma is divided into two large groups:
- Highly differentiated. This diverse group is represented by angiosarcomas, fibrosarcomas, carcinosarcomas, hemangiopericytomas, rhabdomyosarcomas, neurosarcomas, liposarcomas, chondrosarcomas, lymphosarcomas and a number of other tumors. The morphological diversity of this group of sarcomas gives some researchers a basis for treating lung sarcoma not as an independent nosological form, but as a collective concept.
- Low-differentiated. Tissues of low-grade tumors are formed from cells at different stages of development, so it is not possible to determine their subsequent functions. No one can predict which part of an anatomical structure will be one or another cell of a cancerous tumor. This explains the unpredictability of the flow of low-grade sarcomas. They are characterized by frequent and rapid metastasis of hematogenous( through the blood) pathway. The group of low-grade neoplasms is represented by round-celled, polymorphous-cell and spindle cell sarcomas.
Causes of
Any specific causes leading to the development of lung sarcoma have not been identified to this day, but there are a number of risk factors that provoke the disease.
Sarcoma of the lung can develop under the influence:
- Hereditary predisposition to the development of cancer. All members of the family, in whose anamnesis were cases of such diseases, are in the group at high risk. Regular preventive examinations will be able to detect the disease in the early stages of development and prevent a lethal outcome.
- Prolonged inhalation of air containing a large amount of carcinogenic substances( exhaust, soot).Smoking and other bad habits.
- Harmful environmental factors, in particular - high doses of radioactive radiation.
- Chemical carcinogens contained in household chemicals.
- Uncontrolled intake of medications not used by the doctor.
- Ionizing effects of ultraviolet rays. It is unacceptable for too long stay in the open sun and abuse of the solarium.
- Adverse environmental environment.
Symptoms of lung sarcoma
Clinical manifestations of sarcoma are almost identical to those of lung cancer. The intensity of their expression is determined by the size of the tumor, its location, histological type and stage of development. The ailment of the initial stage, as a rule, proceeds asymptomatically.
Sarcoma of the lungs is characterized by the following symptoms:
- Characteristic signs of total poisoning( intoxication) of the sick person's body: weakness, high fatigue, constant drowsiness, pale skin, increased sweating, complete lack of appetite, cyanosis of the fingers and lips, pallor of the nasolabial triangle.
- Stagnation of blood in the affected lung( most often the sarcoma affects the left lung) leads to hypertrophy of the right side of the heart muscle, coupled with the appearance of dyspnea.
- Constant signs of a cold. Patients are harassed by long-running pneumonia, from which no drugs are saved.
- Dry( "barking") cough, often resulting in hemoptysis.
- Strong pains in the chest, sensation of a foreign body in it.
- When metastasizing the tumor into the esophagus, a swallowing disorder - dysphagia - can develop. The patient experiences difficulties with swallowing both solid and liquid food.
- Slowness of voice.
- Seizures. Vomiting and nausea.
- Severe dizziness and headache.
- Continuous growth of malignant neoplasm.
With distant metastasis of the sarcoma, the specific symptoms of newly affected organs of may adhere to the above symptoms.
Stages of
In the clinical course of the lung sarcoma, the following stages are distinguished:
- The first stage disease is characterized by the presence of a clearly defined single unit or infiltrate, whose diameter does not exceed three centimeters. There are no metastases.
- The lung tumor, entering the second stage, has a diameter within six centimeters and metastasizes into the peribronchial nodes and lymph nodes of the lung root.
- Malignant sarcoma of the third stage has a diameter exceeding six centimeters, gives numerous metastases to the intrathoracic lymph nodes with subsequent germination into the pleura tissue.
- Malignant neoplasm of the fourth stage, metastasizing to other internal organs, can have any size and localization.
Diagnosis
The insidiousness of all cancers is the difficulty of their early diagnosis, because in the initial stage they do not betray their presence. As a result, the patient, who has experienced painful symptoms, turns to the doctor already when the disease enters one of the last stages and is difficult to treat.
Accidental detection of tumors with a routine examination or with a routine X-ray examination can be classified as a happy exception.
A number of laboratory and instrumental studies are used to diagnose sarcomas of the lung:
- The analysis of blood( general and biochemical) allows to reveal signs of a progressing tumor process, manifested in increased ESR and the presence of anemia.
- To determine the classification of the tumor, its shape and size, computer and magnetic resonance imaging procedures are used.
- Radiography of the lungs allows us to clarify the parameters and shape of the affected organ, as well as the presence of malignant neoplasm and the nature of the damage caused to them.
- The recently used procedure of videotorakoscopy opens up new possibilities for more accurate diagnosis of primary sarcomas, because with its help it is possible not only to establish the nature and extent of the spread of the tumor process, but also to obtain a tissue sample( from the surface of the lungs take small scrapings) for further investigation( cytologicaland histological).
- The electrocardiogram allows you to monitor the functioning of the heart muscle.
- Using a radioisotope study, the boundaries separating healthy and diseased tissues are determined.
- The procedure of contrast angiography allows to assess the general condition of the circulatory system, as well as to establish the presence of irregularities in its work.
- To determine the histological type of tumor, the patient is subjected to a biopsy procedure.
Treatment of
Treatment of lung sarcoma can be successful only in an integrated approach that combines immunotherapy, radiotherapy, chemotherapy and surgical intervention.
- For the treatment of each patient, an individual treatment protocol is developed, compiled taking into account the results of all studies.
- In the treatment of lung sarcoma, surgical intervention is given a leading role. Depending on the stage of the disease, the age and condition of the patient, the histological type of the tumor, one of three possible types of surgery is chosen: lobectomy( removal of the lobe of the lung), pneumonectomy( removal of the entire affected lung) or segmentectomy( removal of any segment of the lung).
- If the patient is in serious condition or has contraindications for performing a cavitary operation, resort to radiosurgical removal of the tumor focus( the so-called cyberknife).With this method of treatment is not required to violate the integrity of the skin and chest, as cancer cells are exposed to X-rays. Modern equipment allows a specialist to accurately affect the tumor tissue, destroying them.
- The operation, as a rule, precedes the course of polychemotherapy. The impact of modern medicines( cytostatics) can significantly slow down the process of active division of cancer cells. With inoperable sarcomas, chemotherapy is the only treatment that significantly improves the patient's quality of life.
- Radiotherapy, directed both against the sarcoma itself and against its metastases, is often used together with chemotherapy. Not being interchangeable, both methods perfectly complement each other. Radiation therapy is often prescribed prior to surgery, since its effect can significantly reduce the size of the tumor and thereby increase the effectiveness of surgical intervention.
How long live and the survival prognosis
Sarcoma of the lung is a disease characterized by the highest percentage of mortality.
- Sarcoma is so aggressive and prone to recurrence that even with long-term treatment, being detected in the early stages, it leaves only 50% of patients alive for five years.
- A tumor diagnosed in the second stage kills 70% of patients.
- If treated with sarcoma, identified in the third stage of development, within five years, survive only a fifth of all patients.
- There is almost no chance for survival for patients with the fourth stage of sarcoma: almost all of them within three to four months are expected to die due to embolism, pulmonary insufficiency or a number of concomitant infections.
The video shows an operation to remove the lung: