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]

• Prolonged Immobilization

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

• Snoring

  Following the staged surgery, the patient exhibited no symptoms of cerebrospinal fluid leakage, and the main symptoms of snoring and apnea had almost disappeared. [doi.org]

• Otalgia

  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, dysphagia, or otalgia [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]

• Neglect

  A 25 years old female had neglected an extremely large midline sacral mass for 2 years. On presentation to hospital, she had been bed bound for the past 2 years. [ncbi.nlm.nih.gov]

  Ambiguous segments were subsequently neglected. The mean BAF of all SNPs in the segment that were heterozygous in the germline was calculated for imbalanced segments using the allele with the higher read count as B allele. [doi.org]

• Communicating Hydrocephalus

  Following primary resection, the patient's postoperative course was complicated by recurrent meningitis secondary to CSF leak, which responded to antibiotics, and communicating hydrocephalus, for which a ventriculoperitoneal shunt was placed. [ncbi.nlm.nih.gov]

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].

1. Williams BJ, Raper DM, Godbout E, Bourne TD, Prevedello DM, Kassam AB, et al. Diagnosis and treatment of chordoma. J Natl Compr Canc Netw. 2013 Jun 1; 11(6):726-31.
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7. Bergh P, Kindblom LG, Gunterberg B, Remotti F, Ryd W, Meis-Kindblom JM. Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer. 2000 May 1; 88(9):2122-34.
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