Chordoma is a benign tumor arising from the notochord (the embryonic precursor of the spine). It is located close to the spinal column, in the re-gion of the sacrum or the base of the skull. Most often, this ailment is diagnosed in middle-aged men. But it can also occur in women, children, and the elderly.
Causes of chordoma
In most cases, sacrum chordoma is diagnosed in men in old age. In young people, chordoma most often occurs only at the base of the skull. To date, the exact causes of chordoma are not known.
Chordoma is not inherited. Also, no specific risk groups have been identified that provoke this malignant formation. A chordoma develops from the residual notochord of the embryo.
Types of chordoma
All chordomas are divided into several basic types:
- undifferentiated chordoma;
- common chordoma;
- chondroid chordoma.
The least aggressive of these is the chondroid chordoma. Undifferentiated chordoma is prone to metastasis and is particularly aggressive in contrast to conventional and chondroid chordomas.
In some cases, chordoma is mistaken for chondrosarcoma. This is due to the fact that they have a similar structure and localization. When making a diagnosis, it is required to differentiate them.
A distinctive feature of chondrosarcoma from chordoma is that it is more sensitive to radiation therapy and has a more favorable prognosis.
The symptoms of chordoma depend on its location. When a tumor forms at the base of the skull, the patient has bulbar disorders (disruption of the hypoglossal and glossopharyngeal nerves). Patients have difficulty swallowing food, their voice becomes weak and nasal, and speech disorders appear. Other common symptoms are irregularities in the rhythm of breathing, deterioration of the heart, and vision problems. These processes are associated with disruption of the respiratory and cardiovascular centers in the medulla oblongata.
The sacral chordoma grows on the surface of the body or in the direction of the small pelvis and abdominal cavity. The patient can experience weakness of the lower extremities, pain in the pelvic area, movement disorder, sexual dysfunction, numbness of the extremities, impaired sensitivity of the pelvic organs. Chordoma in an atypical place delivers painful sensations and provokes neurological disorders.
The malignant course of the disease leads to rapid tumor growth, damage to the bones of the skull and vertebrae, the formation of metastases in the lungs, liver and lymph nodes. In the later stages of the disease, cancer intoxication is observed. In these circumstances, the following symptoms are recorded in patients:
- loss of appetite;
- weight reduction;
- constant weakness;
- sudden changes in mood;
- depressive disorders;
The formation of metastases in the lungs makes the patient suffer from excessive shortness of breath, coughing up blood, and respiratory failure. The defeat of the liver turns into jaundice, ascites and hepatomegaly.
Diagnosis of the disease
In the early stages, chordoma can only be detected by a neurologist. It is diagnosed based on the patient’s complaints and the results of the initial examination. To obtain an accurate diagnosis, the following procedures are carried out:
- analysis of urine and blood;
- CT scan;
- X-ray of the affected area;
- biopsy of tumor cells;
- angiography – examination of blood vessels in the problem area;
- magnetic resonance imaging.
Chordoma is difficult to remove with surgery, as it is located near critical structures of the body. Only a few patients can expect complete elimination of the tumor. Usually, patients undergo palliative surgery (partial excision of the chordoma). With their help, they reduce pressure on nerve structures, restore body functions, reduce or eliminate pain.
Radiation therapy is used to treat the disease. It minimizes pain and helps shrink swelling. Irradiation is carried out using an X-ray machine or a gamma device. Stereotaxic surgery is also used to combat chordoma. It is based on the destruction of the tumor using concentrated gamma rays. They are directed to a specific point and hardly touch the healthy tissues of the body.
Chordoma has a high resistance to chemicals. The treatment with chemotherapy gives little or no results. It is important – this disease has a tendency to recur. Because of this, patients must be regularly examined by a doctor even after complete removal of the tumor.
The prognosis depends on the size of the malignant tumor and its localization. In the case of an aggressive form of chordoma, a five-year survival rate is observed in 30%. With a more favorable course of the disease, with appropriate treatment, the life span is from 5 to 10 years. Conditionally, the forecast can be called unfavorable.
To a large extent, the further prognosis depends on the quality of the operation. Therefore, it is very important to find good specialists with a positive experience in the treatment of diseases such as chordoma.
Chordoma of the base of the skull very often continues to grow even after surgery and radiation. The more cells failed to remove, the more likely it is that the pathology will reoccur. According to statistics, about a quarter of all patients complained of the progression of this disease even after a full course of medical therapy. Recurrent cases require repeated operations.