What is a chordoma?

Chordomas are rare malignant (cancerous) tumors that are found in the spine and skull. These tumors affect about 1 person in 1 million people per year. One to four percent of all primary bone tumors are chordomas.

Chordomas typically grow slowly. They are often found

in the front (anterior) part of the spine or base of the skull. They can occur at any point along the spine.

Common locations include the base of the spine (sacrum), the tailbone (coccyx), and the place where the spine meets the skull (skull base). There is bone called the clivus behind the back of the throat but in front of the brainstem. Chordomas there are called clival chordomas.

Who develops chordomas?

These tumors are mostly found in adults aged 40 to 70 years. A small percentage (about 5%) are found in children. Males develop chordomas about twice as often as females do.

What are the symptoms of a chordoma?

The symptoms depend on where the chordoma is located. Symptoms can include:

  • Headaches
  • Visual problems, such as double vision
  • Nerve or muscle weakness in the back, arms, or legs
  • Pain
  • Nosebleeds
  • Runny nose
  • Bowel or bladder problems

What causes chordomas?

There is evidence that a person can have a higher risk of chordoma by inheriting an altered gene from a parent. However, other cases of chordoma happen to people who have no family history of chordomas.

There are reports that children who have a genetic condition called tuberous sclerosis complex have a higher incidence of developing chordomas.

Last reviewed by a Cleveland Clinic medical professional on 09/05/2018.

References

  • Chauhan A, Ahluwalia V, Saharan P, Sharma N, Narayan S, Gupta R. RADIOLOGICAL CASE. Clival chordoma in a young child. Applied Radiology [serial online]. November 2017;46(11):37-41.
  • Thompson L. Chordoma. ENT: Ear, Nose & Throat Journal [serial online]. January 2011;90(1):16-18.
  • Tsuji T, Chiba K, Matsumoto M, et al. Differentiation of spinal giant cell tumors from chordomas by using a scoring system. European Journal Of Orthopaedic Surgery & Traumatology [serial online]. October 2016;26(7):779-784.
  • Young V, Curtis K, Temple H, Eismont F, DeLaney T, Hornicek F. Characteristics and Patterns of Metastatic Disease from Chordoma. Sarcoma [serial online]. December 30, 2015;:1-7.
  • Sawke G K, Sawke NG. Sacrococcygeal chondroid chordoma: A case report with brief literature review. Clin Cancer Investig J 2014;3:116-8
  • George B, Bresson D, Herman P, Froelich, S. Chordomas: A Review. Neurosurgery Clinics of North America, 2015-07-01, Volume 26, Issue 3, Pages 437-452, Copyright © 2015 Elsevier Inc.
  • Chordoma Foundation. Understanding Chordoma. Accessed 9/14/2018.
  • Bone Cancer Research Trust. Chordoma. Accessed 9/14/2018.

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