This is a report of 12 cases of clival chordomas that were surgically treated at the Catholic University Medical School, Rome, Italy, over a 7-year period. Chordomas can arise from bone in the skull base and anywhere along the spine. The two most common locations are cranially at the clivus and in the sacrum at the bottom of the spine.Genetics · Mechanism · Treatment · Epidemiology. Twelve patients with histologically confirmed clivus chordoma were treated at the Johns Hopkins Hospital between and Eight of the patients were.
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There were no small chordomas less than 2 cm in our series. Ten patients underwent a total of chordoma del clivus sublabial transseptal transsphenoidal procedures, 21, 30, 33 and two patients were approached via the posterior fossa Table 1.
The surgical technique used for the extreme-lateral transcondylar ELTC approach is that described by Chordoma del clivus, et al. The extent of surgical resection was assessed by performing MR imaging at 3 and 6 months after surgery and then every 6 to 12 months.
The following definitions were applied in this paper: Postoperative adjuvant radiotherapy was not administered routinely; however, two patients did receive radiotherapy after partial tumor resection.
One of them received proton-beam radiation at another institute. All histological specimens were carefully reviewed by a single pathologist L. Chondrosarcomas are not included in this study.
In 10 cases, the tumor was nonhomogeneously hyperintense on T2-weighted MR imaging, thus allowing a presumptive diagnosis of chordoma.
Magnetic resonance imaging was invaluable in the evaluation of superior and inferior limits as well as in the assessment of the lateral extension of the tumor. In the cases undergoing operation via the transsphenoidal approach, MR imaging performed 3 months after surgery was not as useful as that procedure done at 6 months because of the persisting blood signal in the sphenoid sinus.
Making chordoma del clivus differential diagnosis between recurrent tumor and postoperative changes was difficult on MR imaging, particularly when adipose tissue grafts had been used to fill dead spaces in the tumor bed.
Under these circumstances, an enhanced CT scan using an algorithm close chordoma del clivus the bone was chordoma del clivus to be superior to MR imaging.
Angiography revealed that the VA was stretched and narrowed but not encased by the tumor in two cases. The petrous carotid artery was encased without narrowing in one case. In no case was there any tumor blush.
Surgical Resection and Pathological Findings Chordoma del clivus the 10 patients with chordoma of the upper and middle clivus in whom chordoma del clivus transsphenoidal approach was used, a total removal was achieved in seven cases five medium-sized and two large chordomas Figs.
Chordomas are relatively radioresistant, requiring high doses of radiation to be controlled.
The proximity of chordomas to vital neurological structures such as the chordoma del clivus stem and nerves limits the dose of radiation that can safely be delivered. Therefore, highly focused radiation such as proton therapy and carbon ion therapy are more effective than conventional x-ray radiation.
Epidemiology[ edit ] In the United Statesthe annual incidence of chordoma is approximately 1 in one million new patients each year.
Surgical treatment of clival chordomas: the transsphenoidal approach revisited
As noted above germline duplication of brachyury has been identified as a major susceptibility mechanism in several chordoma families. This suggests that some people may be genetically predisposed chordoma del clivus develop chordoma.
Because genetic or hereditary risk factors for chordoma may exist, scientists at the National Cancer Institute are conducting a Familial Chordoma Study to search for chordoma del clivus involved in the development of this tumor.
Received Oct 18; Accepted Feb License allowing third parties to download articles and share them with others as long chordoma del clivus they credit the authors and the publisher, but without permission to change them in any way or use them commercially.
Summary Chordomas are rare tumors that can develop anywhere chordoma del clivus the craniospinal axis.
These tumors present challenges with respect to diagnosis and treatment due to a high rate of recurrence, even after multiple chordoma del clivus, and the propensity to involve any region within the craniospinal axis.
New developments in radiation therapy have improved recurrence-free survival in patients with chordomas.
Chordoma - Wikipedia
Different regimens of chemotherapy and molecularly-targeted therapies, as adjuvants to surgery, have been described in individual case reports and case series. The purpose of this paper is to describe a case of clival chordoma and review recent developments in diagnostic and therapeutic options.
A year-old female was referred because of diplopia and progressively worsening chordoma del clivus. Head imaging revealed a large expansile and erosive mass in the skull base.