In case youd like to merge pdf files locally, download pdfmerge, install it then open programsneeviapdf and run pdfmerge. Youve got multiple files documents, spreadsheets, and email attachments from virtually everyone. Chordoma is a musculoskeletal tumor derived from notochord. 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. Jan 28, 2010 chordoma is a rare, lowgrade, primary malignant bone tumour arising from primitive notochord remnants of the axial skeleton. Gokaslan introduction chordomas are a rare, benign tumor arising from remnant notochordal cells. Experimental study on differences in clivus chordoma bone. Chordoma is a rare, slow growing but locally aggressive malignant tumor derived from primitive notochordal elements, and.
Chordomas and cartilaginous tumors at the skull base. The clival chordoma and chondrosarcoma are the most common of these tumors. For radical removal of clival chordoma, it will be necessary to remove the tumour extensively including normal bone. Cervical and thoracic vertebral chordomas have been described. Clival chordoma a clival chordoma is a malignant tumor of the clivus. 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. The evidence for this is the location of the tumors, 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 fetal life.
Original research extraosseous chordoma of the nasopharynx r. Chordoma is a rare cancer that affects bones in the spine and skull. Combined use of maxillomandibular swing approach and. A sagittal postcontrast t1weighted mr image shows a lobulated clival tumor demonstrating marked but heterogeneous enhancement. Combined endoscopic endonasal and transoral surgery for a. The 30 cases of chordoma discussed in this report have all been. Chordoma of the clivus areport of four cases byhans f. Chordoma is the most common primary malignant sacral tumor 9,10. Only eight patients were in the first decade of life, and all the tumors involved the sphenooccipital region. Congenital clivus chordoma eva neumaier probst, friedheim e. Patient underwent a macroscopic complete surgical excision with endoscopic transnasosphenoidal approach followed by proton therapy 74 gy radio biological efficacy rbe in 37 fractions in 7. The classic midline clival chordoma can spread inferiorly, thereby affecting the foramen magnum and nasopharynx, with erosion of the atlas and other cervical vertebrae. 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.
Development of a novel orthotopic primary human chordoma. Commonly, contrast with gadolinium does not show a considerable enhancement in healthy patients 5. For clivalsphenooccipital lesions differentials to consider include. Chordoma genetic and rare diseases information center gard. Clivus chordoma with an arachnoid cyst, coincidence or causative factor. A cytologic study with histologic and radiologic correlation. Dec, 2015 differential diagnosis between chordoma and invasive pituitary adenoma. These areas were merging with chondroid matrix with. It directly supplies a large territory of vital brain tissue including the brainstem and cerebellum and provides the main conduit for blood flow to the. Purely ectopic pituitary adenomas are exceedingly rare. Chordoma is a cytogenetically heterogeneous tumor which displays complex karyotypes. They often recur after treatment, and in about 40 percent of cases the cancer spreads metastasizes to other areas of the body. When chordomas occur in an extraosseous location, they may mimic other lesions. 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.
Clival lesions comprise tumoral and nontumoral pathologies. Metastatic disease to the clivus mimicking clival chordomas. Pdf best practices for the management of localregional. Clinical characteristics, management and prognosis of a case series of 25 patients. Clival ectopic pituitary adenoma mimicking a chordoma. Chordoma selective expression of t brachyury and cd24. An evaluation of the diagnosis and treatment of chordoma. Dec 28, 2017 chordoma is a rare type of bone cancer that happens most often in the bones of the spine or the skull. 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. A position paper by the chordoma global consensus group article pdf available in annals of oncology 286 february 2017. Factors predicting recurrence after resection of clival.
We present three patients with clival chordomas whose sole or initial recurrence was along the pathway of prior surgical access. Surgical pathway recurrence has been noted only rarely in the literature. Clivus chordoma tumor doctor answers on healthcaremagic. Webmd explains what causes this cancer, and why it can be tricky to treat. Neurologically, he was conscious, alert and oriented. Leaving invaded dura to avoid cerebrospinal fluid leak is leaving involved tissue as a source for recurrence and is in violation of the principles of chordoma resection and will ensure eventual recurrence. Establishment of clival chordoma cell line mugcc1 and lymphoblastoid cells as a model for potential new treatment strategies. Radiation dose coverage in outcomes for clival chordoma. She is also dying of chordoma, a cancer so rare that only one in a million is diagnosed each year. Chordomas are rare, usually benign, neoplasms that are thought to arise from.
Prepontine cistern an overview sciencedirect topics. Chordomas are locally aggressive malignant tumors of notochordal origin whose metastatic potential is increasingly recognized. Chordomas arising from the skull base clivus are typically locally aggressive with lytic bone destruction. A 9yearold boy with mild left hemiparesis and left abducens nerve palsy was found to have chordoma of the clivus. Endoscopic endonasal transclival removal of tumors of the. Chordomas are challenging tumours which usually occur in the axial skeleton, and are particularly difficult to manage when they are situated in the clivus and upper cervical spine due to the proximity of eloquent structures. Chordoma and chondrosarcoma constitute most primary bone tumors arising within the skull base, with a collective incidence of 0.
Learn more about clivus chordoma from related diseases, pathways, genes and ptms with the novus bioinformatics tool. Eight of the patients were men and four were women. Chordoma is a rare slowgrowing neoplasm thought to arise from cellular remnants of the notochord. However surgery is rarely possible for a large intracranial lesion. This study grouped differences of clivus chordoma based on different bone. Typically the mass projects posteriorly at midline, indenting the pons. 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. A number of reports indicate that chordomas are seen in all age groups, with the peak incidence varying by site. The umchor1 cell line derived from a clival chordoma was treated with ara014418 ar, an inhibitor of gsk3. It most often forms where the skull sits atop the spine skull base or at the bottom of the spine sacrum. Chordoma is a rare, slowgrowing malignant tumor arising from notochordal remnants. However, mr imaging is the single best imaging modality for both pre and posttreatment evaluation of intracranial chordoma.
A chordoma can develop anywhere along the spine from the base of the skull to the tailbone coccyx. Note the extended approach with the removal of middle. Response to imatinib plus sirolimus in advanced chordoma. Babak alijani 1, mohammadreza emamhadi1, ali azadi 1, arash daryakar 2 1 department of neurosurgery, guilan university of medical science, rasht, iran 2 department of pathology, guilan university of medical science, rasht, iran abstract clivus chordoma coincidence with. The histology and imaging appearance of her tumor, though, changed with recurrence 2. Ct demonstrates a large destructive isodense soft tissue mass arising from the clivus and extending both anteriorly into the sphenoid sinus and posteriorly into the pre pontine cistern, where it indents the pons and displaces the basilar artery to the left. Surgical approaches for clival chordoma described in the literature include craniotomies, transoral 6 and endoscopic endonasal approaches. Yang c, schwab jh, schoenfeld aj, hornicek fj, wood kb, nielsen gp. The median recurrencefree survival for patients, 32% of chondroid chordoma patients, and chordoma was 88 months. This difficulty is enhanced by the very small likelihood of a successful complete surgical resection or nonsurgical treatment of chordoma. Those with rare cancers like chordoma feel lost the star. Chordomas grow slowly, gradually extending into the bone and soft tissue around them. Clivus chordoma with posterior extension into the pontine cistern and compression of brainstem.
After the surgery, gammaknife radiotherapy was performed. The clival chordoma is a malignant tumor arising from remnant cells of the central notochord neuraxis, representing 0. Case at the origin of the primary orthotopic model. Surgery is the main mode of treatment with adjuvant radiotherapy. 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. The clival region is the second most common location, accounting for 3035% of cases 2,3. She was operated on again and a pathology examination revealed chordoma.
Chs are lowgrade malignancies that in about one third of cases occur at the skull base, particularly developing in the clivus, with alocally invasive spread. Intracranial chordoma and allied tumors of the clivus are of significance to the ophthalmologist, who may be the first to see the patient, since diplopia and visual disturbances occur in over a third of the cases and may, together with headache, be the only symptoms. However, resection of invaded clival dura is another critical step in radical removal of chordoma. In the t1 weighted sagittal images they represent distensions of the clivus with secondary displacement of. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. Surgical management of lesions of the clivus clinical gate. 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. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. Chordoma ofthe clivus may cause paresis of nearly allof the cranial nerves and thus challenge the diagnostic acumen of theclinician. Postoperative stereotactic radiotherapy to residual mass in her clivus at a total dose of 12. A chondroid matrix is frequently found in clival intradural chordomas. Mri of case 1 showing primary clival chordoma after regrowth.
Radiation oncologychordoma wikibooks, open books for an. The clivus is localized at the center of the skull base, and its deep location renders surgical access a challenge. Recurrence of clival chordoma along thesurgical pathway. Intracranial chordomas present in a much younger age group than their spinal counterparts because the relevant anatomy of the clival region produces earlier symptomatology. How to merge pdfs and combine pdf files adobe acrobat dc. Chordoma is 4th most frequent primary malignant tumor most patients are 40 to 70 years of age physaliferous cell is the most representative cell present in this tumor most common in sacrococcygeal and sphenooccipital region occurs along central axis of sacrum and spine 20% have calcifications clinical data. The treatment of clival chordoma remains highly challenging. Infrasellar craniopharyngioma mimicking a clival chordoma neurology india, 50, june 2002 examination was normal except for short stature and right scrotal hydrocele.
Dec 14, 2014 those with rare cancers like chordoma feel lost. It has some chromosomal aberrations and is characterized by chromosomal gains and losses at various regions throughout the wide genome. Noah was diagnosed with clival chordoma, a rare skullbased tumor, in april 2019, and he began his treatments on may 20. Hmwmaa expressed in chordoma 62%, chondrosarcoma 48%. There is no need to install special software and uploaded files can be in various formats like doc, xls, ppt and so on. Our patient, an 8yearold girl presenting with headaches and nausea, was found to have a typical clival chordoma, which initially fit the expected pattern of disease presentation. Furthermore, quantitative changes in the tumor cell genome are frequently detected using cgh 18, 19. Chordoma ch and chondrosarcoma chs are rare tumors. Clival chordomas, arising in the clivus posterior to the sella turcica.
Differential diagnosis of clival lesions literature. The petrous bone between chordoma and chondroid chordoma was involved with the clivus in 12% of chordoma p 5. Twelve patients with histologically confirmed clivus chordoma were treated at the johns hopkins hospital between 1971 and 1989. Magnetic resonance imaging mri was most useful not only for diagnosis but also for selection of operative approaches. A characteristic mass found along the pathway of prior. Symptoms of intracranial chordomas vary with lesion location and proximity to critical structures. 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. On t2weighted sequences, clivus is usually isointense relative to the pons. This artery lies within the prepontine cistern and is the main stem of the posterior circulation.
Visual acuity, visual fields and fundi were normal. New developments in radiation therapy have improved recurrencefree survival in patients with chordomas. Craniospinal dissemination of clival chondroid chordoma. Nov 10, 2016 stacchiotti s, marrari a, tamborini e, palassini e, virdis e, messina a.
Chordoma periphericum is a primary soft tissue chordoma and is very rare. Chordoma is a rare malignant tumour with an incidence of metastasis of less than 10 percent. The authors report a case of a congenital clivus chordoma that caused cranial nerve palsy and hydrocephalus within a few days after birth. May 25, 2016 chordoma is distinctly uncommon in patients younger than 30 years. It most commonly arises at the tail tip but may occur anywhere along the vertebral column. Pdf natural history and surgical treatment of chordoma. Both show overexpression of extracellular matrix genes compared to other sarcoma types. Surgical treatment of chordoma and chondroma of the skull base in. The two vertebral arteries merge at the pontomedullary junction to form the basilar artery ba. Itposes differentialdiagnostic problems fortheradiologist aswell. Ulm, germany, the clivus chordoma cell lines mugcc1.
Chordoma is a rare bone cancer that is believed to arise from notochordal remnants that persist along the axial skeleton into adulthood. Characteristics and patterns of metastatic disease from chordoma. A t2 weighted mri, axial view, showing a clivus chordoma, compressing the brainstem. Chordomas of the clivus are skull base tumors which arise from stem cells of the chorda dorsalis in front of the basilar artery. These lesions occur most commonly in the clivus and sacral spine, at the embryological end points of the notochord. Clivus chordoma with an arachnoid cyst, coincidence or. They also have a tendency for local recurrence since enbloc resection is rarely possible in these locations.
Chordoma dedifferentiation after proton beam therapy. The mean age at first operation was 51 years range, 10 to 80. The cells resemble mature adipocytes and have eccentrically located small nuclei. Chordoma and chondrosarcoma of the skull base are rare tumors. B fibrous septae with lobulating pattern is often observed in spinalsacral chordomas. Chondroid chordoma commonly presents as clival osseous and extradural mass. Usually arising from clivus its posterior extension may involve the brainstem before presenting as nasal mass and obstruction. Affymetrix gene expression profiles, 6 chordoma and 14 chondrosarcoma. The tumor appears lobulated and enhances heterogeneously, whereas the pituitary gland shows more marked enhancement, suggesting that the tumor does not arise from it. The clivus is a portion of bone located at the base of the skull. Chordoma nord national organization for rare disorders. Learn how to easily bring multiple files together to create a single pdf with adobe acrobat dc. Pdf chordomas are rare, malignant bone tumors of the skullbase and axial. Sagittal contrastenhanced t1weighted magnetic resonance image.
Clival tumors are growths on the clivus, a portion of bone at the base of the skull. Chordoma is a rare type of bone cancer that happens most often in the bones of the spine or the skull. Infrasellar craniopharyngioma mimicking a clival chordoma. Feb 10, 2011 br video of a transnasal extended endoscopic approach for the removal of a large clival chordoma in a 54 yo male. Pdf establishment of clival chordoma cell line mugcc1 and. Tumors that arise purely from the clivus are rare, but this region can be involved with numerous processes extending from the structures near the clivus, mainly from the petrousclival line. Chordoma is a relatively rare tumor of the skull base and sacrum thought to originate from embryonic remnants of the notochord. Natural history and surgical treatment of chordoma.
Eighteen patients were in the second decade, and twelve had tumors involving the clivus. Brave beautiful boy threetime cancer champ amador rings. Best practices for the management of localregional recurrent chordoma. It accounts for 14% of all primary skeletal tumors and its incidence rate is inferior to 0.1119 1313 1042 1334 1116 1408 238 1426 758 1044 878 434 1119 337 156 196 948 498 789 459 473 498 320 345 1132 646 1625 568 1100 537 1483 1047 593 1375