Hanna sa, tirabosco r, amin a, pollock rc, skinner ja, cannon sr, et al. The clivus is the surface of a portion of the occipital and sphenoid bones in the base of the skull. Chordoma tumors typically grow slowly, so unless your doctors are saying you are in immediate or critical danger, you have time to decide what to do to get the best treatment. A 41yearold male presented with an incidental clival lesion presumed to be a chordoma. Clival tumors are growths on the clivus, a portion of bone at the base of 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. The authors describe a case of a giant clivus chordoma in a 42yearold male presenting with symptoms due to compression of the brain stem and hypothalamus, including the unusual manifestation of pathological laughter.
Dec 28, 2017 chordoma is a rare type of bone cancer that happens most often in the bones of the spine or the skull. Chordomas are rare slowly growing tumors that originate from remnants of the notochord. Chordoma is a rare slowgrowing neoplasm thought to arise from cellular remnants of the notochord which is a structure in an embryo that helps in the development of the spine. Clival tumors symptoms, diagnostic, skull base surgery and treatment at at columbias skull base tumor center. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Visit our research pages for current research about chordoma treatments. Feb 10, 2011 br video of a transnasal extended endoscopic approach for the removal of a large clival chordoma in a 54 yo male. 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. Giant clival chordoma causing pathological laughter. Similarly, lesions of the sacrum are notoriously difficult to visualize on roentgenograms. 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. Chordoma is a rare cancer that affects bones in the spine and skull. These sites are the ends of the neuraxis and the vertebral bodies.
They have a malignant local behavior, causing symptoms due to bone infiltration and compression of neurovascular structures. Clival chordomas are a rare type of cancer with low metastatic potential and primary metastasize to the lung or bones. Clival chordoma represent the second commonest site of chordoma sacrococcygeal location being the first location. 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. The base of the skull is the second most common site of disease after the sacrococcygeal region. Metastasis from chordoma is uncommon but if occurs, it tends to spread to the lungs. It is surrounded by the neurovascular structures of the brainstem, as well as both internal carotid arteries. Sometimes even a fairly large chordoma will be missed if a specific search is not made on rectal examination. The treatment of clival chordoma remains highly challenging. Astrokytomas at columbia neurosurgery, teamwork carries the day. The clival chordoma and chondrosarcoma are the most common of these tumors. Webmd explains what causes this cancer, and why it can be tricky to treat.
It accounts for 14 % of all primary skeletal tumors, and its incidence rate is inferior to 1. A chordoma can develop anywhere along the spine from the base of the skull to the tailbone coccyx. Chordoma is a rare, slow growing but locally aggressive malignant tumor derived from primitive notochordal elements, and it is usually found in the sacrococcygeal area. 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.
Matriz acuosa gelatinosa es remplazada por cartilago mejor pronostico. A chordoma at the base of the skull occipital chordoma may lead to double vision diplopia and headaches. Chordomas typically occur in adults between ages 40 and 70. A systematic search of the literature was done by labidi et al. For radical removal of clival chordoma, it will be necessary to remove the tumour extensively including normal bone and soft tissues surrounding the. Our treatment pages will help you learn about the way chordoma should be treated so you. Mri features are likely representing clival chordoma, which was proven on histopathology postoperatively. Chordoma is a rare type of cancer that occurs in the bones of the skull base the bones at the bottom part of the skull that separate the brain from other structures. Spontaneous csf rhinorrhea as a presenting symptom of a.
Sep 25, 2007 chordoma and chondrosarcoma of the skull base are rare tumors. A vertebral extra dural chordoma at c5, possibly deriving. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. More detailed information about the symptoms, causes, and treatments of chordoma is available below symptoms of chordoma.
Chordoma symptoms, diagnosis, treatments and causes. Chordoma is a rare slowgrowing neoplasm thought to arise from cellular remnants of the notochord. Chordoma nord national organization for rare disorders. Khawaja1abdef, anand venkatraman2bdef, maira mirza3adef 1 department of neurology, massachusetts general hospital, boston, ma, u. Myxoid liposarcoma negative for ema and cytokeratins. 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. Although it can occur at any segment of the spine, the predominant site of chordoma are fused segments like clivus and sacrococcyx.
Histological subtype also has a substantial impact on prognosis with chondroid chordoma having the best prognosis and dedifferentiated chordoma the worst prognosis. Clival ectopic pituitary adenoma mimicking a chordoma. Our treatment pages will help you learn about the way chordoma should be treated so you can be wellinformed when you speak with doctors. 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. Chordoma at the base of the skull shows destructive, sclerotic lesion of the clivus. The notochord disappears when the fetus is about 8 weeks old, but some notochord cells remain behind in clivus and sacrococcygeal regions. Esmo also hosted a parallel consensus meeting on chordoma that included more than 40 chordoma experts from several disciplines and from both sides of the atlantic, with the contribution and sponsorship of the chordoma foundation, a global patient advocacy group. Chordoma is a rare midline malignant tumor arising from embryonic remnants of the primitive notochord. According to di chiro and anderson,8 it isnow awell accepted concept that the combination ofdense retrosellar calcification together with bone destruction ofthe clivus, dorsum sellae and petrous bones ispractically pathognornonic ofclivus chordoma. Purely ectopic pituitary adenomas are exceedingly rare.
Chordoma, a rare tumor arising from notochordal remnants, has been described to date only by singleinstitution case series or small populationbased surveys. Histological examination of the lung mass revealed findings consistent with a dedifferentiated chordoma, confirm. Chordomas grow slowly, gradually extending into the bone and soft tissue around them. This difficulty is enhanced by the very small likelihood of a successful complete surgical resection or nonsurgical treatment of chordoma. Chordoma is a rare, lowgrade, primary malignant skeletal tumor.
The aspirate shows large cells with pale stained vacuolated cytoplasm and ovoid nuclei. This case report describes a possible metastatic, paravertebral chordoma at level c4c5 in a patient with a past medical history of a clival chordoma. The clival region is the second most common location, accounting for 3035% of cases 2,3. A chordoma that occurs in the tailbone coccygeal chordoma may result in a lump large enough to be felt through the skin and may cause problems with bladder or bowel function. Chordomas are uncommon malignant tumors that account for 1% of intracranial tumors and 4% of all primary bone tumors. Chordomas are rare tumors that arise from embryonic notochordal remnants along the length of the neuraxis at developmentally active sites. We used data from the surveillance, epidemiology, and end results seer program of the national cancer institute, 19731995, to calculate ageadjusted incidence and survival rates for 400 cases of. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. It is a tumor with lobules and vacuolated, moderately atypical, neoplastic cells. The clivus is located in front of the brainstem and behind the back of.
Molecular targeted therapy in the treatment of chordoma. Intracranial chordoma constitutes about 3035% of chordoma cases. Chordoma genetic and rare diseases information center gard. It is composed of areas of conventional chordoma and regions that have the morphology of a highgrade or poorly differentiated spindle cell sarcoma.
Chordoma is a rare type of bone cancer that happens most often in the bones of the spine or the skull. Note the extended approach with the removal of middle. 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. Chordoma and chondrosarcoma of the skull base are rare tumors. Chordoma makes up only 6% of all bone sarcoma diagnoses and most commonly affects adults in their 40s and 50s the majority of chordomas 50% arise in the sacrum the bottom of the spine. The 5year survival rate is estimated to be 51%, and the 10year survival is estimated to be 35%. The chordoma foundation works to accelerate the development of new treatments for chordoma, while helping chordoma patients get the best care possible. We used data from the surveillance, epidemiology, and end results seer program of the national cancer institute, 19731995, to calculate ageadjusted incidence and survival rates for 400 cases of microscopically. 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. It accounts for 14% of all bone malignancies, and 20% of primary spine tumors.
The consensus reached at that meeting is shown in this position paper. Chordoma has characteristic cytological features and a correct preoperative diagnosis is possible. Nov 10, 2017 chordoma is a rare slowgrowing neoplasm thought to arise from cellular remnants of the notochord which is a structure in an embryo that helps in the development of the spine. Oct 22, 2015 chordoma is a rare, lowgrade, primary malignant skeletal tumor. However, mr imaging is the single best imaging modality for both pre and posttreatment evaluation of intracranial chordoma. Typically the mass projects posteriorly at midline, indenting the pons. They often recur after treatment, and in about 40 percent of cases the cancer spreads metastasizes to other areas of the body, such as the lungs. It most often forms where the skull sits atop the spine skull base or at the bottom of the spine sacrum. Chordoma is a relatively rare malignant bone tumor with an incidence of 0. For clivalsphenooccipital lesions differentials to consider include. 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. 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. Chordoma is the most common primary malignant sacral tumor 9,10. Only a few cases of brain tumors associated with pathological laughter have been reported in the literature.
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