Benign notochordal lesions of the posterior clivus. The patients were followed up with serial imaging at regular intervals as well as with neurological evaluation. A new, wellcharacterized clival chordoma cell line, as well as a. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. A, preoperative sagittal t1weighted magnetic resonance imaging mri scan with contrast, showing a clival chordoma. We use cookies to offer you a better experience, personalize content, tailor advertising, provide social media features, and better understand the use of our services. The resulting consensus guidelines were published in the medical journal the lancet oncology.
It is surrounded by the neurovascular structures of the brainstem, as well as both internal carotid arteries. Atypical clival chordoma in an adolescent without imaging. Although there is no racial predilection for chordomas, the incidence in. Stereotactic radiosurgery for skull base chordomas and. Evidencebased optimization of posttreatment surveillance. We present the first case of a patient with a clival chordoma in which complete tumor ablation was achieved using magnetic resonance guided laser induced thermal therapy litt. Nonchordomatous lesions of the clivus the jama network. Pdf endoscopic laser ablation of clival chordoma with.
The clival chordoma is a malignant tumor arising from remnant cells of the central notochord neuraxis, representing 0. The clinical, pathologic, and imaging characteristics of clival chordomas in 14 patients who underwent magnetic resonance examinations were evaluated. Purely ectopic pituitary adenomas are extremely unusual and must be confirmed via imaging and intraoperative visualization to be completely separate from an intact pituitary gland and the sella turcica. Methods a total of 242 patients were retrospectively analyzed.
Establishment of clival chordoma cell line mugcc1 and. B, postoperative sagittal t1weighted mri scan with contrast, showing a clival chordoma after gross total resection was achieved with the endoscopic endonasal approach eea. Chordoma symptoms and treatment upmc pittsburgh, pa. Patients with recurrent disease or previously treated with radiation therapy may be more likely to have decreased rates of local control and os. Residual tumors have been treated with adjuvant focal radiation therapy employing highenergy. Such specialized imaging techniques may include computerized tomography ct scanning and magnetic resonance imaging mri. You will get magnetic resonance imaging mri and computed tomography. On a rare cutaneous metastasis from a sacrococcygeal chordoma ct imaging showed a suboccipital cranial cleft extending through a clival defect and a schisis of the anterior arch of the c1 vertebral body and hypoplasia of the left mandibular ramus. Morphological and dynamic contrast enhanced mr imaging. Sep 25, 2007 chordoma and chondrosarcoma of the skull base are rare tumors. Chordoma cancer causes, symptoms, life expectancy, treatment.
Chordomas originate from embryonic remnants of the primitive notochord which extends from the level of rathkes pouch to the coccyx. Visual complications of proton beam therapy for clival. The symptoms and imaging features of lumbar spine chordoma are not specific, and the pathological findings are. The primary site of origin is the clivus hence, clival chordomas, which is a junction between two skull bones, the sphenoid and occipital bones. Magnetic resonance imaging mri revealed a large clival tumour extending into the retropharyngeal space and displacing the. On imaging, both are characterized with osteolytic bone destruction, lesions of heterogeneous signal intensity, and cystic regions.
The high recurrence rate of these tumors requires frequent followup and monitoring. Jul 22, 2017 chordomas of the skull base have high recurrence rates even after radical resection and adjuvant radiotherapy. The clival region is the second most common location, accounting for 3035% of cases 2,3. Endoscopic management of clival chordomas and chondrosarcomas. Chordoma and chondrosarcoma of the skull base are rare tumors with overlapping presentations and anatomic imaging features but different prognoses. The lesion has a markedly prolonged t2, producing marked hyperintensity within it on t2 weighted scans. Clival chordoma is a rare primary bone tumour that arises from the remnant of the notochord and typically occurs in older adults. Background the clival chordoma is a malignant tumor arising from remnant cells of the central notochord neuraxis, representing 0. Solitary intracranial plasmacytoma sip is very rare. Chordomas are generally slow growing and are most commonly found within the head at the base of the skull near a bone called the clivus and in the lower portion of the spine. A 41yearold male presented with an incidental clival lesion presumed to be a chordoma. Clival ectopic pituitary adenoma mimicking a chordoma. Recent advances in threedimensional reconstruction techniques and measurement software have greatly improved the surgeons ability to perform detailed preoperative planning. Currently, available imaging software allows 3d anatomical.
Visual complications of proton beam therapy for clival chordoma. This difficulty is enhanced by the very small likelihood of a successful complete surgical resection or nonsurgical treatment of chordoma. The patient tolerated the procedure well and is being followed with serial imaging. T2weighted mr image show an intracranial chordoma with heterogeneous but mostly high signal. Head imaging revealed a large expansile and erosive mass in the skull base. Sometimes, especially at the base of the spine, a lump will be found. The lesions are expansile with destructive or lytic lesions in the bone. This sagital from the side t1weighted mri image shows a large, destructive mass involving the clivus, sphenoid sinus, and sella turcica which represents a chordoma. Imaging by computed tomography ct or magnetic resonance imaging mri can identify clival chordoma based on its midline location and destruction of the clivus 5.
Typically the mass projects posteriorly at midline, indenting the pons. The imaging findings are compatible with a clival chordoma. Clinical information, including extent of resection, al. Purpose to determine differentiation of chordoma and chondrosarcoma of the skull base with conventional magnetic resonance imaging cmri and diffusionweighted mr imaging dwi in.
An awardwinning, radiologic teaching site for medical students and those starting out in radiology focusing on chest, gi, cardiac and musculoskeletal diseases containing hundreds of lectures, quizzes, handout notes, interactive material, most commons lists and pictorial differential diagnoses. The visualase software provides a realtime estimate of thermal damage area. It is most often found near the tailbone called a sacral tumor or where the spine meets the skull called a clival tumor. Soft tissue mass in the left side of the clivus extending into the tip of the left petrous bone and the left cerebello pontine angle. We analyzed the safety and feasibility of this approach and quantified the response of this pathology to thermal energy. Chordoma located in the skull base is usually a challenging surgical condition. Their research is aimed at discovering the pathogenesis of chordomas and uncovering tumor markers to guide future therapies. For diagnosis, this team should include a radiologist and pathologist with chordoma experience. Three standard treatments for skull base chordoma are surgery, chemotherapy, and radiation therapy.
A report of a case of recurrent chordoma of the clivus is presented to illustrate. Were excited to announce some fantastic new additions to both our board of directors and our boards patient services committee. We believe passionately that every chordoma patient should be able to make informed. In this study, the authors aim was to research clinical features and prognostic factors in patients harboring clival chordomas and explore the relationship between plateletderived growth factor receptor. Chordoma slowly growing destructive tumor histologically benign, but locally invasive onethird in sphenooccipital region most in midline. A 77yearold female was referred because of diplopia and progressively worsening headaches. Adc maps were generated with commercially available software. The authors provide important data on the outcomes following a consistent aggressive approach to the management of clival chordoma in the pediatric population. Apr, 2016 establishment of clival chordoma cell line mugcc1 and lymphoblastoid cells as a model for potential new treatment strategies. Dec 29, 2015 the imaging findings are compatible with a clival chordoma. Stateoftheart imaging in human chordoma of the skull base ncbi. Endoscopic laser ablation of clival chordoma with magnetic.
Each of these individuals brings a unique set of skills that will improve our efforts to serve and support the needs of the chordoma community. Oct 03, 2014 imaging strongly enhancing, highly vascular nasopharyngeal soft tissue mass. Clival disorders comprise a large range of entities with different characteristics and behaviors. Chordoma and chondrosarcoma are locally invasive skull base tumors with similar clinical symptoms and anatomic imaging features as reported in the literature. As pointed out by the authors, these tumors present insidiously and are almost always large at. While imaging tests like an mri 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. Both computed tomography ct and magnetic resonance imaging mri are crucial in the initial diagnosis. Although chordoma are generally slowgrowing lesions, the recurrence rate is high and the location makes it often difficult to treat. Chordoma treatment program massachusetts general hospital. Chordoma is a slow growing cancer of tissue found inside the spine.
Influence of residual tumor volume and radiation dose. Chordomas are rare primary bone tumors arising from embryonic remnants of. Chordoma is a rare tumor originating from notochordal remnants that is usually diagnosed during midlife. Learningradiology sacrococcygeal, chordoma, sacral, coccyx. Patients with saddle area chordoma are more prone to dizziness and other symptoms. This case report describes our diagnosis of clival chordoma in an adolescent with no clival destruction seen on mri or ct. The chordoma foundation and the european society for medical oncology brought this group together to define the recommendations for treating and diagnosing chordoma based on all available medical and scientific evidence. Dec 05, 2018 although the underlying causes of chordoma are less understood, chordomas originate in the bone, and as they grow, they destroy the bone and extend to the brain and soft tissue. They include both chordoma foundation directors and external advisers who provide expertise and perspective in subjects relevant to the business of the committee. Chordoma is a rare cancer that affects bones in the spine and skull. The maximum twopoint lod score based on only the affected individuals was 2. Chordoma is a primary malignant bone cancer that develops from remnants of embryonic notochord in the skullbase head and spine. If the chordoma has a significant chondroid component, focal regions of hyperdensity may be present. A chordoma occurs when additional notochord cells are enclosed by the developing bones.
Imageguided, intensitymodulated radiation therapy igimrt. Diffusion weighted mr imaging may be useful in assessing clival tumors, particularly in differentiating chordoma from. Between 1984 and 2015, 22 patients diagnosed with an intracranial chordoma were treated at the karolinska university hospital, stockholm, sweden. Close inspection of imaging reveals bncts at a similar frequency to its pathologic prevalence. The two most common locations for chordomas are the lower back sacral area. Clival chordomas are a visual diagnosis for radiologists with a differential diagnosis of osteochondroma and ecchordosis physaliphora. The clival region is the second most common location, accounting for 3035. Differential diagnosis of clival lesions literature. Imaging findings of the pediatric clivus chordomas request pdf.
An mri is the best way to see a chordoma and how it is affecting the tissue around it, such as muscles, nerves, and blood vessels. A 54 yearold female with a newly diagnosed clival chordoma elected mri. Residual tumors have been treated with adjuvant focal radiation therapy employing highenergy particles most commonly through proton beam. We present the first case of a patient with a clival chordoma in which complete tumor ablation was achieved using magnetic resonance guided laser induced thermal therapy litt delivered via an endoscopic endonasal approach. Barbara rombi and beate timmermann 2014 proton beam therapy for pediatric chordomas. Imaging disclosed large tumors that often involve multiple anatomical compartments. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. Radical tumor resection was achieved in 58% of the group. The tissue is examined under a microscope, the chordoma can have one. Report atypical clival chordoma in an adolescent without. There was no clival chordoma in our group of patients, which is the most common tumor in this region. Webmd explains what causes this cancer, and why it can be tricky to treat. Recent advances in understanding and managing chordomas ncbi. The clivus is the surface of a portion of the occipital and sphenoid bones in the base of the skull.
Chordoma surgery is the main type of treatment for bone. Aug 23, 2016 for one patient with clival chordoma, recist showed an increase of 5 %, whereas volume indicated a decrease of 32. She underwent placement of the laser catheter into the chordoma via an endoscopic endonasal. Overexpression of the emt driver brachyury in breast carcinomas. The treatment of clival chordoma remains highly challenging. Your doctor may recommend imaging tests to help visualize your chordoma and determine whether it has spread beyond the spine or skull base. Is there a role for conventional mri and mr diffusion. Complete tumor resection is directly related to lower recurrence rates and potential cure. Because chordoma lie in bone, they are usually extradural and induce bone destruction see the images below. Stateoftheart imaging in human chordoma of the skull. Threedimensional volume imaging to increase the accuracy of. Dec, 2015 because chordoma lie in bone, they are usually extradural and induce bone destruction see the images below.
Chordoma surgical approach neurosurgery stanford medicine. Diffusion mr imaging may be useful in assessing clival tumors, particularly. Low back pain is a common symptom of thoracolumbar spinal chordoma. Upon imaging, the tumour can be seen arising from the clivus and causes clival destruction. We report our preliminary outcomes following highdose imageguided intensity modulated radiotherapy igimrt for skull base chordoma and chondrosarcoma. In 38 patients, the imaging studies were evaluated for listed features. Data were acquired and analyzed with rtca software version 1. Bilateral synchronous involvement of knee and hip joints with primary lipoma arborescence in a young male. Endoscopic transsphenoidal resection of a large clival chordoma in the posterior fossa. Frontiers management of chordoma and chondrosarcoma with. This case demonstrates fairly characteristic imaging features of a clival chordoma which was subsequently histologically confirmed. Patients who present with meningitis and cerebrospinal fluid rhinorrhoea could have an underlying erosive lesion which can sometimes be. Chordoma is a rare slowgrowing neoplasm thought to arise from cellular remnants of the notochord. Chordoma is the most common primary malignant sacral tumor 9,10.
Mar 02, 2016 for chordomas, ct imaging is superior for detecting bony destruction, whereas mri is useful for assessing the diagnosis and for defining tumor extension. A 54 yearold female with a newly diagnosed clival chordoma elected mri guided litt. Case summary a 28yearold male presented to the orthopedic outpatient department with a threeyear. Chordoma is a tumor arising in the nucleus pulposis, presumably from remnants of notochord thus its exclusive origin is in the sacrococcygeal region, sphenooccipital region, and vertebral bodies, otherwise known collectively as the axial skeleton. The neurosurgical team at upmc may recommend one or a combination of these treatments. Purely ectopic pituitary adenomas are exceedingly rare. On mri, the appearance of a chordoma is similar to the appearance on ct scan, with better resolution of the softtissue component, resulting in better anatomical definition.
Welcoming new board and committee members chordoma foundation. 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. Bncts such as ep vary in size and may be easily overlooked. In other cases, lesions show up on computed tomography ct and magnetic resonance imaging mri scans a biopsy will be done to diagnose a chordoma and to differentiate it from other types of tumors.
This novel technique is intended to provide chordoma patients with an alternative to surgery and radiotherapy. Familial chordoma, a tumor of notochordal remnants, is linked. Your doctor may recommend imaging tests to help visualize your chordoma and. Often, sacral chordomas do not cause symptoms until the tumor is quite large, and.
Solitary intracranial plasmacytoma located in the spheno. Endoscopic endonasal surgery is the preferred surgical option for most skull base chordomas, however. Mri, or merged ctmri image sequences using software applications on navigational systems. Plain xrays radiography or other specialized imaging techniques may be used to diagnose a chordoma. A biopsy may also be needed to definitively diagnose and type the clival tumor as a chordoma or chondrosarcoma. Magnetic resonance imaging mri was compared with skull series, tomography, computed tomography ct, and magnification angiography in the diagnosis of clival chordomas. In addition, sacral lesions may go undetected on imaging, as ct and mri.
Endoscopic anatomy of the clivus and posterior fossa neupsy key. We hypothesized that these tumors might be distinguished by using diffusionweighted mr imaging. The readers were aware that all patients were initially diagnosed histopathologically as having chordoma. Fortytwo consecutive igimrt patients, with either skull base chordoma n 24 or chondrosarcoma. Skull basexthe bones at the bottom part of the skull that separate the brain from other structures. Developed by renowned radiologists in each specialty, statdx provides comprehensive decision support you can rely on chordoma. We evaluate the longterm clinical outcome using multidisciplinary management in the treatment of clival chordomas.
Chordomas are rare cancerous tumors that can occur anywhere along the spine, but most often are found either at the top skull base or bottom sacrum of the spine. It is often not possible to achieve gross total resection. This case report presents serial findings of sip located in the spheno clival region in a 54year old female who presented with an inferior he. At stanford, our primary goal when performing chordoma surgery is maximal and safe resection of the tumor. Detailed imaging with magnetic resonance imaging mri or computed tomography, or ct scans can also be used to distinguish a clival tumor from other types of skull tumors. During ct scanning, a computer uses xrays to create crosssectional images of the body. Chordoma nord national organization for rare disorders. Fat suppression imaging with suppression of the fatty bone marrow of the clivus may be useful in the delineation of clival chordoma 44. Peer connect is a free, confidential peertopeer support program that connects.
Board committees provide ongoing oversight and strategic planning for the foundations key functional areas. We performed a genomewide analysis for linkage in a family with 10 individuals affected by chordoma. Chordoma are rare tumours of the axial skeleton which occur most often at the base of the skull and in the sacrum. Differentiating benign notochordal cell tumors from chordomas.
Jun 23, 2017 fractionated stereotactic radiotherapy for chordoma and chondrosarcoma is feasible, well tolerated, and provides rates of os and lrfs comparable to published data for other treatment modalities. At first, radiological imaging did not reveal a tumoral condition, though intraoperative exploration and tissue histology revealed a chordoma which eroded her clivus and had a transdural extension. Understanding chordoma in children and young adults. These are aggressive primary malignant tumours of notochordal remnants. Many notochordal lesions do not fit neatly into the diagnostic criteria for either ep or chordoma. Predicting clinical outcomes in chordoma patients receiving.
However, mr imaging is the single best imaging modality for both pre and posttreatment evaluation of intracranial chordoma. It most often forms where the skull sits atop the spine skull base or at the bottom of the spine sacrum. The goal of surgery for a sacral spine cancer is to remove all of the cancer. 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. Imaging by computed tomography ct or magnetic resonance imaging mri can identify clival chordoma based on its midline location and destruction of the clivus. It may be useful to consider these atypical cases along a spectrum of notochord remnant lesions.
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