Craniofacial involvement by this entity is well recognized and is known to cause neurovascular impingement and cosmetic deformity. Clival tumors symptoms, diagnostic, skull base surgery and treatment at at columbias skull base tumor center. We discuss the subtle presentation, difficulties in diagnosis and. Treatment with imatinib was successful in stabilizing tumor growth. Purely ectopic pituitary adenomas are exceedingly rare. Characteristics and patterns of metastatic disease from. A multicenter phase ii clinical trial has confirmed the clinical efficacy of imatinib mesylate in the treatment of chordoma. The dural plane was intact, and no bone involvement was observed. Sections show a lobulated tumor with cells arranged in cords and sheets, present in a predominantly myxoid and focally sclerotic stroma. Clival chordomas present with headache, commonly vi cranial nerve palsy or sometimes with lower cranial nerve involvement. 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. Chordomas are uncommon malignant tumors that account for 1% of intracranial tumors and 4% of all primary bone tumors.
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. Fibrous dysplasia of the clivus neurosurgery oxford academic. Surgical pathway recurrence has been noted only rarely in the literature. Features are characteristic of a chordoma which was subsequently confirmed on biopsy. Yang c, schwab jh, schoenfeld aj, hornicek fj, wood kb, nielsen gp. Chordoma is a rare slowgrowing neoplasm thought to arise from cellular remnants of the notochord. When the tumor grows in the sacrococcygeal region or elsewhere in the spine, symptoms might include lower back pain and incontinence. Intracranial chordomas present in a much younger age group than their spinal counterparts because the relevant anatomy of the clival region produces earlier symptomatology. Describe the surgical management of clival chordomas in the pediatric population, with. Very rarely, they present with cerebrospinal fluid rhinorrhoea due. Clival chordoma represent the second commonest site of chordoma sacrococcygeal location being the first location.
Jns172321 on a molecular prognostication panel for clival chordoma after resection. Singh and others published anesthetic management of clival chordoma with retropharyngeal extension. A chordoma can develop anywhere along the spine from the base of the skull to the tailbone coccyx. Clivus chordoma tumor doctor answers on healthcaremagic. Apr 19, 2020 chordoma symptoms depend on the location of primary tumor growth.
Radiation oncologychordoma wikibooks, open books for an. Chordoma genetic and rare diseases information center gard. 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. Sections show a lobulated tumor with cells arranged in. Chordoma symptoms, foundation, causes, types, treatment. 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. Recurrence of clival chordoma along thesurgical pathway. Surgery for clival chordoma is challenging because infiltration to the. Pdf congenital chordoma of the skin in a lamb first report. Metastatic disease to the clivus mimicking clival chordomas. Chordoma is a very rare tumor, but in the last 10 years much progress has been made in the understanding of the biology of this disease, and. Although uncommon, it is important to consider chordoma when evaluating a patient of any age presenting with a hemorrhagic lesion of the clivus.
Chordoma is a rare type of bone cancer that happens most often in the bones of the spine or the skull. Williams bj, raper dm, godbout e, bourne td, prevedello dm, kassam ab, et al. The mean age at first operation was 51 years range, 10 to 80. We read with great interest the recent article by zenonos et al. Intracranial chordoma presenting as acute hemorrhage in a. Although uncommon, it is important to consider chordoma when. Chordomas are rare tumours of the bone arising along the spine from clivus to sacrum. 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. Giant clival chordoma causing pathological laughter. Grade 3 auditory toxicty 1 patient, grade 2 pituitary failure 7 patients. These lesions occur most commonly in the clivus and sacral spine, at the embryological end points of the notochord. About 300 people are diagnosed with chordoma in the united states each.
Nov 10, 2016 a multicenter phase ii clinical trial has confirmed the clinical efficacy of imatinib mesylate in the treatment of chordoma. Chordoma is a tumor that arises from cells that were originally involved in the formation of the skull and axial skeleton spine during the embryological development of the affected. A characteristic mass found along the pathway of prior. Paediatric chordomas orphanet journal of rare diseases. Mri features are likely representing clival chordoma, which was proven on histopathology postoperatively. Congenital chordoma of the skin in a lamb first report article pdf available in comparative clinical pathology 195. We compared three chordoma cell lines of the clivus region including the newly established clivus. Characterization of clivus chordoma tumor cell line mugcc1. Chordomas account for less than one percent of all central nervous system tumors and. Characteristics and patterns of metastatic disease from chordoma. At imaging, a chordoma typically manifests as a large destructive sacral mass with secondary softtissue extension. Only 5 % of them occur in the first two decades such less than 300 paediatric. Establishment of clival chordoma cell line mugcc1 and. Nov 29, 2012 clival chordomas present with headache, commonly vi cranial nerve palsy or sometimes with lower cranial nerve involvement.
Chordoma are rare tumours of the axial skeleton which occur most. The clival chordoma and chondrosarcoma are the most common of these tumors. Best practices for the management of localregional recurrent. Chordomas are rare, malignant bone tumors of the skullbase and axial skeleton. Chordomas grow slowly, gradually extending into the bone and soft tissue. Chordoma ofthe clivus may cause paresis of nearly allof the cranial nerves and thus challenge. Summary of 12 pediatric cases of lntracranial chordoma present case included age author year years sex location operation radiation survival note adson et al 1935 8 m clivus craniotomy yesb. We compared three chordoma cell lines of the clivus region including the newly established clivus chordoma. Radiographic distinction between an intradural retroclival chordoma and ecchordosis physaliphora may be difficult. They often recur after treatment, and in about 40 percent of cases the cancer spreads metastasizes to other areas of the body. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. Twelve patients with histologically confirmed clivus chordoma were treated at the johns hopkins hospital between 1971 and 1989. After a oneyear cultivation period, the mugcc1 cell line was established and the chordoma cells were characterized phenotypically.
Local control chordoma 2126 81%, chondrosarcoma 33 100%, chondroma 11 100%. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. Itposes differentialdiagnostic problems fortheradiologist aswell. Nov 10, 2016 a number of reports indicate that chordomas are seen in all age groups, with the peak incidence varying by site. The distinction between clival chordoma and ecchordosis physaliphora is important.
We present a giant clival chordoma with disseminated disease but without involvement of the clivus. Treatment with imatinib was successful in stabilizing tumor growth 84% or shrinking tumor size 16% in a cohort of patients with progressing, advanced chordoma. The floor of the sinus and the clivus were drilled out between the paraclival bulging of the internal carotid arteries. Intracranial chordoma is a locally aggressive and relatively rare tumor of the skull. Clival ectopic pituitary adenoma mimicking a chordoma. Clival chordoma represent the second commonest site of chordoma sacrococcygeal. We describe the establishment of a clivus chordoma cell line, mugcc1. 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 is a rare bone cancer that is believed to arise from notochordal remnants that persist along the axial skeleton into adulthood. Radiographs may show sacral osteolysis with an associated softtissue mass and calcifications.
May 17, 2017 chordomas are rare tumours of the bone arising along the spine from clivus to sacrum. A chordoma is a rare type of bone tumor that develops along the spine or at the base of the skull. Mar 25, 2006 we present a giant clival chordoma with disseminated disease but without involvement of the clivus. Feb 10, 2011 br video of a transnasal extended endoscopic approach for the removal of a large clival chordoma in a 54 yo male.
Stateoftheart imaging in human chordoma of the skull base. 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. Chordoma nord national organization for rare disorders. Chordoma periphericum is a primary soft tissue chordoma and is very rare. Clival tumors are growths on the clivus, a portion of bone at the base of the skull. We present three patients with clival chordomas whose sole or initial recurrence was along the pathway of prior surgical access. A 41yearold male presented with an incidental clival lesion presumed to be a chordoma. The first chordoma was in the clivus and resulted in an abducent cranial nerve palsy, but the second in l5 vertebra was completely asymptomatic. Symptoms of chordomas depend on its location and size.
Importance of imaging find, read and cite all the research you need. Chordoma is a rare malignant tumor of the skull base and axial skeleton, with an incidence of less than 0. Chordoma clivus endoscopic endonasal surgery pediatric tumors. Typically the mass projects posteriorly at midline, indenting the pons.
First clinical guidelines for chordoma treatment published. Chordoma genetic and rare diseases information center. A 60year old caucasian woman presented with meningitis secondary to cerebrospinal fluid rhinorrhoea. The clivus is a portion of bone located at the base of the skull. The tumors usually develop at the clivus which is situated in front of the brainstem, sacrum which is the triangular bone close to the bottom of the spine, and cocyx which is at the bottom part of the vertebral column. Very rarely, they present with cerebrospinal fluid rhinorrhoea due to an underlying chordoma induced skull base erosion.
Chordomas are locally aggressive malignant tumors of notochordal origin whose metastatic potential is increasingly recognized. Surgical management of clival chordomas in children operative. Eight of the patients were men and four were women. 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. Gokaslan introduction chordomas are a rare, benign tumor arising from remnant notochordal cells. Clival chordoma a clival chordoma is a malignant tumor of the clivus. The chordoma foundation provided funding for this meeting. 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 is the most common primary malignant sacral tumor 9,10. Paediatric chordomas are rare malignant tumours arising from primitive notochordal remnants with a high rate of recurrence. Chordoma at the base of the skull shows destructive, sclerotic lesion of the clivus. Common symptoms reported by people with clival chordoma. 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. Chordoma is a kind of cancer that grows in the bones of your skull and spine. Fibrous dysplasia is a developmental skeletal disorder that may lead to distortion, expansion, and weakening of the bone. Chordoma, clivus, pathological laughter introduction 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. We have modified our departmental practice as we believe that all patients diagnosed with chordoma should have mri scanning of their entire spinal tract to exclude a second primary chordoma. Chordoma is a rare, slowgrowing malignant tumor arising from notochordal remnants.
The evidence for this is the location of the tumors along the neuraxis, the similar immunohistochemical. The clival region is the second most common location, accounting for 3035% of cases 2,3. Chordoma ofthe clivus may cause paresis of nearly allof the cranial nerves and thus challenge the diagnostic acumen of theclinician. Dec 28, 2017 chordoma is a rare type of bone cancer that happens most often in the bones of the spine or the skull. When the tumor grows in the clivus, symptoms typically include headaches and vision disturbances. The most common locations for a chordoma are at the triangular bone near the base of the spine. Apr, 2016 characterization of clivus chordoma tumor cell line mugcc1. To our knowledge, this is the youngest child and only the second case, presenting without. Infantile clivus chordoma without clivus involvement. Patients with advanced chordoma have a poor prognosis due to. It most often forms where the skull sits atop the spine skull base or at the bottom of the spine sacrum. 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.
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