Chordoma: causes and symptoms, treatment, prognosis and pictures of the tumor

Chordoma: tumor of the spine and skull base

According to the statistics of the tumor in the bones are some of the most rare among cancers (less than 1 % of cases annually).

However, weakly expressed symptoms and the complexity of treatment make bone cancer a very dangerous problem.

Most bone cancer occurs as a secondary neoplasm due to metastasis and dissemination of tumor of soft tissue.

When cancer is primary in nature and arises from the bone, the symptoms of his appearance blurred in the early stages and do not cause anxiety in the patient, making the treatment begins too late.

In addition, the characteristics of bone tumours is their resistance to irradiation, and, in some cases, to chemotherapy. For such rare cases is true chordoma.

Types of tumors

Chordoma is rare even among primary bone tumors – only in 16 % of cases.

In General, this means that out of 1 million people annually chordomas sick only two or three, but almost every case is fraught with serious complications.

About the disease modern medicine has a relatively small amount of information.

We can identify three types of tumors according to histological features:

  1. Hondroitina – in structure similar to chondrosarcoma, grows slowly and does not metastasize, what the prognosis is more favorable.
  2. Ordinary – has a higher growth rate in 10-30 % of cases give metastases after a long period of asymptomatic development.
  3. Undifferentiated – behaves more aggressively. The rapid invasive growth with active metastasis.

The mechanism of development of pathological formations

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The multiplication of cancer cells in this case starts from the regions with the remnants of the embryonic chords (notochord).

She very slowly and quietly growing, and in the first years of development chondroma gives common symptoms: headaches, pain at the site of tumor development, temporary numbness of the limbs.

Because of these features, this tumor can be considered benign neoplasms.

On the other hand, deep location of the tumor complicates the access to it necessary for the treatment.

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In the case of ordinary and undifferentiated chordoma frequently and quickly (within three years) relapse, requires continuous operations and examinations.

To call this benign tumor cannot. To date, any oncologist will advise the patient to remove Honomu, even if its malignant properties appear.

What is the reason for the appearance of the tumor?

In addition to the main carcinogens (improper diet, alcohol consumption, Smoking, lack of exercise, human papilloma viruses and hepatitis B), called the cause of chordoma is not yet possible.

Statistically not allocated any laws of occurrence of this tumor, as well as the fact of its transmission by inheritance.

Localization of tumor process

As the chordoma arises from remnants of notochord, the area of its localization is the entire spine.

The tumor most often appears on the edges – in the sacrococcygeal and basal-occipital regions (approximately 50 and 35 %, respectively).

For the remaining 15 % of chordoma of the mobile spine (mainly cervical).

When cranial localization of the tumor is usually located in the area of Sella, craniovertebral junction and clivus.

Because of this, the operation to remove it require the participation of several highly qualified specialists (spinal neurosurgeon with skill stabilization of craniovertebral transition, the resuscitator).

Risk factors symptoms

It is known that men develop chordomas of the sacrum is approximately two times more often than women, but the basal-occipital location of the tumor differences by gender no.

The defeat in the sacrococcygeal area is typical for people over 50 years, and in the skull base it occurs predominantly in patients older than 35 years.

Stage of development of the disease

The disease can be divided into three periods.

Appearance and asymptomatic tumor growth

Is seamless for the patient, and therefore usually the treatment can not be.

In rare cases, a tumor in the early stages of development find the x-rays produced in other ways (a survey of injuries of the spine and muscles of the neck and spinal cord, diseases of lungs, brain and others).

The manifestation of the first symptoms

Due to the fact that the tumor grows from the bone, it is able to your weight to put pressure on these critical areas of soft tissues, like nerves, the spinal cord or the arteries.

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Resulting from that, the symptoms differ according to the localization of the chordoma:

  • in the Sacro-coccygeal region: dysfunction of the urinary bladder and rectum due to the compression, leg numbness due to compression of blood vessels, peripheral paresis of the legs, pain at the site of development of tumors;
  • at the base of the skull: headaches, problems with coordination, dizziness, impaired vision and hearing, the failure of the swallowing reflex, disorders of work of the vocal cords, and pain in the area of tumor growth;
  • when chordoma of the spine: back pain, bowel dysfunction, paresthesia and paralysis of the limbs (usually the legs), men are also possible erectile dysfunction.

In any case, with the growth of education is typical for cancer diseases, the clinical picture of intoxication (hyperthermia, weakness, lack of appetite).

Stage latest — lethal

The growth of tumors, metastasis, infiltration of surrounding organs and bone destruction.

At this stage chordoma almost impossible to cure. Cancer spreads throughout the body and disrupts the functioning of vital organs.

Diagnostic methods

In the early stages of the disease suspected tumor can occur when neurological examination. It gives the doctor an idea about the localization and focal symptoms of tumor, is cause for General radiography.

Education detect radiographically, the bone defect in the picture. Confirmation of diagnosis occurs histological study of biopsy material of the affected portion of the bone.

To Refine the size, shape and structure of the tumor and definition of its danger by the presence of metastases and germination in the surrounding tissue, the attending physician, a computer or magnetic-resonant tomography.

In some cases, to establish the degree of destruction of bone and surrounding tissues helps angiography, and scintigraphy pneumoencephalography.

Method of treatment — only operation

Due to the inappropriate use of radiation and chemotherapy, when assigned to chordoma surgery. Resection (complete excision) of the tumor with the capsule.

If the lesion is in the spine is resection of the vertebral bodies by the method of corporate — or spondylectomy with the plastic of the arisen defect.

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Tumors perform resection of the sacrum block (en block) and a combined access.

With the growth of the tumor in the cranial region produce transoral endoscopic removal, extradural, transaxillary way.

Despite radioresistance of bone tumors, the likelihood of recurrence is reduced when postoperative radiation therapy, which destroys the single cancer cells remaining after removal education.

It is also possible the treatment of tumors radiation therapy using modern devices (CyberKnife, gamma knife).

As already stated, chordoma resistant to chemotherapy.

The first operation to remove the tumor is the most important and significant, it cannot effectively conduct it is important to find an experienced, capable make a correct treatment plan and coordinate the actions of several doctors in accordance with this plan.

If there is local recurrence, complete cure of the disease becomes highly unlikely.

The prognosis is poor

Due to frequent recurrence and late metastasis, the percentage of five-year survival rate of patients with aggressive forms of chordoma less than 30, therefore, the forecast conditional unfavorable.

When chordoma of the sacrum and the treatment by the combined method (surgery with radiotherapy), depending on the experience of the doctors value survival over 5 years in the range of 72-90 %, and the probability of relapse – 35-47 %.

Preventive methods

Special preventive measures to prevent chordoma has not been developed due to the unknown causes of the disease.

Recommendations doctors usually repeat the text of the brochure of the world Health Organization:

  • exercise;
  • do not expose yourself to the influence of harmful habits;
  • observe personal hygiene;
  • eat foods with anti-carcinogenic components (vitamins, minerals, capsaicin, lycopene and other plant compounds).