Chordoma Treatment- Cancer/Oncology, Neurosurgery

Chordoma Treatment (PDQ®)–Patient Version was originally published by the National Cancer Institute.

Chordoma is a very rare type of bone tumor that forms anywhere along the spine from the base of the skull to the tailbone. In children and adolescents, chordomas develop more often in the base of the skull, making them hard to remove completely with surgery.

Childhood chordoma is linked to the condition tuberous sclerosis, a genetic disorder in which tumors that are benign (not cancer) form in the kidneys, brain, eyes, heart, lungs, and skin.

Signs and Symptoms

Chordoma may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

• Headache.
• Neck or back pain.
• Double vision.
• Paralysis of the muscles in the face.
• Numbness, tingling, or weakness of the arms and legs.
• A change in bowel or bladder habits.

Other conditions that are not chordoma may cause these same signs and symptoms.

Chordomas may recur (come back), usually in the same place, but sometimes they recur in other areas of bone or in the lungs.

Treatment

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of chordoma in children may include the following:

• Surgery to remove as much of the tumor as possible, followed by radiation therapy. Proton beam radiation therapy may be used.

Treatment of recurrent chordoma in children may include the following:

• A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change. Patients with changes in the SMARCB1 gene may be treated with tazemetostat in this clinical trial.

Cancer of Unknown Primary Site

Carcinoma of unknown primary is a rare disease in which malignant (cancer) cells are found in the body but the place the cancer began is not known. Cancer can form in any tissue of the body. The primary cancer (the cancer that first formed) can spread to other parts of the body. This process is called metastasis. Cancer cells usually look like the cells in the type of tissue in which the cancer began. For example, breast cancer cells may spread to the lung. Because the cancer began in the breast, the cancer cells in the lung look like breast cancer cells.

Sometimes doctors find where the cancer has spread but cannot find where in the body the cancer first began to grow. This type of cancer is called a cancer of unknown primary or occult primary tumor.

Tests are done to find where the primary cancer began and to get information about where the cancer has spread. When tests are able to find the primary cancer, the cancer is no longer a cancer of unknown primary and treatment is based on the type of primary cancer.

Because the place where the cancer started is not known, many different tests and procedures may be needed to find out what type of cancer it is. If tests show there may be cancer, a biopsy is done. A biopsy is the removal of cells or tissues so they can be viewed under a microscope by a pathologist. The pathologist views the tissue to look for cancer cells and to find out the type of cancer. The type of biopsy that is done depends on the part of the body being tested for cancer. One of the following types of biopsies may be used:

• Fine-needle aspiration (FNA) biopsy : The removal tissue or fluid using a thin needle.
• Core biopsy : The removal of tissue using a wide needle.
• Incisional biopsy : The removal of part of a lump or a sample of tissue.
• Excisional biopsy : The removal of an entire lump of tissue.

When the type of cancer cells or tissue removed is different from the type of cancer cells expected to be found, a diagnosis of cancer of unknown primary may be made. The cells in the body have a certain look that depends on the type of tissue they come from. For example, a sample of cancer tissue taken from the breast is expected to be made up of breast cells. However, if the sample of tissue is a different type of cell (not made up of breast cells), it is likely that the cells have spread to the breast from another part of the body.

Adenocarcinomas, melanomas, and embryonal tumors are common tumor types that appear and it is not known where the cancer first formed. Embryonal tumors such as rhabdomyosarcomas and neuroblastomasare most common in children.

Treatment

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment depends on what the cancer cells look like under a microscope, the patient's age, signs and symptoms, and where the cancer has spread in the body. Treatment is usually the following:

• Chemotherapy.
• Targeted therapy.
• Radiation therapy.

Treatment of recurrent cancer of unknown primary in children may include the following:

• A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.

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