A rare and extremely dangerous disease of the spine, it is difficult to treat and poorly diagnosed, the number of deaths is very high. Such problems are explained by the anatomical and physiological characteristics of the spine and the proximity of vital human organs to it. According to the international classification ICD-10, the disease is assigned code C41.2. Medical standards of care have been developed according to the assigned code.
Spinal sarcoma
The recommendations have three separate groups.
- Special examinations to make an accurate diagnosis of the disease at stages I–IV . Based on the data obtained, a final diagnosis is made and the patient is prepared for treatment. The standard is being developed for primary health care.
- The second standard also applies to primary health care and is used during dispensary monitoring of the patient’s condition during treatment of the disease.
- The third standard was created for specialized care for patients and describes the algorithm for the actions of doctors. Used to monitor the use and effectiveness of various types of chemotherapy.
Sarcoma of the spine on an MRI image
General symptoms and clinical signs of spinal sarcoma
Without special tests and examinations using modern devices, it is impossible to make an accurate diagnosis. The reason is that there is too much variation in the clinical signs of the disease; there are many similar symptoms to other pathologies. What factors influence the clinical picture of sarcoma?
- Where exactly is the affected vertebra located, which element suffered the most, how did this affect the condition of the spinal cord. These are very important factors not only for diagnosis, but also for choosing a treatment protocol according to available standard recommendations. Lesions can be in the cervical, thoracic and lumbar spine. Tumors in the sacrum, according to the classification, belong to another category of oncological diseases.
Causes of spinal sarcomas - What type of tumor (intramedullary or extramedullary) is developing ? The first type of neoplasm is located directly in the brain column, the second develops nearby. The specific location of the tumor is classified separately. It may be adjacent to the front, back, or side of the spinal cord.
- The rate of growth of a malignant neoplasm, the actual size of the free space in the medullary canal of the spine before the start of compression . The condition of the patient’s body is also taken into account.
What does spinal sarcoma look like?
Important! If pathologies develop within the spinal cord (intramedullary tumors), then negative symptoms appear quickly and have an avalanche-like development. The patient's standard of living decreases significantly. Severe pain must be localized with narcotic painkillers. In addition to critical pain, dysfunction of the upper or lower extremities and problems with internal organs appear.
The intensity of tumor growth is related to the nature of their formation. Metastatic neoplasms have rapid dynamics and damage not only bone tissue, but also nerve bundles and the spinal cord. Without proper effective treatment, death occurs within 2–3 months. The treatment is very complex, and positive results are rarely observed. Primary tumors of the bone tissue of the spine can develop for more than a year, doctors have time to develop an optimal treatment plan, and surgical intervention is often performed.
Osteogenic sarcoma of the spine
Is osteosarcoma cancer?
Official medicine in Russia and some other countries refers to cancer as malignant tumors - life-threatening neoplasms that develop in epithelial cells contained in the skin or mucous membranes and lining the inner surface of organs. Osteosarcoma is not cancer, but an oncological disease. It is formed from osteoblasts involved in the creation of bones, and its cells are also able to divide uncontrollably, spread throughout the body and create metastases - additional foci of disease in various parts of the body.
The most common spinal sarcomas
The disease has several types; an accurate diagnosis can only be made in specialized clinics with the most modern equipment.
Table. Types of vertebral sarcoma.
| Type of vertebral sarcoma | Short description |
| Osteosarcoma | The rarest disease, the percentage of all osteogenic neoplasms does not exceed 2%. The disease most often affects teenagers. During skeletal development, pathogenic points are formed, which are extremely rarely diagnosed in patients aged approximately thirty years or older. Medicine has described cases of the disease in older people, but they are extremely rare and are considered an exception. Most scientific medical workers attribute such diseases in adults not to primary spinal tumors, but to malignancy during Paget's disease or chondromatosis. Only one vertebra is affected; over time, the disease metastasizes to the lungs or brain. Osteogenic sarcoma very rarely manifests as bone metastases. |
| Chondrosarcoma | The disease is also a rare type of spinal tumor; the disease is found in no more than 3% of all patients. According to the clinic, it can be primary or secondary. Primary develops from unchanged spinal cartilage, secondary as a consequence of other types of malignant tumors. It almost never occurs in children; the vast majority of patients are over thirty years old. Men suffer more often than women, the reason for this phenomenon is still unknown to science. The younger the patient is, the faster the disease progresses; its course is rapid and requires the immediate use of a complex of complex special treatment. Chondrosarcoma primarily affects the sacrum or vertebrae of the lumbar spine. |
| Ewing's sarcoma | An extremely aggressive type of disease, difficult and late to detect. At the time of diagnosis, up to 50% of patients already have distant sites with metastases, which greatly complicates treatment. Fatalities exceed 80%. This type of sarcoma appears only in children. It almost never occurs in subjects over thirty years of age. Men suffer much more often than women. At the initial stage, they resemble a thickened node that is located in the vertebral body. The neoplasm progresses and over time grows into nearby elements of the spine. Metastases quickly appear in other vertebrae and migrate to the lungs. Lymph nodes and bronchi are affected very rarely, the clinic of such pathologies is little known. |
| Angiosarcoma | A malignant neoplasm that appears due to vascular cancer. It progresses very quickly, the rate of cell growth is much higher than the rate of cell growth of benign vascular tumors. Metastases destroy the bone structure and necessarily migrate to soft tissues, which causes their pathological changes. A complex type of sarcoma necessarily provokes severe problems of a neuralgic nature. The vertebrae become very fragile, they break even under minor loads, and mechanical damage is complex. |
| Rustitsky-Kahler disease | A malignant neoplasm consists of plasma blood cells modified at the gene level. The bone marrow is mainly affected. Most often found in older people. The disease affects many vertebrae at the same time, involving not only the spine, but also the ribs and tubular bones. Single lesions are rarely observed. The pathology results from complex fractures of altered areas of bones; the clinical picture of partial curvature of the skeleton is described. Paraplegia often develops due to compression of the spinal cord. |
Surgery
The most radical way to combat soft tissue sarcoma is surgery. If the cancer focus is on the arms, legs or torso, the so-called “case principle” is used. According to this principle, the cancer focus, growing inside a special capsule, which consists of muscles and fascia, is removed along with this capsule (it is also called a case, hence the name of the principle). If the described method is not suitable, the “zoning principle” is used, in which that part of the healthy tissue components that is close to the tumor is eliminated. This way it is possible to reduce the risk of relapses, because even on tissues that are seemingly untouched at first glance, there may be micro-foci.
Amputation of an arm or leg is done only when there is massive damage to the arteries or nerves. Such a radical approach is not recommended in the presence of metastatic organ damage. If such complications occur, the emphasis is on “chemistry” and rays, which can reduce the size of the lesion for subsequent surgery.
General clinic for various spinal sarcomas
Oncological pathology develops rapidly; the duration of the anamnesis can be only a few months with neoplasms of soft tissues and the brain. The patient has a little more time during tumors of the bone tissue of the spine.
MRI of the lumbar spine. T1-weighted sagittal MRI. Metastasis to L3 vertebra
If you want to learn in more detail how the MRI procedure of the spine is performed, and also consider when a magnetic resonance examination is indicated, you can read an article about this on our portal.
Patients feel pain of varying intensity; it can be constant or with sudden attacks of intensification. Such pain is almost impossible to localize with ordinary analgesics; it is necessary to use drugs containing narcotic substances.
Spinal pain
The mobility of the spine is limited, patients often take unnatural positions, and it is in this position that the pain subsides a little. Neurological complications appear quickly, patients suffer from paresis and disturbances in the physiological functions of the pelvic organs. The general condition of the body deteriorates sharply.
Diagnostic methods
Based on the symptoms, one can only suspect bone cancer or another disease. In order to get an accurate diagnosis, the doctor will prescribe an examination, which may include:
- Radiography . A quick and simple diagnostic method, in some cases it helps to immediately identify a tumor. If there is a suspicion of metastases in the lungs, a chest x-ray is performed.
- Magnetic resonance imaging (MRI) and computed tomography (CT) help to examine the tumor in more detail and detect growth into neighboring tissues.
- Positron emission tomography . A weak radioactive substance is injected into the body in safe doses, which accumulates most strongly in tumor cells. Then photographs are taken. This allows not only to examine the main tumor, but also to detect metastases in different parts of the body.
- General and biochemical blood tests. It is used as an additional diagnostic method to assess the general condition of a person. They can also indirectly indicate the degree of tumor aggressiveness.
- Biopsy. The most accurate diagnostic method. It is always used, even if other studies already conclusively show that the patient has bone cancer. A biopsy helps establish a final diagnosis, distinguish osteosarcoma from other tumors, and determine the degree of malignancy. The doctor may take a tissue sample with a needle or through an incision.
Stages of development of sarcoma
Medical science describes four stages of tumors, each of which requires specific treatment methods. The general trend is that the earlier the fight against malignant neoplasms is started, the higher the patient’s chances of a favorable outcome.
First stage
Unfortunately, at this stage the disease is diagnosed extremely rarely. Most often, pathology is discovered by chance during examinations for suspected other diseases. In the first stage, patients have a great chance of recovery. But in any case, it will take a lot of time and effort on the part of both doctors and the patient. At this stage, the tumor does not spread beyond the areas of primary localization, metastases are completely absent, and the lymph nodes do not react with thickening and inflammation. The temperature does not rise.
Front view of a spinal sarcoma (Panel A) and lateral view (Panel B). Tumor (T) and spinal cord (SC) are labeled
Second stage
The malignant tumor increases in size, the rate depends on the type of sarcoma and the state of the immune system. Tumors can extend beyond the vertebrae and compress nearby soft tissue, including the spinal cord or nerve bundles. Pain occurs periodically, but it is impossible to associate it specifically with sarcoma without special tests and examinations. Metastases have not yet been observed.
Third stage
The disease enters a severe phase, and the chances of a cure are rapidly decreasing. Primary metastases are found in soft tissues, malignant cells migrate through the lymph nodes. The immune system cannot cope with them, and nodes of varying sizes and pain appear on the lymph nodes. The state of health worsens, the pain increases and becomes continuous.
X-ray of spinal sarcoma
Fourth stage
The most severe stage, the mortality rate approaches 100%. The tumor is significant in size and is clearly visible on ordinary x-rays. The patient suffers severe pain, and the physiological functions of vital organs are quickly disrupted. Death becomes inevitable. Doctors use only symptomatic treatment to minimize pain and slightly improve the quality of the patient’s last days of life.
At the last stage, death is inevitable
If you want to learn new information about what types of spinal sarcoma there are, as well as consider symptoms and treatment methods, you can read an article about this on our portal.
Diagnosis of pathology
Unfortunately, a standard scheme for diagnosing this oncopathology has not been developed to this day. The clinical picture may be similar to a regular bruise or swelling resulting from sprains. For this reason, it is impossible to accurately detect sarcomatous soft tissue lesions through external examination. But an external examination of the patient and palpation of problem areas is also extremely important, because this helps to raise at least rough suspicions. Thanks to palpation, the doctor can find out the approximate dimensions of the tumor and the depth of its localization. In addition, this method assesses the degree of involvement of nearby tissue structures.
Further diagnosis involves undergoing the following procedures:
• histological analysis of the biopsy; • x-ray examination; • CT; • MRI; • angiography.
In the presence of cancer lesions localized in the extremities and with a diameter greater than 5 cm, a biopsy is a mandatory procedure. As a rule, a subcutaneous method is used - fine needle aspiration. The needle is inserted into the affected area so that no difficulties arise during further treatment (surgeries, radiation treatment). An open biopsy is performed less frequently (if the tumor lesion is located in the deep tissue layers). This procedure is dangerous in its own way, because it can provoke the spread of cancer cells through the bloodstream. The extracted material is examined in laboratory conditions. Thanks to this analysis, it is possible to find out the histological appearance of the cancer lesion and prescribe effective treatment. For imaging purposes, an MRI is usually prescribed. With its help, you can clearly determine the contrast between the lesion, muscles and blood vessels.
Treatment options for spinal sarcoma
Treatment of a disease is a complex and long process; success largely depends on the timeliness and correctness of the diagnosis, the condition of the body, the technical equipment and qualifications of the doctors of the medical institution. The choice of method is made taking into account the patient’s condition, the type of sarcoma and the stage of its development.
- Chemotherapy. The most common treatment method, modern drugs can effectively fight malignant tumors in the early stages of pathology development. Chemotherapy shows the highest rates of effectiveness in the treatment of osteosarcoma and Ewing's sarcoma. For other types of pathologies, a combination of chemotherapy and radiation therapy is often used.
Chemotherapy - Targeted therapy . The most modern treatment method minimizes the negative effects of taking medications. The method is based on precise therapy; the causes of tumor growth are localized with minimal harm to surrounding healthy tissue.
- Radiation therapy with simulated intensity. Efficiency depends on several factors: the correctness of the diagnosis, the capabilities of the body’s immune system and the technical characteristics of the device. New devices allow focusing beams only on damaged areas, accurately adjusting the depth and area of exposure, and automatically control the intensity of the beams. Such opportunities significantly increase the effectiveness of radiation therapy and reduce negative consequences.
Radiotherapy-radiation therapy - Surgery . Only some types of spinal sarcoma are surgically removed; cutting off pathological tissue is performed by experienced surgeons after carefully preparing a surgical plan. The results can be very positive; in some cases, after surgery, patients are prescribed a course of chemotherapy.
- Brachytherapy . It is used at the most severe stages of the disease to eliminate deep-lying tumors. It is impossible to get to them by ordinary means. The essence of the method is that a capsule with radioactive elements is injected into the tumor. They generate rays that destroy the tumor or slow its growth.
Brachytherapy
The prognosis for each patient is individual; with favorable and intensive treatment, the five-year survival rate reaches 50%. Regular medical examinations make it possible to promptly notice relapses and take appropriate actions to eliminate them.
Discussion
Achieving local control of highly malignant tumors such as neurogenic sarcoma is usually associated with a large number of complications, but partial tumor removal leads to a large number of local relapses and can negatively affect the quality of life of patients [10]. Neurofibroma of a plexiform structure in neurofibromatosis type 10 has almost 100% potential for malignant transformation into PVD. Angiomatous and epithelioid neurofibromas, as well as simple noncellular schwannomas, degenerate less frequently [11]. Partial removal of malignant neurogenic tumors increases the possibility of transformation with increasing grade of malignancy and leads to rapid local recurrence and spread. In this regard, the primary biopsy is of no small importance for determining the treatment tactics for a neurogenic tumor of the sacrum, and en-bloc resection should be the obvious choice of the surgeon in most cases when identifying potentially aggressive neurogenic tumors [2, 6, 12-15]. At the same time, the results obtained in this study indicate that sacrectomy is associated with a number of disabling complications [6, 9].
Over the past decade, it has become possible to reduce surgical aggression in patients with malignant tumors through the use of radiation therapy. Stereotactic radiation therapy has become an effective method of local control for PVD [17].
Stereotactic radiation therapy (SRT) involves a single or fractional high-precision delivery of a large dose of ionizing radiation to a target in order to achieve the desired biological effect in the irradiated volume with minimal impact on surrounding tissue. Local impact is achieved through the use of a large number of beams, high conformity and a sharp reduction in dose outside the target [18].
A system that is capable of performing SLT is CyberKnife. This system, combining a small linear electron accelerator with an energy of 6 MV, fixed on a robotic manipulator with six degrees of freedom, and an X-ray navigation system, appeared as a result of a number of works carried out in the 90s of the XX century [5, 7, 10, 20] .
Radiotherapy is an integral part of the complex treatment of high-grade sarcomas. Although sarcomas of bone and cartilage tissue are considered completely resistant to ionizing radiation, radiotherapy as an adjuvant to surgical treatment improves local tumor control rates and affects overall survival [5].
The overall survival of patients in the analyzed group of neurogenic sarcomas was less than 50%. This is quite consistent with the data of the world literature and is explained by the high degree of malignancy of PVD. Therefore, the most important thing for patients with signs of this disease is the optimization of the treatment approach strategy and the choice of optimal tactics. For patients with locally aggressive tumors, local tumor control is more important. However, the results of the analysis of the relapse-free survival period of patients with aggressive neurogenic tumors are not entirely reliable due to the small volume of material.
Publications in recent years indicate the need for chemotherapy as a treatment option for patients with aggressive tumors during the period of generalization of the process [19, 20]. However, according to publications, 2-year survival is observed in only 50% of patients.
Chemotherapy treatments
For soft tissue sarcoma, “chemistry” can be used as the main or auxiliary method. Often, this therapy involves the combined use of several chemotherapy drugs. The most commonly used drug combination is ifosfamide and doxorubicin. Other medications are sometimes used: dacarbazine, methotrexate, vincristine. The most serious complications after chemotherapy are: damage to the heart muscle, abnormalities in the functioning of the ovaries in women and testicles in men.