A chordoma is a rare type of central nervous system (CNS) malignancy that occurs in some region of the neuraxis. Chordomas develop from remnants of the notochord. The notochord is a midline embryonic structure that is formed during early development and is usually not present in adults.
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 .
Patients affected by chordoma are typically between the age of 50 and 60 years, with this type of malignancy occurring very rarely amongst children . 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 . Pain in the legs and feet, as well as paresthesias, may occur, and one's gait may be impaired . 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 . 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 
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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]
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]
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 . 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 .
Abstract The treatment of clival chordoma remains highly challenging. This difficulty is enhanced by the very small likelihood of a successful complete surgical resection or nonsurgical treatment of chordoma. [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]
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) 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 [dovemed.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]
Chordoma: An Update on the Pathophysiology and Molecular Mechanisms. Current Reviews in Muscoskeletal Medicine. 2015; 8(4): 344-352. Available at: ... 4. Chugh, R, Tawbi, H, Lucas, D.R, Biermann, J.S, Schuetze, S.M, Baker, L.H. [bcrt.org.uk]
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 adjacent [indiacancersurgerysite.com]
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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