Brain & Nerve
Diseases & Conditions
Contributed byKrish Tangella MD, MBADec 29, 2021

What are the other Names for this Condition? (Also known as/Synonyms)

  • Notochordoma
  • Skull Base Chordoma
  • Spinal Chordoma 

What is Chordoma? (Definition/Background Information)

  • A Chordoma is a rare, slow-growing, malignant tumor. The tumor arises from cells called the ‘notochordal remnants’, situated at the sides of the vertebral column. Notochord is the embryonic structure from which the brain and spinal cord develop, during fetal development
  • Chordomas comprise less than 1% of central nervous system (CNS) tumors. They are commonly observed in 3 locations:
    • 50% of them are reported in the sacrococcygeal region (bottom of the spine)
    • The next common site is the brainstem (the posterior part, where the spinal cord joins the cerebrum/brain)
    • The least common site is the spinal axis cervical region
    • Rarely, Chordomas may also occur in extra-axial locations (away from the vertebral column midline)
  • Individuals with a positive family history of this condition have a higher risk of developing these malignant tumors, later in one’s life
  • Generally, mid-to-elderly adults are seen with this infrequent CNS tumor. Depending on the location of these slow-growing tumors, they can cause local bone destruction and extend deep into the soft tissues
  • The treatment of choice for Chordoma that affects the brain and central nervous system is a complete surgical removal of the tumor, which reduces the risk of recurrence
  • The prognosis depends on many factors, such as the individual’s age, site and size of the tumor; though largely, the prognosis of Chordoma is poor

Who gets Chordoma? (Age and Sex Distribution)

  • Chordomas are rare tumors; only 1 in 500 central nervous system (CNS) tumors, are Chordomas. This implies that each year about 1 in a million individuals may be affected by this malignant condition
  • They are usually observed in middle-aged to elderly adults and have a wide age range of presentation (27-80 years); with an average age of diagnosis at 60 years. However, it is found that:
    • Intracranial chordomas are diagnosed at around 48 years (average age of individuals)
    • Sacrococcygeal chordomas are diagnosed at around 56 years
    • Spinal axial chordomas are diagnosed at around 46 years
  • The age distribution depends on the site of the tumor. Intracranial chordomas are diagnosed earlier than their spinal counterparts; since, intracranial chordomas show signs and symptoms much earlier
  • Chordomas is observed with a higher incidence in men (2:1 male-female ratio); although, for skull base tumors, the male-female ratio is almost 1:1
  • Children are also affected by Chordoma, though the incidence is much less. It is estimated that only about 5% of all Chordoma cases are seen in children

What are the Risk Factors for Chordoma? (Predisposing Factors)

Chordoma risk factors may include:

  • The presence of a genetic syndrome, called tuberous sclerosis complex, increases the risk
  • Individuals with a family history of the condition are at an increased great risk of developing Chordomas; however, most tumor occurrences are sporadic 

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider. 

What are the Causes of Chordoma? (Etiology)

  • Brain and spinal chordomas occur, when the embryonic remnants of the notochord outside the dura mater (outermost covering of the meninges), get transformed into a malignant Chordoma
  • During development of the embryo, the notochord is surrounded by the vertebral column. In adults, the remnants of the notochord are represented by a structure, called nucleus pulposus, which is present inside the vertebral body. If by any chance, these remnants are present outside the dura mater, covering the spinal cord and brain, they are prone for malignant transformations into Chordoma
  • A genetic basis, as the cause of development of Chordoma is reported. Many of these tumors occur sporadically without a family history
  • Genetic defects reported are: Partial losses in chromosomes 3, 4, 10, and 13; gains in chromosome 7; and certain rearrangements in chromosome 1. These mutations are implicated as the causes of this CNS tumor
  • Deficiencies in the DNA mismatch repair gene have also been demonstrated in Chordomas. According to the US National Institute of Health, transformations or changes in the T gene have been associated with Chordoma

What are the Signs and Symptoms of Chordoma?

The signs and symptoms of Chordoma are based on the location of the tumor. The signs and symptoms of different Chordoma types are explained:

Sacral and other spinal chordomas:

  • Lower back pain
  • Radiating pain to the legs
  • Rectal and urinary incontinence
  • A palpable mass may be observed at these regions
  • Chordomas along the cervical vertebrae (neck region) may be present with:
    • Hoarseness of voice
    • Swallowing difficulties
    • Pharyngeal bleeding

Intracranial chordomas:

  • Double vision
  • Headache
  • Damage to 6th nerve, called sixth nerve  palsy (dysfunction of cranial nerve VI or abducens nerve) that primarily affects the vision
  • Damage to 7th nerve, called facial nerve palsy (dysfunction of a branch of trigeminal nerve) affecting facial movement
  • Infrequently, these tumors may present with:
    • Nasal cavity, which is filled with cerebrospinal fluid (termed as CSF rhinorrhea), due to CSF leakage from the brain into the nose
    • Bleeding from the nose
    • Nasal obstruction causing breathing problems

A type of Chordoma, called chondroid chordoma, is known to grow much more slowly, compared to other types of Chordomas. 

How is Chordoma Diagnosed?

The location of the Chordoma may determine the kind of diagnostic tests that are required. A diagnosis of Chordoma may involve:

  • Physical examination, evaluation of the individual’s medical history
  • MRI and CT scans of the affected region: These are required for finding the location of the tumor and to assess the local spread/invasion of the tumor
  • Fine needle aspiration cytology (FNAC) biopsy - the specimen is examined under a microscope by a pathologist to arrive at a definitive diagnosis
  • A tissue biopsy of the tumor: A tissue biopsy is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together the clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be the gold standard, in arriving at a conclusive diagnosis
  • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, histochemical stains, molecular testing, and very rarely, electron microscopic studies
  • Differential diagnosis, to eliminate other tumor types are often considered, before arriving at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis. 

What are the possible Complications of Chordoma?

Complications depend on the tumor site and the surgery type that is performed. Following are the potential complications of Chordoma:

  • A subtotal resection of tumor has lesser complications than a radical resection
  • Tumor recurrence on an incomplete surgical removal of the tumor
  • In case of sacrococcygeal tumors, bowel and bladder dysfunction may occur
  • A distant metastasis of the tumors may take place, in very rare cases

How is Chordoma Treated?

The treatment of Chordoma may include:

  • Surgical resection is the main stay of treatment. A complete surgical removal of the tumor with clean margins, decreases the chance of recurrence
  • Sub-total resection may be the only option, either due to inaccessibility of the tumor, or due to proximity to delicate anatomical structures. In such cases, the recurrence rate is high
  • Adjuvant radiation therapy may be used to increase the time interval for recurrence
  • Chemotherapy with Imatinib mesylate is under trial. It has shown promising results in stabilizing tumor growth and shrinking tumor size
  • Post-operative care is important: A minimum activity level is to be ensured, until the surgical wound heals
  • Follow-up care with regular screening and check-ups are important 

How can Chordoma be Prevented?

  • Currently, there are no specific methods or guidelines to prevent Chordoma
  • Active research for Chordoma is currently being performed to explore the possibilities for treatment and prevention
  • Regular medical screening at periodic intervals with scans and physical examinations are important 

What is the Prognosis of Chordoma? (Outcomes/Resolutions)

  • Chordomas grow in size and erode the surrounding soft tissue and underlying bone. Thus, these tumors exhibit a malignant behaviour through locally aggressive growth patterns
  • The prognosis of Chordoma is defined by the following:
    • Tumor size
    • Site of the tumor
    • Type of Chordoma
    • And, whether the tumor can be completely resected/surgically removed. Tumors that can be removed completely have better prognosis, than partially resectable tumors
  • Typically, the survival rate at 5 years is estimated to be at 50%; for 10 years, the survival rate is around 35%
  • Some of the factors that may greatly improve Chordoma outcomes are:
    • Age of the individual (younger the age, better is the prognosis)
    • Possibility of a complete surgery
    • Possibility of radiation therapy for incompletely resected tumors
  • Rarely, metastasis due to Chordoma has been reported; metastatic Chordomas have poorer prognosis 

Additional and Relevant Useful Information for Chordoma:

  • The US National Cancer Institute is conducting a Familial Chordoma Study, to research the genes involved in the development of Chordoma. In the US, about 300 cases of Chordoma are diagnosed each year
  • There are 2 benign conditions that resemble Chordoma. These include:
    • Ecchordosis physaliphora: These are well-defined, gelatinous, and tumor-like that is found around the brainstem
    • Intraosseous benign notochordal cell tumors: These are benign lesions found on the vertebra, similar to Chordoma
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team


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