A revision endoscopic excision of a clival chordoma is challenging and has been highlighted in this report. Chordoma is a rare, indolent primary bone malignancy. Mri of the brain and orbit revealed clival mass involving the left cavernous sinus measuring 5. Endoscopic management of clival chordomas and chondrosarcomas.
Apparent diffusion coefficient as a prognostic factor in clival. Clival chordoma in early childhood without bone involvement. Clival chordoma is a rare neoplasm that arises from embryological remnants of the notochord along the spinal axis. There was significant compression of the brain stem, upper cervical cord and the right hypoglossal nerve, with occlusion of the right c12 and c23 intervertebral foramina. With a working diagnosis of clival chordoma, the patient underwent transnasal transsphenoidal endoscopic resection of the tumor under general anesthesia. Therefore, most are treated epidemiology with radiotherapy rt. Positron emission tomography with ct scan revealed fluorodeoxyglucose avid lesions in the clival region and skeleton sternum and skull. A, sagittal t1weighted image 6001 obtained at initial presentation shows a large soft tissue mass centered on the clivus, consistent with known chordoma. At the time of diagnosis, imaging demonstrated a t2 hyperintense and t1 isointense midline skull base mass, centered in the nasopharynx, with scalloping of the ventral clivus consistent with a chordoma measuring 3. We developed the following chordoma educational materials to help you make informed medical decisions and get the best care possible for yourself or your loved one. Itposes differentialdiagnostic problems fortheradiologist aswell.
Mr findings in a 33yearold woman with clival chordoma. The presence of brainstem and cord displacement indicated urgent surgical intervention. Chordomas are very rare bone malignant tumours that have had a shortage of effective treatments for a long time. Primary chordoma of the nasopharynx is an extremely rare malignant tumor of notochordal origin in the extraosseous axial skeleton. Tumors frequently invaded the petrous bone, occurring in 88% of cases 19% on the right, 17% on the left, and 52% bilaterally. C, contrastenhanced axial t1weighted image 6002 shows. A 66yearold female presented with a 6month history of headaches and lightheadedness. Very rarely, they present with cerebrospinal fluid rhinorrhoea due to an underlying chordoma induced skull base erosion.
We account that fungal infection at the skull base is extremely rare,1, 2 but there is every possibility under the sun that neurosurgeon may encounter with this saprophyte to have caused bilateral sixth nerve palsy in hisher patient with disease masquerading as a clival chordoma on imaging. Clivus chordoma witha an arachnoid cystemamhadi et al international clinical neuroscience journal vol 2, no 2, spring 2015 79 decades of life, whereas chondroid chordoma present at an earlier age and has a female predilection 1,9. Chordoma ofthe clivus may cause paresis of nearly allof the cranial nerves and thus challenge the diagnostic acumen of theclinician. Purpose combining maximal surgical resection with highdose proton radiation therapy is reported to be currently the best management of patients with clival chordoma.
Establishment of clival chordoma cell line mugcc1 and. Radiographic distinction between an intradural retroclival chordoma and ecchordosis physaliphora may be difficult. The lesion was grayishpink in color, moderately vascular, completely destroying the clival region, and locally infiltrating and extending up to sphenoid sinus. Locally aggressive clival chordomas can even results in lytic bony distruction. Approximately 35% affect the skull base, and most involve the clivus. Clival tumors are generally rare, comprising 1% of all intracranial neoplasms.
Jun 28, 2011 transnasal endoscopic resection of clival chordoma. Most clivus chordomas show extradural extension and bone destruction. Comprising one third of central nervous system chordomas, clival chordomas. Feb 22, 2021 the most common location of the tumor in adults is sacrococcygeal, while in children, the craniovertebral junction is the most common site 54%. Roentgen examination will often show osteolysis of the clivus.
Chordoma educational materials download or request a. The approaches described in this article have been used in an attempt to minimize morbidity while maximizing exposure. Visual complications of proton beam therapy for clival chordoma. Due to their aggresive behaviour including intradural infiltration of neurovascular. To download or request hard copies of these materials, please use the form at the end of the page. Clivus chordoma in paper received 7 april 2006 childhood. Subarachnoid hemorrhage associated with clival chordoma case. Intradural extraosseous chordoma has clearly different features from those of typical chordoma it is uncommon, occurs almost exclusively in the prepontine and clival regions, and is encountered in 0. Magnetic resonance imaging of the intradural prepontine. Frommhold h, mikuz g 1987 clivus chordoma in a 9yearold child. Surgical treatment of chordoma and chondroma of the skull. Gigantism, clivus chordoma, primary hypothyroidism, hypogonadism, pancytopenia introduction pituitary gigantism is a condition caused by the excessive secretion of growth hormone gh. Skull base surgery for clival chordoma treatment pacific. Recurrence of clival chordoma along thesurgical pathway.
In conclusion, mean and minimum adc values could be useful in predicting aggressiveness of clival chordoma. T2weighted mri revealed a hyperintense mass consistent with a clival chordoma fig. Tumor involved the upper clivus in 43%, the middle clivus in 88%, and the lower clivus in 69% of cases. Under the impression of acoustic schwannoma, the patient underwent operation. Anatomical imaging demonstrated a clival lesion most suspicious for chordoma. Pdf surgical pathway seeding of clivocervical chordomas. Imaging revealed a clivus chordoma and an asymptomatic synchronous second primary chordoma in the fifth lumbar. Calcification within the tumor occurs more frequently than reported in the past. Posterior displacement of the brainstem facilities radiosurgery in the management of chordoma and chondro safer high dose radiosurgery for clival chordoma. However, mr imaging is the single best imaging modality for both pre and posttreatment evaluation of intracranial chordoma. The distinction between clival chordoma and ecchordosis physaliphora is important. After a oneyear cultivation period, the mugcc1 cell line was established and the chordoma cells were characterized phenotypically.
The differential diagnosis for a clival lesion is vast, including chordoma most commonly 40%, meningioma, chondrosarcoma, astrocytoma, craniopharyngioma, germ cell tumor, nonhodgkins lymphoma, melanoma, metastatic carcinoma, and rarely pituitary adenoma. Petrous apex chordomas are possible but extremely rare. Skull base chordomas usually arise in the clivus and are rarely completely resectable. A, axial t2weighted image 2500801 shows a small, wellcircumscribed, hyperintense nodule along the right side of the nose arrow. 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.
The transseptaltranssphenoidal approach is appropriate for biopsy or for subtotal removal of small midline lesions of the upper retrosellar clivus only. Chordoma educational materials download or request a hard copy. Apr, 2016 characterization of clivus chordoma tumor cell line mugcc1. The symptoms of a clival chordoma include diplopia and headache.
Clival chordomas present with headache, commonly vi cranial nerve palsy or sometimes with lower cranial nerve involvement. Clinical information, including extent of resection, al. Chordoma dedifferentiation after proton beam therapy. Case presentation a 60year old caucasian woman presented with meningitis secondary to cerebrospinal fluid rhinorrhoea.
Results the overall surgical pathway recurrence rate for clival chordoma resection based keywords on analysis of the open nonendoscopic published case studies was 14 of 497 2. The following flow chart illustrates the possible options for a skull base the bones at the bottom part of the skull that separate the brain from other structures. Endoscopic endonasal transphenoidal approach for clival chordoma provides a safe and reliable tumor resection. New developments in radiation therapy have improved recurrencefree survival in patients with chordomas. Mr findings in a 47yearold man with clival chordoma. 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 tumor arising from embryonic remnants of the notochord and can be locally aggressive with a tendency to recur. The treatment of chordoma and chondrosarcoma of the skull base. Microscopic and endoscopic magnification ensured both grosstotal intra and extradural resection, and dural margins were assessed. Download fulltext pdf download fulltext pdf read fulltext.
Pdf combined endoscopic endonasal and posterior cervical. Tumorbiology and current treatment of skullbase chordomas. Chordoma of the clivus belong to the rare tumors of the skull base. The chondroid variety represents 14% of all chordomas mainly developing in the sphenooccipital region and presenting between the third and fifth decades of life. When developing intracranially, symptoms can range from headaches and neck pain to cranial nerve neuropathies and. We conducted a pooled and metaanalysis comparing recurrence rate, postoperativecomplications, an.
Transnasal endoscopic resection of clival chordoma mundi. 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. Major differential diagnoses of the chordoma in the clivus are the other central. A chordoma will displace the artery away from the midline, while a nerve sheath tumor will displace the artery toward the midline. Surgical treatment of chordoma and chondroma of the skull base murray a.
It presents as a soft tissue mass without involvement of the skull base bone clivus and may mimic other lesions of the nasopharynx. Any information contained in this pdf file is automatically generated from digital. Cavernous sinus involvement was present in 45% of patients 19% on the right, 7% on the left, and 19% bilaterally. Charged particle radiosurgery clinical presentation depends on the anatomical in clival chordoma in a large number of patients has location of the tumour. We report the clinical presentation and management of a woman presenting with an ectopic prolactinoma located in the clivus. Jul 02, 2012 intracranial chordomas most often originate from the sphenooccipital synchondrosis of the clivus.
Surgical approaches to clivus chordoma are discussed. Chordoma of the clivus is a rare invasive and destructive tumor. Microsurgical versus endoscopic transsphenoidal approaches. Therefore, fungal infections are important among the various differentials, leading to skull base.
Chordoma ch and chondrosarcoma chs are rare tumors. A chordoma may also be mistaken for a nerve sheath tumor if its soft tissue component extends laterally to the neural foramen. Skull base the bones at the bottom part of the skull that separate the brain from other structures. However, they show an invasive local growth and high recurrence rate, leading to an overall survival rate of 65% at 5 years and 35% at 10 years. Ectopic pituitary adenomas are a rare clinical entity and are frequently mistaken for other base of skull lesions on imaging. Different regimens of chemotherapy and molecularly. Clinical study characteristics and patterns of metastatic. Twelve patients with histologically confirmed clivus chordoma were treated at the johns hopkins hospital between. Pdf dr avinash haridas, sohail ansari, fary afshar. New treatments are now available for both the local and the metastatic phase of the disease, but the degree of uncertainty in selecting the most appropriate treatment remains high and their adoption remains inconsistent across the world, resulting in suboptimum outcomes for many. Because of the risk of severe late com plications, the dose is often limited with conventional photon rt, chordoma is a rare malignancy thought to arise and the probability of cure is low. Clivus chordoma with an arachnoid cyst, coincidence or.
We present the case of a 5yearold boy with clival chordoma without bone involvement. 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. Combined endoscopic endonasal and posterior cervical approach to a clival chordoma. Guidelines for skull base isolated recurrence chordoma. Chordoma is a rare slowgrowing neoplasm thought to arise from cellular remnants of the notochord. Hemorrhagic chondroid chordoma mimicking pituitary apoplexy.
Aspergillus masquerading as clival chordoma surgical. Chordoma of the clivus areport of four cases byhans f. Chordoma found in a 38 year old male patient who then underwent resection. Pdf apparent diffusion coefficient as a prognostic. Chordoma genetic and rare diseases information center. Expert recommendations for the diagnosis and treatment of. If the inline pdf is not rendering correctly, you can download the pdf file here. When ghsecreting pituitary tumors appear before the fusion of epiphyses growth plates in children its called gigantism.
Threedimensional volume imaging to increase the accuracy of. The tumor is mainly locally destructive, and distant metastases are relatively rare. Coronal t1weighted, contrastenhanced magnetic resonance image of a clival chordoma demonstrating irregular borders at the clivus basisphenoid junction and abutting the carotid arteries laterally. Clival chordoma is a rare disease with high recurrence rates even after a combination of surgical resection and.
They occur most commonly in the skull base clivus and lower spine. Endocrinological assessment revealed modestly increased prolactin level with lower. Nov 20, 2020 chordoma are locally invasive slowgrowing malignant tumors that arise from the remnant of the primitive notochord. Khawaja1abdef, anand venkatraman2bdef, maira mirza3adef 1 department of neurology, massachusetts general hospital, boston, ma, u. Chordoma, proton beam therapy, dedifferentiation, pediatric tumors, oncology, case. 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. B, axial t2weighted image 6 months later shows interval enlargement of the mass involving the skin and subcutaneous tissues. Chordoma is a rare slowgrowing neoplastic bone lesion. Nov 29, 2012 introduction clival chordomas present with headache, commonly vi cranial nerve palsy or sometimes with lower cranial nerve involvement. Methods a total of 242 patients were retrospectively analyzed.
Nov 12, 2019 in this case report, we present a rare and previously unreported case of spontaneous regression of a histologically consistent clival chordoma. Chordoma genetic and rare diseases information center gard. We describe a case of cooccurrence of a sellarsuprasellar craniopharyngioma and a clival intradural chordoma, successfully treated via an extended, endoscopic endonasal approach. A 24yearold male with gigantism, growth hormone deficiency. A 26yearold male patient is presented with nasal obstruction and congestion for the last 3 years. Clival ectopic pituitary adenoma mimicking a chordoma. Case report open access clival chordoma with an atypical. If large enough, they may affect facial sensation or movement. Approximately 40% of chordomas and chondrosarcomas arise in the clivus directly below the sella turcica and pituitary gland.
Clival chordoma presenting with acute brain stem hemorrhage. Chordomas are rare, slowgrowing, and locally aggressive malignant neoplasms derived from primitive notochord remnants. Chordoma is a rare, slowgrowing malignant tumor arising from notochordal remnants. The optimal management of this rare tumor remains controversial. The most common signs of chordoma are pain and neurological changes. To distinguish chordoma from a nerve sheath tumor, scrutinize the adjacent vertebral artery. Chondroid chordoma has a lower recurrence rate and better survival as compared to conventional chordoma 9,10. We report an autopsy case of clival chordoma in a 61yearold woman. Clival chordomas are usually benign, although aggressive clival chordomas have been reported. Very rarely, a chordoma can arise intradural ly without any bony involvement 2.
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