Spinal sarcoma are a group of malignant tumors originating from immature connective tissue. It can affect bones, cartilage, blood vessels and other connective tissue structures of the spine. Manifested by pain, pelvic disorders, impaired sensitivity and movement. The clinical picture is determined by the type of spinal sarcoma, the level of lesion, the location of the tumor relative to the spinal cord and the degree of damage to the spinal cord. The diagnosis is made taking into account anamnesis, complaints, examination data, results of radiography, CT, MRI, biopsy and other studies. Treatment – surgery, chemotherapy, radiotherapy.
Iinformation
Spinal sarcomas are a group of rarely occurring connective tissue malignant tumors of the spine characterized by explosive growth and rapid progression. Despite the insignificant share in the overall structure of oncological morbidity, spinal sarcomas are the second cause of death after cancer in patients with malignant neoplasms. The cause of a large number of deaths are infiltrative growth, early metastasis and frequent recurrence. Spinal sarcomas are particularly aggressive in children and adolescents, which is due to the rapid growth of connective tissue structures in this age period. Men are more likely to suffer. Treatment is carried out by specialists in the field of oncology, vertebrology and neurology.
Causes
The causes of the development of spinal sarcoma are not precisely established. It is assumed that the disease occurs under the influence of a group of factors, which include some DNA and RNA-containing viruses, ionizing radiation, contact with carcinogenic substances, etc. Scientists note that spinal sarcoma develops more often in people who have suffered fractures and bruises of the spinal column in the past. At the same time, most researchers believe that trauma is not the direct cause of the development of spinal sarcoma, but only provokes the growth of abnormal cells already present in the body.
There is scientific evidence in favor of the theory of unfavorable heredity. Intensive growth of bone, cartilage and muscle tissue is important in children and adolescents. It has been established that osteosarcoma and Ewing’s sarcoma usually develop in children and adolescents and are extremely rare in patients older than 30-35 years. However, some types of spinal sarcomas mainly affect people over 30 years of age. It is assumed that in such cases, some diseases of the spine may have a certain significance. With the development of spinal sarcomas in people older than 40-45 years, malignancy of a benign tumor or the development of the disease against the background of an inflammatory or degenerative-dystrophic process is usually assumed.
Classification
Taking into account the origin, the following types of spinal sarcomas are distinguished:
- Osteosarcoma – occurs directly from the bone tissue.
- Chondrosarcoma – comes from cartilage tissue.
- Ewing’s sarcoma – originates from the reticuloendothelial tissue of the bone marrow.
- Angiosarcoma – occurs from the tissue of the vessels that provide blood supply to the spinal column.
- Periosteal fibrosarcoma – occurs from the outer layer of the periosteum.
Along with the listed spinal sarcomas, oncological lesions of the connective tissue of the vertebral column include myeloma – a malignant tumor of plasma cells close to leukemia, the foci of which are often found in the vertebrae. In addition, nodes that have arisen as a result of metastasis of sarcomas of other localizations may be detected in the spine. Such neoplasms are called metastatic sarcomas.
Taking into account the level of differentiation of cells, three types of spinal sarcomas are distinguished: highly differentiated, medium-differentiated and low-differentiated. Low-grade spinal sarcomas grow faster, metastasize earlier and recur more often. The lower the level of differentiation, the more unfavorable the prognosis. Taking into account the level of lesion, tumors of the cervical, thoracic, lumbar, sacral and coccygeal spine are isolated, taking into account localization – neoplasms located on the anterior, posterior and lateral surfaces of the spinal cord.
Symptoms
The first symptom of the disease is usually pain. Initially, the pain syndrome is mild or moderate, the pain is intermittent, usually intensifying at night. Unlike pain syndrome caused by degenerative-dystrophic processes, pain in spinal sarcoma does not decrease at rest. Due to the erased symptoms, the lack of oncological alertness towards young patients and the incorrect interpretation of the manifestations of the oncological process (for example, assumptions about the traumatic genesis of pain syndrome due to frequent minor injuries in children and adolescents) in the early stages of spinal sarcoma often remains unrecognized.
With progression, the intensity of pain increases. Patients cannot sleep or wake up from pain at night. The mobility of the spine is limited. There is a radiculopathy, depending on the level of lesion, patients with spinal sarcoma complain of pain in the arms, legs, lower back or internal organs. When the spinal cord is compressed, sensitivity and movement disorders first occur, then paresis, paralysis and pelvic disorders develop. In all patients suffering from spinal sarcoma, there is a decrease in working capacity and a violation of daily activity. Anemia, fever, weakness, apathy, loss of appetite and weight loss are detected. In the later stages of spinal sarcoma, pathological fractures occur. Hematogenous metastasis with damage to the lungs, bones and brain is observed. Other organs rarely suffer.
Certain types of spinal sarcomas
Osteosarcoma of the spine is very rare and accounts for only 1-2% of the total number of osteogenic sarcomas. Usually occurs during the period of intensive growth of the skeleton, sometimes it is detected in adult patients younger than 30 years. The literature describes cases of osteogenic sarcoma of the spine in older patients, but experts suggest that in such cases it is not a primary lesion, but malignancy in chondromatosis or Paget’s disease. Usually affects one lumbar vertebra. It metastasizes to the brain and lungs. Bone metastases in osteogenic spinal sarcoma are rarely diagnosed.
Chondrosarcoma of the spine is also a rare malignant tumor and accounts for about 2.5% of the total number of chondrosarcomas. It can be primary (arising from unchanged cartilage) or secondary (developed against the background of chondromatosis, chondroma, chondromyxoid fibroma or chondroblastoma). This type of spinal sarcoma is diagnosed in people over 30 years of age, it is rare in childhood. Men suffer more often than women. Younger patients have a more violent course and rapid progression. The sacrum and lumbar vertebrae are mainly affected.
Ewing’s sarcoma is one of the most aggressive malignant tumors. At the time of diagnosis, distant metastases are detected in 14-50% of patients. This type of spinal sarcoma develops in childhood and adolescence, after 30-35 years it is extremely rare. Men suffer more often than women. The primary tumor is a single node, usually located in the vertebral body. With the progression of spinal sarcoma, nearby structures of the spinal column can germinate, metastasize to other vertebrae and lung tissue. Lymph nodes and other bones of the skeleton rarely suffer.
Angiosarcoma is a malignant tumor of vascular origin. Progresses faster than benign vascular tumors, destroys bone structures and sprouts surrounding soft tissues. This type of spinal sarcoma causes pronounced neurological disorders, often causes pathological fractures of the vertebrae.
Myeloma (Rustitsky-Kaler’s disease) is a malignant neoplasm of plasma cells. It mainly affects the bone marrow. It is diagnosed in elderly people, men suffer more often than women. It is usually multiple (involving vertebrae, ribs, tubular bones and skull bones), single foci are less common. Causes numerous pathological fractures and curvature of the bones of the skeleton. It can cause compression of the spinal cord with the development of paraplegia.
Diagnostics
The diagnosis is made taking into account complaints, anamnesis, results of general and neurological examination, data from instrumental and laboratory studies. During radiography in patients with spinal sarcoma, a focus of destruction with uneven contours is found in the affected vertebra. The destruction of the cortical layer and the rounded shadow of the soft tissue component of the neoplasm are revealed. CT and MRI of the spine make it possible to clarify the size of the tumor and its relationship with nearby anatomical structures. In some cases, scintigraphy is prescribed to clarify the diagnosis.
Along with the above methods, in the process of diagnosing spinal sarcoma, blood test for cancer markers, immunohistochemical and molecular genetic studies are used. The final diagnosis is made based on the results of histological examination. The material is taken by puncture biopsy of the vertebra under the control of CT or X-ray. To detect distant metastases, chest radiography, CT and MRI of the brain, radiography and scintigraphy of the entire skeleton are prescribed.
Treatment and prognosis
Radical surgical removal of the tumor is often impossible due to the germination of nearby tissues. In spinal cord compression, palliative surgical interventions are performed. The leading role in the treatment of spinal sarcoma is assigned to combination therapy. Radiotherapy and polychemotherapy are prescribed using etoposide, cyclophosphane, doxorubicin and other drugs. In case of distant metastases, radiation therapy in high doses is used to increase the effectiveness of treatment, followed by bone marrow transplantation or the introduction of stem cells.
The prognosis depends on the stage of the process, the prevalence and level of differentiation of spinal sarcoma. The use of combination therapy for local highly differentiated tumors provides stable remission in 60-70% of patients. High-dose irradiation followed by bone marrow transplantation can cure 30% of patients. With a high sensitivity of spinal sarcoma to chemotherapy and radiotherapy, the probability of a favorable outcome increases, half of the patients manage to live up to 7 years from the end of treatment.