Almost all documented chordomas are located at some point of a neuraxis, with only very few isolated cases of chordoma appearing in regions other than a neuron's axial course. The clinical presentation is dependent on the location of the tumor, as they can occur at any place, from the intracranial nerves to the sacrum [1].

Patients affected by chordoma are typically between the age of 50 and 60 years, with this type of malignancy occurring very rarely amongst children [2]. The sacrum, the mobile spine, and clivus are the sites most commonly diagnosed with a chordoma, in approximately 30% of the cases. A sacral chordoma leads to pain in the lower part of the back that is described by patients as throbbing and mild and typically deteriorates when one sits [3]. Pain in the legs and feet, as well as paresthesias, may occur, and one's gait may be impaired [4]. Sometimes symptoms suggesting an autonomic dysfunction such as bladder or bowel incontinence is reported by the majority of the patients affected by a sacral chordoma.

If a chordoma is growing on the clivus, neurological impairment can be seen. Diplopia, cephalalgia, and symptoms related to the cranial nerves, including palsies commonly occur. The abducens nerve (VI) and the sensory branch of the trigeminal nerve are frequently affected. Some patients with intracranial chordomas may suffer from nasal bleeding, discharge of the cerebrospinal fluid into the nasal cavity or subarachnoid hemorrhage [5]. If a chordoma is located in the cervical nerves, the most commonly reported symptoms are bleeding of the pharynx, hoarse voice and dysphagia.

Chordomas grow very slowly over time, something which also accounts for their being diagnosed at an advanced stage [6]

• Weakness

  In contrast, KIT immunoreactivity was weak and focal in each of the seven positive cases. Total EGFR staining was variable; weak staining for phosphorylated EGFR was detected in nine cases. [ncbi.nlm.nih.gov]

  These symptoms may include pain, weakness, numbness, clumsiness, or tingling in the arms or legs. [orthoinfo.aaos.org]

• Swelling

  A 63 year old male presented with a three month history of dysphagia, neck swelling and an oropharyngeal swelling on examination. [ncbi.nlm.nih.gov]

  Ganglia constitute about 60% of chronic soft-tissue swellings affecting the hand and wrist. They usually develop spontaneously in adults aged 20 to 50, with a female:male preponderance of 3:1. [merck.com]

• Unconsciousness

  Case presentation A 60-year-old Caucasian woman, living alone and previously in good health, was found unconscious at home and fortunately helped by her friends and the emergency medical team. [jmedicalcasereports.com]

• Fever

  There was no history of neck pain, irradiation or trauma, recent fever or viral illness. Pre-treatment TSH was normal. After 18 weeks of treatment, the patient presented hyperthyroidism with positive anti-TSH receptor antibodies. [ncbi.nlm.nih.gov]

• Dyspnea

  In 1 case (4%), surgery led to immediate dyspnea followed by death on the following day; in another case (4%), ischemic infarction led to sudden death. [ncbi.nlm.nih.gov]

  Extraocular complaint can be dysphagia, dyspnea, dysphonia, facial pain, facial paresis, hearing loss, tinnitus, dizziness and ataxia, after brain stem compression. [hccpjournal.biomedcentral.com]

• Nausea

  64-year-old male with 20 years of dizziness history complaining of 6 months of severe dizziness: significant with the changing of the body posture, vertigo which can be self-remissioned within 1 minute and hearing loss of both ears, without headache, nausea [ncbi.nlm.nih.gov]

  The most common ones include: increased risk of infection anaemia (reduced number of blood cells) hair loss or thinning feeling sick (nausea) or vomiting mouth ulcers tiredness (fatigue). [macmillan.org.uk]

  Radiation therapy may cause fatigue, and nausea and vomiting. Bladder control and sexual function may be impaired after surgery on sacral chordomas. Mental functions may be impaired because of inoperable chordomas near the brain. [encyclopedia.com]

  Case Report Presentation and preoperative assessment This is the case of a 78-year-old gentleman who was referred to his internist with a chief complaint of sudden onset diplopia not associated with dizziness, nausea, headache, tunnel vision, or ptosis [skullbaseinstitute.com]

• Vomiting

  The authors report a case of an 11-year-old boy that presented with headache and vomiting that was present for several months. CT and MR imaging revealed a large prepontine mass and an obstructive hydrocephalus. [ncbi.nlm.nih.gov]

  The most common ones include: increased risk of infection anaemia (reduced number of blood cells) hair loss or thinning feeling sick (nausea) or vomiting mouth ulcers tiredness (fatigue). [macmillan.org.uk]

  Radiation therapy may cause fatigue, and nausea and vomiting. Bladder control and sexual function may be impaired after surgery on sacral chordomas. Mental functions may be impaired because of inoperable chordomas near the brain. [encyclopedia.com]

  However, in May 2010, he had multiple admissions for persistent vomiting secondary to subacute intestinal obstruction from his recurrent chordoma and adhesions. [jco.ascopubs.org]

• Hypotension

  On physical examination, the patient presented postural hypotension and dry skin, any other alteration was not observed. [ncbi.nlm.nih.gov]

• Headache

  We report a 26-year-old male presenting with gradually progressive right-sided sixth nerve palsy and headache. Computed tomography and magnetic resonance imaging of the brain showed a cystic lesion in the right cavernous sinus. [ncbi.nlm.nih.gov]

• Confusion

  An intrasellar chordoma can be confused clinically and radiologically with a pituitary adenoma. These two lesions are nearly identical on MRI, although T2-weighted imaging sometimes demonstrates higher intensity with a chondroid chordoma. [ncbi.nlm.nih.gov]

  Unfortunately, this leaves the patient anxious, confused, torn and stricken not only with their grave disease, but also with the outcome of their presumed “choice” [ 5 ]. Owing to their rarity, studies and experience in chordoma are very limited. [link.springer.com]

  Unfortunately, the diagnosis is difficult to make and confusion occurs trying to differentiate chordoma from the more common mucinous carcinomas or myxoid low-grade chondrosarcomas. [journals.lww.com]

  […] in which chromosomes have already begun to fragment, telomere dysfunction), resulting in localized chromosomal fragmentation (involving one or a few chromosomes) and rejoining with incorrect orientations Differential diagnosis Chondrosarcoma : may be confused [pathologyoutlines.com]

  Because of their location, lumbar chordomas may easily be confused with more common tumors in the lumbar spine such as aneurysmal bone cyst, giant cell tumor, hemangioma, myeloma and metastasis, and preoperative diagnosis can be difficult 8. [scielo.isciii.es]

• Agitation

  Note: To avoid clumping do not agitate the cells by hitting or shaking the flask while waiting for the cells to detach. Cells that are difficult to detach may be placed at 37°C to facilitate dispersal. [lgcstandards-atcc.org]

  At the forfeiture hearing, she testified as to her husband's mental condition and his use of the drug Paxil, which she described as an “anti-depressant drug used both to treat depression in ‘an agitated form’ as well as to control the behavior of persons [caselaw.findlaw.com]

Chordomas are usually diagnosed at a late stage. A detailed medical history is required to define the potential site of tumor development.

Laboratory blood tests do not contribute to the diagnosis. However, that is a standard procedure to comprehensively evaluate a patient's status and to prepare them for surgery. Radiographs are the simplest initial imaging modality employed. They cannot depict the chordoma itself, but they can illustrate sites of bone infiltration, seen as a mass extending into the soft tissue, an endosteal margin or bone erosion. Computerized tomography (CT) scans and magnetic resonance imaging scans (MRI) are the best imaging techniques in order to visualize a chordoma. On a CT scan, it is seen as a site of decreased attenuation, sometimes with localized hyperdensity. On an MRI scan, a chordoma will appear as a hypointense area on a T1 image and a hyperintense one in a T2 image.

Fine needle aspiration (FNA) is employed to finalize the diagnosis in cases of uncertainty in a preoperative stage and is preferred to the classic open biopsy [7]. However, a histological examination of the excised material, once the surgery has taken place, still remains the most secure way to diagnose a chordoma. Histologically, chordomas are classified as conventional, dedifferentiated and chondroid, with the two latter ones being the less common types of chordoma [8].

On the other hand, chordomas show limited response to non-surgical treatment modalities. Up to now, no well-established interdisciplinary treatment algorithms for clival chordomas exist. [ncbi.nlm.nih.gov]

The publication of these treatment guidelines marks a major step forward in the Chordoma Foundation’s efforts to improve the lives of patients by working with the medical community to improve the diagnosis and treatment of this rare cancer. [chordomafoundation.org]

These new advances are likely to improve future oncology therapies by complementing surgery and radiotherapy, changing the currently poor prognosis. [ncbi.nlm.nih.gov]

The prognosis in such cases is poor. [radiopaedia.org]

Chordomas are rare sacrococcygeal/sacral, sphenooccipital/clivus, and spinal tumors whose molecular etiology remains relatively understudied. [ncbi.nlm.nih.gov]

As several anecdotal reports had described chordomas in individuals with tuberous sclerosis complex (TSC), a multisystem hamartoma syndrome, we hypothesized that the genes that cause TSC may have an etiological role in chordomas. [doi.org]

Bones::Chordoma WHO/OMS Classification Bones Note Chordoma is a malignant tumour derived from remnants of the fetal notochord; it occurs along the spinal axis, predominantly in the sphenooccipital (35%), vertebral (15%) and sacrococcygeal (50%) regions Etiology [atlasgeneticsoncology.org]

Etiology Chordomas are thought to arise from primitive notochordal remnants along the axial skeleton. During development, the notochord is surrounded by the developing vertebral column. [indiacancersurgerysite.com]

The aim of the study is to describe the epidemiological data and determine the prognostic factors for decreased survival in patients with primary spinal chordoma. [ncbi.nlm.nih.gov]

Familial Chordoma Study Name of the Trial Genetic, Clinical, and Epidemiological Study of Individuals and Families at High Risk of Cancer (NCI-78-C-0039). See the protocol summary. Principal Investigators Dr. Margaret Tucker and Dr. [web.archive.org]

Epidemiology Predominantly in older (age 50–70) men for sacrococcygeal region and age 20–40 for sphenooccipital chordoma. Management Surgery, RT. [medical-dictionary.thefreedictionary.com]

[…] of Medical Research, Medical University of Graz, Stiftingtalstraße 24, 8010, Graz, Austria. 2 Chair of Cell Biology, Histology and Embryology, Gottfried Schatz Research Center, Neue Stiftingtalstraße 6, 8010, Graz, Austria. 3 Chair of Immunology and Pathophysiology [ncbi.nlm.nih.gov]

Control 2001;12:1 ) Base of skull / clivus of occipital bone Vertebral bodies Sacrococcygeal bones Extra-axial skeleton (rare) Soft tissue (chordoma periphericum): rare ( Skeletal Radiol 2013;42:1451 ) Children and young adults: usually cranial chordoma Pathophysiology [pathologyoutlines.com]

To get free no obligation Quote For Chordom Cancer, Childhood Treatment in India : click here India & International : +91 9371770341 Pathophysiology Chordomas are characterized by slow growth, with local destruction of the bone and extension into the [indiacancersurgerysite.com]

Chordoma: An Update on the Pathophysiology and Molecular Mechanisms. Current Reviews in Muscoskeletal Medicine. 2015; 8(4): 344-352. Available at: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC463023... 4. [bcrt.org.uk]

Nevertheless, surgery is still worthwhile to improve the quality of life and to prevent complications secondary to prolonged immobilization. [ncbi.nlm.nih.gov]

Prevention There is no known way to prevent chordomas. Chordoma Case Study A 66-year-old woman began to develop double vision, which was worse when she looked to the right. [mountsinai.org]

Treatment Options: The preferred treatment of a chordoma is surgical removal of as much of the tumor as possible without causing significant neurological damage followed by radiation therapy to prevent recurrence. [ucdenver.edu]

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