Neuronavigationguided endoscopic endonasal excision of. Xia m, huang r, sakamuru s, alcorta d, cho mh, lee dh, et al. Mr image of a chordoma shows a destructive bone lesion in the sacrum that is hypointense on t1w images, hyperintense signal on t2w and enhancement of the lesion after gadolinium. Chordoma of the clivus with endonasal extension remedy. Metastatic disease to the clivus mimicking clival chordomas. Chordoma and chondrosarcoma constitute most primary bone tumors arising within the skull base, with a collective incidence of 0. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. Typically the mass projects posteriorly at midline, indenting the pons.
Intradural clival chordomas are very rare, and 27 cases have been reported 2,3,4,5,6. Chordoma begins in cells that once made up a collection of cells in the developing embryo that go on to become the disks of the spine. Chordoma is a rare type of bone cancer that happens most often in the bones of the spine or the skull. Cranial ch is a very rare entity in the pediatric population, since it represents less than 1% of intra cranial malignancies and 5% of all chs. Sep 17, 2003 chordoma is a rare tumour arising from notochordal remnants in the axial skeleton, which is characterized by slow growth, frequent local recurrences and rare metastases. Methods a schematic description of the anatomy of the clivus and its surrounding structures is provided based on the modular classification of the surgical corridors used in endoscopic skull base surgery. Chordomas form from the leftover cells that were important in. Chondrosarcoma patients had a significantly better outcome compared with chordoma patients with regard to survival and recurrencefree survival p. Remote work advice from the largest allremote company. Chordomas are rare, slowgrowing, and locally aggressive malignant neoplasms derived from primitive notochord remnants. Pdf clival chordoma is a rare intracranial neoplasm located in the clivus with bony extension and destruction.
Chordomas grow slowly, gradually extending into the bone and soft tissue around them. It most often forms where the skull sits atop the spine skull base or at the bottom of the spine sacrum. When developing intracranially, symptoms can range from headaches and neck pain to. However, mr imaging is the single best imaging modality for both pre and posttreatment evaluation of intracranial chordoma. Endoscopic endonasal removal of laterally extended clival. Ct scan of a sacral chordoma demonstrates a lytic lesion of the sacrum with a soft tissue mass. Basiocciput develops from fusion of four occipital sclerotomes. Clival ectopic pituitary adenoma mimicking a chordoma. Chordoma of the clivus a bone in the middle of the head is the most common location for the development of a chordoma, which is a primary bone cancer that develops from remnants of embryonic notochord in the skullbase head and spine. Find, read and cite all the research you need on researchgate. Chordoma is the most common primary malignant sacral tumor 9,10. The treatment of clival chordoma remains highly challenging.
The clival chordoma and chondrosarcoma are the most common of these tumors. They often recur after treatment, and in about 40 percent of cases the cancer spreads metastasizes to other areas of the body. Eight of the patients were men and four were women. The clivus of blumenbach is the sloping midline surface of the skull base anterior to the foramen magnum and posterior to the dorsum sellae 1. Skull base surgery for clival chordoma treatment pacific. Purely ectopic pituitary adenomas are exceedingly rare. We present a case of chordoma involving l 4 and l5 vertebral body and disc mimicking metastasis clinically and aneurysmal bone cyst radiologically. Chordoma is a rare slowgrowing neoplasm thought to arise from cellular remnants of the notochord. At ct, intracranial chordoma typically appears as a centrally located, wellcircumscribed, expansile softtissue mass that arises from the clivus with associated extensive lytic bone destruction. Tumours of the clivus are rare and metastases involving this area have been previously described only as single case reports or included in series with other skull base tumours. Khawaja1abdef, anand venkatraman2bdef, maira mirza3adef 1 department of neurology, massachusetts general hospital, boston, ma, u.
Chordoma of the clivus areport of four cases byhans f. Pdf chordomas are rare tumors that can develop anywhere along the. Very rarely, they present with cerebrospinal fluid rhinorrhoea due to an underlying chordoma induced skull base erosion. Further investigations revealed on ct and mri brain a large tumour destroying the clival region with nasopharyngeal and intracranial extension with pressure effect on brain stem.
Those with rare cancers like chordoma feel lost the star. The gold standard treatment for chordomas of the mobile spine and sacrum is enbloc excision with wide margins and. Establishment of clival chordoma cell line mugcc1 and lymphoblastoid cells as a model for potential new treatment strategies. Specifically, it is formed by the sphenoid body and the basiocciput, which join at the sphenooccipital synchondrosis. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. Chordoma are locally invasive slowgrowing malignant tumors that arise from the remnant of the primitive notochord. Dec 14, 2014 those with rare cancers like chordoma feel lost. Chordoma periphericum is a primary soft tissue chordoma and is very rare. Jul 01, 2003 at ct, intracranial chordoma typically appears as a centrally located, wellcircumscribed, expansile softtissue mass that arises from the clivus with associated extensive lytic bone destruction. Dedifferentiated chordoma is the rarest subtype of chordoma. A technical case report naoshi hagihara 1, toshio matsushima 1, masatou kawashima 1, takashi hikita 2 1 department of neurosurgery, faculty of medicine, saga university 551 nabeshima, saga, japan 2 department of neurosurgery, hamanomachi hospital, 3527 maizuru, chuo ku, fukuoka, japan. Yet few reports define the variable imaging characteristics of bnct.
Clival chordoma has a high rate of recurrence due to its aggressive biology as well as technical difficulty with gross total resection. To overcome the limitations, a sideviewing endoscope and malleablesteerable instruments were employed. Jun 29, 2010 chordoma, chondrosarcoma, osteosarcoma, and metastasis present as more aggressive lesions and have illdefined borders. When a chordoma is suspected, you will need magnetic resonance imaging a type of imaging scan that is used initially to help diagnose chordoma, as well as during follow up to check for recurrence or metastasis. The notochord usually disappears before birth, though a few cells may remain embedded in the bones of the spine or at the base of the skull. The c1 anterior ring was identified and the dens and clivus removed, ultimately exposing the softtissue lesion that was then sent for biopsy and confirmed to be a clival chordoma. This study aims to evaluate the prevalence and characteristics of bnct.
This leads to a tendency for occurrence at extreme ends of the vertebral axis, with 40% occurring at the clivus or cervical spine and 60% occurring in the sacrococcygeal junction of the spine. The clivus is best analysed in the sagittal and axial view. Factors predicting recurrence after resection of clival. Aug 11, 2017 a chordoma is a rare tumor that develops from cells of the notochord, a structure that is present in the developing embryo and is important for the development of the spine. Clival chordomas present with headache, commonly vi cranial nerve. If the address matches an existing account you will receive an email with instructions to reset your password. Feb 10, 2011 br video of a transnasal extended endoscopic approach for the removal of a large clival chordoma in a 54 yo male. They arise purely intradurally without bone or dural involvement and differ from classic clival chordomas in treatment and prognosis. They occur most commonly in the skull base clivus and lower spine. Chordoma is a rare bone cancer that is aggressive, locally invasive, and has a poor prognosis. Clival tumors symptoms, diagnostic, skull base surgery and treatment at at columbias skull base tumor center.
Clival tumors are growths on the clivus, a portion of bone at the base of the skull. Chordomas are extraaxial tumors originating from embryologic remnants of the notochord. However, some extensive andor laterally placed chordomas may require different skull base surgical approaches. A 64yearold male with 20 years of dizziness history complaining of 6 months of severe dizziness. Chordoma is a kind of cancer that grows in the bones of your skull and spine.
The most common locations for a chordoma are at the triangular bone near the base of the spine sacrum, the coccyx, and the clivus, which is a bone in the base of the skull. Superiorly, it reaches at the level of the posterior clinoid process. Patient was investigated and was seen having a mass in the nasopharynx. Given their midline location, most clival chordomas and chondrosarcomas are best removed via an endoscopic endonasal approach. Kellys tumour sits on the clivus, a bone at the base of. Presentation mode open print download current view. Mapping of candidate region for chordoma development to. An mri is the best way to see a chordoma and how it is.
Sep 25, 2007 chordoma and chondrosarcoma of the skull base are rare tumors. Surgical management of lesions of the clivus clinical gate. A tongue of tumor on the right protrudes to abut the right side of the basilar artery and mildly indents the right anterior pons with normal pontine signal preserved thumb sign. Clival chordomas present with headache, commonly vi cranial nerve palsy or sometimes with lower cranial nerve involvement. Osteoblastoma is a common primary benign tumor of the osseous spine that rarely involves the clivus. Microscopic and endoscopic magnification ensured both grosstotal intra and extradural resection, and dural margins were assessed. The evidence for this is the location of the tumors along the neuraxis, the similar immunohistochemical staining patterns, and the demonstration that notochordal cells are preferentially left behind in the clivus and sacrococcygeal regions when the remainder of the. Dec, 2015 clivus chordoma with posterior extension into the pontine cistern and compression of brainstem.
Radiation oncologychordoma wikibooks, open books for an. It is most often found near the tailbone called a sacral tumor or where the spine meets the skull called a clival tumor. The clivus is a portion of bone located at the base of the skull. Learn more about clivus chordoma from related diseases, pathways, genes and ptms with the novus bioinformatics tool. Distinguishing bnct from chordoma with imaging is critical because of the profound differences in prognosis and management. Any information contained in this pdf file is automatically generated from. The topographic anatomy of the clivus makes several surgical approaches feasible. Clivus is best seen in the axial and sagittal view.
Major differential diagnoses of the chordoma in the clivus are the other central skull base. Pdf congenital chordoma of the skin in a lamb first report. Sep 01, 2009 in summary, chordoma is the most common primary malignant tumor of the sacrum. Index terms clivus, chordoma, brainstem meningiomas, blood vessels, nerves i. The gold standard treatment for chordomas of the mobile spine and sacrum is enbloc excision. Clinical, radiological and followup data of patients who had undergone surgery for clivus tumours at. New developments in radiation therapy have improved recurrencefree survival in patients with chordomas. Nov 10, 2016 yang c, schwab jh, schoenfeld aj, hornicek fj, wood kb, nielsen gp. However,it is found to be rare involving the lumbar spine about 2% 2. Upon imaging, the tumour can be seen arising from the clivus and causes clival destruction. Incidentally this junction also marks the cephalic limit of notochord. Chordoma ofthe clivus may cause paresis of nearly allof the cranial nerves and thus challenge the diagnostic acumen of theclinician. Twelve patients with histologically confirmed clivus chordoma were treated at the johns hopkins hospital between 1971 and 1989. It arises from notochordal remnants in the sacrum and commonly extends to the pre and postsacral soft tissues.
Initial treatment for a clival chordoma is with surgical removal or debulking. Clival chordoma is a rare primary bone tumour that arises from the remnant of the notochord and typically occurs in older adults. The tumor appears lobulated and enhances heterogeneously, whereas the pituitary gland shows more marked enhancement, suggesting that the tumor does not arise from it. A chordoma is a rare type of cancerous tumor that can occur anywhere along the spine, from the base of the skull to the tailbone. Approximately 40% of chordomas and chondrosarcomas arise in the clivus directly below the sella turcica and pituitary gland.
Imaging studies classically show a destructive sacral mass with lobular growth, high water content high t2 signal intensity, and foci of hemorrhage and. Rosenberg, in chordomas and chondrosarcomas of the skull base and spine second edition, 2018. Chordomas are rare malignant tumors that arise from notochordal remnants and have a predilection for the upper and lower ends of the neural axis. A chordoma can develop anywhere along the spine from the base of the skull to the tailbone coccyx. Local control chordoma 2126 81%, chondrosarcoma 33 100%, chondroma 11 100%. Inferiorly, it drapes along the posterior surface of the clivus to 1 cm from the clival tip. Approximately 35% affect the skull base, and most involve the clivus. Introduction livusis formed by the union of basiocciput and basisphenoid. Chordoma is a slow growing cancer of tissue found inside the spine. The most frequently involved sites are the clivus, vertebrae and sacrum. Benign notochordal lesions of the posterior clivus. The c2 ganglion sectioning epidural approach to craniocervical junction chordoma. We report a case of congenital clivus chordoma that caused cranial nerve palsy and hydrocephalus. It is composed of areas of conventional chordoma and regions that have the morphology of a highgrade or poorly differentiated spindle cell sarcoma.
It presents as an expansile sclerotic lesion in the clivus. Although a chordoma of the clivus in an 18yrold female was documented as early as 1923, 25 and in an 8yrold boy in 1935, 26 chordomas in childhood and adolescence remain extremely rare. Petrous apex chordomas are possible but extremely rare. Congenital chordoma of the skin in a lamb first report article pdf available in comparative clinical pathology 195. About 300 people are diagnosed with chordoma in the united states each.
This difficulty is enhanced by the very small likelihood of a successful complete surgical resection or nonsurgical treatment of chordoma. Identification of repurposed small molecule drugs for chordoma therapy. Differential diagnosis of clival lesions literature. In a sagittal view, clivus seems a triangle with the base in. Chordomas are thought to arise from transformed remnants of notochord and have a predilection for the axial skeleton, with the most common sites being the sacrum, skull base, and spine. A three year child had presented to our opd with complaints of nasal obstruction, change of voice and difficulty in ingestion. Chordoma is a rare tumour arising from notochordal remnants in the axial skeleton, which is characterized by slow growth, frequent local recurrences and rare metastases. Mapping of candidate region for chordoma development to 1p36. Report atypical clival chordoma in an adolescent without. The evidence for this is the location of the tumors along the neuraxis, the similar immunohistochemical staining patterns, and the demonstration that notochordal cells are preferentially left behind in the clivus and sacrococcygeal regions when the remainder of the notochord regresses during. Computed tomography and magnetic resonance imaging mri were performed before surgery which.
Case report within the first days after birth, a female newborn presented with torticollis, muscular hypotonia, and acro cyanosis. Grade 3 auditory toxicty 1 patient, grade 2 pituitary failure 7 patients. The transsphenoidal approach provides a straight and direct route to the clival chordoma, but has limitations for removing the tumor compartment extending laterally into the space posterior to the paraclival internal carotid artery. Sphenooccipital the clival region is the second most common location, accounting for 3035% of cases 2,3. A few foci of bright signal are seen in clival marrow. Chordoma nord national organization for rare disorders. The mean age at first operation was 51 years range, 10 to 80. Clival chordoma a clival chordoma is a malignant tumor of the clivus. The clival region is the second most common location, accounting for 3035% of cases 2,3. Itposes differentialdiagnostic problems fortheradiologist aswell. The chondroid variety represents 14% of all chordomas mainly developing in the sphenooccipital region and presenting between the third and fifth decades of life.