Cervical Chordoma Research Articles

Pediatric cervical chordoma: report of two cases and a review of the current literature

The majority of chordomas occur between the fifth and seventh decades of life and are thus extremely rare in children, which account for less than 5% of all spinal chordoma cases. We report on the development of this rare condition in two boys aged 7 and 10 years. One patient presented with a palpable neck mass and dysphagia, while the other presented with posterior neck pain. Radiological studies revealed an extensive mass in the cervical vertebrae and paravertebral soft tissue of both patients. The tumors were subtotally removed in an attempt to improve the success of adjuvant proton beam radiotherapy. Pathological examination, which included immunohistochemical staining, revealed chordoma of the cervical spine in both patients. Although en bloc resection is the ideal modality for treatment of chordoma, such a procedure is often associated with a significant risk of surgical morbidity due to the tumor location. Therefore, piecemeal resection followed by postoperative adjuvant radiotherapy, including proton radiotherapy or radiosurgery, should be considered in such cases.

Cervical chordomas

Cervical chordomas

Recurrent cervical chordoma: A case illustration

A case is described of multi-recurrent cervical chordoma in a man over a 5 year period. The clinical features were of progressive spinal cord compression. The authors report a chordoma at C4 that recurred 3 times in five years. The patient underwent four operations and suffered distant metastases. This case confirms that thorough resection of the tumor during the first surgery and post-operative adjuvant treatment are the best assurance of a good prognosis with a chordoma. Multiple surgeries can stimulate biological activity of a chordoma and make its recurrence and distant metastases much more likely. The authors discuss the diagnosis, surgical treatment and the relationship between the histopathological changes and malignancy of a spinal chordoma after four operations. To our knowledge, this represents the first report of a 4th surgery for cervical chordoma.

Chordoma masquerading as a nerve root tumour — a clinical lesson

Chordomas usually arise in bone and are most commonly found in the midline axial skeleton. An accurate pre-operative diagnosis of chordoma is crucial, as survival is optimal when radical en bloc resection is performed at primary surgery. We report a rare case of cervical chordoma masquerading radiologically as an extracranial nerve sheath tumour. A laterally situated chordoma (centred extra-osseously in the neural foramen) was diagnosed radiologically as a neurofibroma pre-operatively. We review the key radiological features for diagnosis of chordoma. We consider the importance of pre-operative diagnosis of chordoma in guiding management and in determining survival.

18F-FMISO Guided CyberKnife Irradiation, to Overcome Hypoxic Tumor Resistance in Recurrent Cervical Chordoma

18F-FMISO Guided CyberKnife Irradiation, to Overcome Hypoxic Tumor Resistance in Recurrent Cervical Chordoma

Image-Guided, Endoscopic Transcervical Resection of Cervical Chordoma

Image-Guided, Endoscopic Transcervical Resection of Cervical Chordoma

Navigated Anterior Approach to the Upper Cervical Spine After Occipitocervical Fusion

Technical note. To introduce the application of navigation system with software for brain surgery to the upper cervical spine of patients who have previously had occipitocervical (O-C) fusion. The anterior approach to the spine using a navigation system with software for spine surgery is difficult because the registration tends to be inaccurate. However, after O-C fusion, the upper cervical spine is considered part of the skull, and a navigation system with software for brain surgery in which the registration is performed using the head with several markers attached to it can be applied. Three patients with previous O-C fusion-2 with upper cervical chordoma and 1 with a disc herniation at C2/3-were treated using this technique. In the first case, with a huge retropharyngeal C1 chordoma, this technique was very helpful in blindly dissecting the nonvisible parts of the tumor. In the second case, with a C2 chordoma, the vertebral arteries were successfully exposed under the guidance of the navigation system at both primary and revision surgery. In the third case, with disc herniation at C2/3, the herniated disc was removed successfully with the totally fused spine. In this application, computed tomography images can be merged freely with magnetic resonance images, which is helpful to clarify the soft tissues such as tumor, disc herniation, or the dural tube. This technique greatly supports surgeons inexperienced in the anterior approach to the upper cervical spine or surgeons at revision surgery who may be lost in and daunted by an unfamiliar operation field surrounded by important structures. Although an anterior approach to the upper cervical spine in the patient with O-C fusion may rarely be required, this application should be considered.

DIRECT INTRATUMORAL CHEMOTHERAPY WITH CARBOPLATIN AND EPINEPHRINE IN A RECURRENT CERVICAL CHORDOMA

Chordomas are rare primary bone tumors for which surgery is classically the first-line treatment. However, safe margins are often difficult to obtain, so that patients are at risk of local recurrence. Because radiation therapy and systemic chemotherapy show limited effectiveness, we report the use of direct intratumoral chemotherapy (IC) to treat recurrent chordoma. A 46-year-old man presented with a recurrent cervical chordoma after surgery and radiation therapy. This recurrence manifested as C4-C5 spinal cord compression. Three 22-gauge needles were inserted at the upper, middle, and lower parts of the tumor and advanced under computed tomographic guidance while injecting local anesthetic. A 5-mg/mL carboplatin solution was combined with epinephrine (to increase the concentration and antitumor effect of carboplatin) at a final concentration of 0.01 mg/mL and an iodinated contrast agent. We injected 3 to 5 mL of this solution over 5 minutes through each needle under computed tomographic guidance. Eleven intratumoral treatments were performed during an 18-month period. A marked clinical response with regression of the spinal cord compression was observed, without specific toxicity. A good partial response was obtained with a 42% decrease in tumor volume (from 69 to 40 cm3). Moreover, the central part of the tumor showed tumor necrosis, as confirmed by histological examination. Thus, in patients with this rare tumor, intratumoral chemotherapy may be a valid treatment option when surgery and radiation therapy fail. Furthermore, intratumoral chemotherapy in combination with surgical treatment should be considered to improve the local control rate.

Cervical Chordoma in Childhood Without Typical Vertebral Bony Destruction

Case report. We present a giant cervical chordoma without typical vertebral bony destruction in an 11-year-old girl. Chordomas are rare malignant bone tumors that arise at both the cranial and the caudal ends of the axial skeleton, characteristically destroying the bone. Cervical chordomas comprise only 3% to 7% of all chordomas. To our knowledge, there is no case of cervical chordoma in a child, presenting without vertebral body involvement, in English literature. Discussion on the patient's clinical, radiologic history, and histopathologic diagnosis of the resected tumor, with a review of the relevant background literature. We report the first case of cervical chordoma in a child without typical vertebral bony destruction, the diagnosis of which was difficult to confirm before and after operation. Giant notochordal rest and benign notochordal cell tumors (BNCTs) need to be recognized for differential diagnosis of this atypical chordoma. A long-term follow-up might be necessary for the diagnosis of this nontypical patient.

Parasagittal osteotomy for en bloc resection of multilevel cervical chordomas

En bloc resection of chordoma has been shown to be critical for prolonging long-term survival and disease-free intervals in patients. Cervical spine chordomas pose special challenges because of the vertebral arteries and critical nerve roots involved. Multilevel chordomas pose even greater challenges because of the need to remove multiple segments of the spine in 1 piece without tumor violation. Although there have been 2 case reports describing multilevel spondylectomy for cervical chordoma, to the authors' knowledge, there are no reports of parasagittal osteotomies for en bloc resection of multilevel cervical chordomas. The use of these osteotomies allows us to avoid intralesional resection and adhere to the oncological principle of en bloc tumor excision. The authors report their management of 3 multilevel cervical chordomas and describe their technique of en bloc tumor removal using parasagittal osteotomy.

Cervical Chordoma with Retropharyngeal Extension Presenting with Impaired Voice

We report an extremely rare case of cervical chordoma presenting with impaired voice. Case report and a review of the literature concerning the presentation, diagnosis, and treatment of a cervical chordoma. A singing teacher complaining of dysphonia was examined and surgically treated for a retropharyngeal extension of a cervical chordoma. A local recurrence was treated with proton beam therapy. Among primary malignant tumors of bone, chordomas account for 3-4% of all cases. Chordoma is typically a locally aggressive tumor with a high propensity for local recurrence. Its management involves surgery, radiotherapy, or both. To our knowledge this is the first report in the world literature of a retropharyngeal extension of a cervical chordoma presenting with impaired voice. This case indicates that bony tumors of the spine may present first to voice-disorder clinicians. Increased awareness of this neoplasm may lead to earlier diagnosis and better treatment.

Clinical case / 12th argospine symposiumRecurrent cervical chordomas how often, and when to stop?

Clinical case / 12th argospine symposiumRecurrent cervical chordomas how often, and when to stop?

Cervical chordoma presenting as retropharyngeal mass and dysphonia: Case report and literature review

Chordoma is a rare primary bone tumour that presents with signs and symptoms of local compression and myelopathy. A case of C4 chordoma with unusual clinical and imaging features is presented. The diagnosis of chordoma requires clinical, radiological and pathological correlation. Clinical features may include laryngeal symptoms arising from distortion of local anatomy. Characteristic tumour appearance and distribution on imaging plays a key role in diagnosis and management of this slow growing tumour with potential for major surgical morbidity.

Chordomas of the upper cervical spine: radiographic evaluation. Study of two cases and review of the literature

Chordomas are rare bony tumors, usually involving the skull base and sacrococcygeal spine, while least presented in cervical and thoracic spine. Chordomas of the cervical spine may cause variable neurological symptoms. Adult patients presenting with neck pain, paresthesias or limb paresis are further investigated. The history of progressive tetraparesis, however, implies a long-standing disease. Clinical presentation and imaging investigation of the cervical spine chordoma aims to alert the physicians for its early detection. Herein is reported the clinical presentation of two chordomas with progressive tetraparesis in two patients involving in both cases the third cervical vertebra. Plain radiographs, bone scintigraphy, CT and MR scanning were the imaging modalities depicting the mass, its prevertebral and intraspinal extension.

Letter to the Editor concerning “Infrahyoid muscle flap for pharyngeal fistulae after cervical spine surgery: a novel approach—report of six cases” (by R.O. Seidl et al.)

Dear Editor, We have read with interest the paper by Dr. Seidl et al. “Infrahyoid muscle flap for pharyngeal fistulae after cervical spine surgery: a novel approach–report of six cases” [2]. They describe a rather elegant technique to repair perforation of the pharynx and the esophagus using the strap muscles as a vascularized flap. In their experience, closure of all the defects was achieved with no complications. In addition, they were able to remove the nasogastric tube within 2 weeks of the operations in all patients, which is a remarkable fact. They also compare the infrahyoid muscle flap with the sternocleidomastoid muscle (SCM), stating that the former has distinct advantages: clearly defined vascular blood supply and better capability to be modeled. In a previous similar study, we presented our experience of using the SCM flap to reconstruct esophageal fistulae after anterior cervical spine surgery [1]. We also achieved closure of all the defects using this technique. However, our patients stayed significantly longer on nasogastric feeding. This fact could be partly explained, because of our patient population: one of our cases had significant lower cranial nerve dysfunction secondary to a cervical chordoma, as we mention on the manuscript, and two other patients had a low level of consciousness for a significant time due to concomitant head trauma. We do not agree with the statement that the SCM flap has modeling limitations, since it is the bulkiest muscle on the anterior part of the neck, it is pliable, it has a multifocal blood supply, and therefore it can be fitted in many different ways without the need of using the whole muscle mass. Hence, its function can be mostly preserved (head rotation and deep inspiratory effort). In contrast, in the technique described by Dr. Seidl et al. the whole ipsilateral infrahyoid muscle group is used, and thus its function might be affected (deglutition and deep respiratory effort). Spinal surgeons should not forget that accessory respiratory musculature could be very important when dealing with patients affected by high spinal cord injuries. In addition, it might be easier for spinal surgeons, namely neurosurgeons or orthopaedic surgeons, to harvest the SCM flap rather than an infrahyoid muscle flap, since there is no need either to dissect the ansa cervicalis nor the superior thyroid artery and vein. We understand that head and neck surgeons might feel quite comfortable using the infrahyoid muscle flap, since it was originally designed to deal with otolaryngological conditions. Finally, we do concur with the authors that local vascularized flaps are the method of choice to repair pharyngeal and upper esophageal defects related to anterior cervical surgery.

Surgical management of chordomas of the cervical spine

Chordomas of the cervical spine are rare tumors. Although en bloc resection has proven to be the ideal procedure in other areas, there is controversy regarding this approach in the cervical spine. The goal in this study was to determine whether piecemeal tumor resection was efficient in the management of chordomas that arise in this location. The authors retrospectively reviewed all 74 cases of chordoma treated by their group. Seven patients with isolated cervical chordomas who were treated between October 1992 and January 2006 were identified. There were four male and three female patients, whose ages ranged from 6 to 61 years (mean 34.4 years). Follow-up duration ranged from 7 to 169 months (median 23 months). All cases were managed using a retrocarotid approach with mobilization of the vertebral artery. When the tumor could not be completely resected via the initial anterior approach, a subsequent posterior resection was performed. Tumor resection was intralesional in all cases, and gross-total tumor resection was achieved in six cases. One patient required a second resection 4 months later. In all cases, a posterior stabilization procedure was performed. Five patients underwent anterior fusion (three with fibular allograft and two with iliac crest), whereas two underwent occipitocervical fusion. In two patients with dedifferentiated chordoma metastasis developed, and one of them died 7 months later. The other patient with metastasis died suddenly at home 26 months postsurgery, presumably from aspiration. At the time of this submission, there were no signs of recurrence in five patients. The authors believe that, in most cases, en bloc resection of cervical chordoma is not feasible. This is due to the tendency of chordomas to involve multiple compartments at the time of diagnosis. In the authors' experience, intralesional radical resection remains an effective surgical approach to this disease entity.

Complete removal of an intraspinal and extraspinal cervical chordoma in one stage using the lateral approach

The authors describe a precise surgical technique in which a large intraspinal and extraspinal, multivertebral, cervical chordoma was completely removed in one stage using the lateral approach. The patient in this case was a 29-year-old woman who presented with signs of radicular pain in the left C-3 area. Computed tomography and magnetic resonance imaging demonstrated a large intra- and extraspinal multivertebral tumor from C-2 to C-5, a finding that suggested a cervical chordoma. The tumor was completely removed in one stage using the lateral approach while controlling the vertebral artery (VA), and a partial corporectomy of C2-5 was also performed. Results from a postoperative histopathological examination confirmed that the tumor was a typical chordoma. The patient's postoperative course was uneventful. Cervical chordomas are typically excised using a posterior-anterior surgical approach with partial resection of the tumor. The lateral approach was appropriate in this patient for complete resection in one stage, because it enabled the surgeons to control the VA and access both extraspinal and intraspinal components of the chordoma.

Transient tetraplegia and vegetative dysfunction depending on cervical positioning caused by a cervical spinal chordoma

A 39-year-old man with acquired torticollis suffering from cervicobrachialgia and neurological deficits is presented. Due to a change in head position a transient reproducible tetraplegia and severe vegetative dysfunctions were caused. The origin of this uncommon serious combination of symptoms and signs was a chordoma of the upper cervical spine. After surgical decompression the patient was free of neurological deficit and pain. Review of the literature did not reveal any similar case.

D-IMRT verification with a 2D pixel ionization chamber: dosimetric and clinical results in head and neck cancer

Dynamic intensity-modulated radiotherapy (D-IMRT) using the sliding-window technique is currently applied for selected treatments of head and neck cancer at Institute for Cancer Research and Treatment of Candiolo (Turin, Italy). In the present work, a PiXel-segmented ionization Chamber (PXC) has been used for the verification of 19 fields used for four different head and neck cancers. The device consists of a 32 × 32 matrix of 1024 parallel-plate ionization chambers arranged in a square of 24 × 24 cm2 area. Each chamber has 0.4 cm diameter and 0.55 cm height; a distance of 0.75 cm separates the centre of adjacent chambers. The sensitive volume of each single ionization chamber is 0.07 cm3. Each of the 1024 independent ionization chambers is read out with a custom microelectronics chip.The output factors in water obtained with the PXC at a depth of 10 cm were compared to other detectors and the maximum difference was 1.9% for field sizes down to 3 × 3 cm2. Beam profiles for different field dimensions were measured with the PXC and two other types of ionization chambers; the maximum distance to agreement (DTA) in the 20–80% penumbra region of a 3 × 3 cm2 field was 0.09 cm. The leaf speed of the multileaf collimator was varied between 0.07 and 2 cm s−1 and the detector response was constant to better than 0.6%. The behaviour of the PXC was measured while varying the dose rate between 0.21 and 1.21 Gy min−1; the mean difference was 0.50% and the maximum difference was 0.96%. Using fields obtained with an enhanced dynamic wedge and a staircase-like (step) IMRT field, the PXC has been tested for simple 1D modulated beams; comparison with film gave a maximum DTA of 0.12 cm. The PXC was then used to check four different IMRT plans for head and neck cancer treatment: cervical chordoma, parotid, ethmoid and skull base. In the comparison of the PXC versus film and PXC versus treatment planning system, the number of pixels with γ parameter ⩽1 was 97.7% and 97.6%, respectively.

Proton Dose Escalation in Base of the Skull and Upper Cervical Spine Chordomas

Proton Dose Escalation in Base of the Skull and Upper Cervical Spine Chordomas