Effects of primary and recurrent sacral chordoma on the motor. This survival rate of patients with recurrent sacral chordoma is lower than that of patients with primary tumors in previous reports. Assessment of tumor response to radiation therapy by volumetric analysis is superior to modified response evaluation criteria in solid tumors in chordoma patients, and evaluating the soft tissue target volume is the best indicator of tumor response to radiation. Effects of primary and recurrent sacral chordoma on the. Chordomas are rare malignant tumors, with an incidence of approximately 1% to 4% of all malignant bone tumors. Chordoma is the most common primary malignant sacral tumor 9,10. Imaging studies with pre and postradiation scans can help one understand the effect of radiation on bone. Your doctor may recommend imaging tests to help visualize your chordoma and. Case report, abstract chordomas are rare tumors of notochordal tissue remnants and most frequently affect the sacrum and skull base. Different components that constitute the sacral bone may induce local tumors. Case report longterm tumour control in sacral chordoma following highdose palliative image guided intensitymodulated. Sep 01, 2009 symptoms of sacral chordoma are indolent and include pain, numbness, constipation, weakness, and incontinence. S4854 march 2011 with 173 reads how we measure reads.
Clinical examination was unremarkable with normal power and sensation in the lower limbs. Guidelines for newly diagnosed mobile spine and sacral chordoma. Both computed tomography ct and magnetic resonance imaging mri are crucial in the initial diagnosis. Incidence, treatment, and survival patterns for sacral chordoma in. Data from the surveillance, epidemiology, and end results seer program. Chordoma is an uncommon malignant bone tumor accounting for only 1 to 4% of all primary malignant bone tumors 1, 2. Mr image of a chordoma shows a destructive bone lesion in the sacrum that is hypointense on t1w images, hyperintense signal on t2w and enhancement of the lesion after gadolinium. Chordoma of the sacrum en bloc high partial sacrectomy duration.
Most of these have been situated, as was this one, in the sacral area, the next most frequent site being the sphenooccipital region, although in the. Chordoma s are slower growing, do not spread as easily. The typical chordoma contains these cells, while a chondroid chordoma has areas of cartilaginous tissue. Computer navigationaided resection of sacral chordomas ncbi. Often, sacral chordomas do not cause symptoms until the tumor is quite large, and.
Treatment options surgical resection of sacral chordomas is the firstline treatment option for these tumors. What is the effect of highdose radiation on bone in. Sep 25, 2007 chordoma and chondrosarcoma of the skull base are rare tumors. Jackson a, scott s, romanchikova m, noble dj, burnet ng.
Questionspurposes the purpose of this study was to assess the effects of highdose radiation on the trabecular density of bone in the sacrum using ctderived hounsfield units hu. Radiographs may show sacral osteolysis with an associated softtissue mass and calcifications. Imaging characteristics of metastatic chordoma springerlink. Mar 03, 2016 trauma relating to the sacrum may well be associated with other injury to the pelvic ring and imaging of the pelvis is likely to be required in addition to examination of the sacrum.
Whether recent advances in imaging technology corresponded any. This patient was a man aged fortyone, a manufacturer, who came to the clinic september 10, 1929, complaining of pain in the lower part of his back. Imaging chordoma and chondrosarcoma of the vertebrae and. A chordoma can develop anywhere along the spine from the base of the skull to the tailbone coccyx. A 53yearold man presented to our service with a 1month history of subacute bowel obstruction. Dec 28, 2017 chordoma treatment depends on the size and location of the cancer, as well as whether it has invaded nerves or other tissue. Although the imaging findings of sacral tumors are nonspecific, a patients age and sex, and specific findings such as calcification or fluidfluid levels, can help radiologists in their differential diagnosis. Sacral chordomas often demonstrate a great deal of t2 hyperintensity. Chordoma is a rare type of bone cancer that happens most often in the bones of the spine or the skull. We performed a retrospective analysis of 30 patients with unresectable sacral. For all types of external beam radiation, imaging is needed every day of treatment to make sure that the. Chordoma symptoms and treatment upmc pittsburgh, pa.
Chordoma nord national organization for rare disorders. Noninvasive sacral chordoma presenting as a benign soft. What is the effect of highdose radiation on bone in patient. Journal of jeff heads bone cancer experience sacral chordoma. Chordoma surgery is the main type of treatment for bone cancers.
Usually the diagnosis is late and discovered by signs of pelvic or nervous compression. The gross appearance of chordoma is an expansile softtissue mass with a lobualted appearance and cystic spaces filled with gelatinous material. Preoperative differentiation between primary sacral chordoma sc, sacral giant cell tumor sgct, and sacral metastatic tumor smt is important for treatment decisions. The posttreatment mri 12 months after radiation shows sacral insufficiency fractures in both massae laterales of the sacrum white. Surgical resection is the primary treatment of choice, and the quality of surgical margin is the most important risk factor for. The retropulsed vertebral body narrows the spinal canal and compresses the conus medullaris, seen. Rats engrafted with chordoma into the spines were imaged by cone beam computed tomography cbct at day 450 after tumor implantation, using a small animal research radiation platform xstrahl life sciences as previously described. The purpose of this study was to establish an mr imaging grading system for skull base chordoma. They are not too responsive to chemo or radiation and the primary treatment appears to be to remove it. One hundred ninetyeight chordoma patients were registered in the institutional database and were followed up with ct and mr examinations for periods ranging from 1 to 158 months. Sacral chordomas account for 50% of all chordomas 1, 2, 3. Left axial ct scan demonstrates a large soft tissue mass in the pelvis of mixed attenuation containing at least one fragment of bone black arrow.
The lumbar spine is an unusual location for spinal chordoma. Role of the apparent diffusion coefficient as a predictor. Hi alan i have one off the best doctors on the field on spine tumors i just did a surgery on the back remove the tail bone and also part of the sacrum bone he is one off the best in the us and if you want i will give his name and telephone number he safe my life after some may doctors they try to operate i got diagnose with chordoma cancer on the tail bone also part off my sacrum bone was. Due to its ectodermal origin, chordoma is not properly a sarcoma even if it has clinically retained and. We report a case of a 58yearold woman having cervical chordoma causing related symptoms for 5 years. Sacral chordomas represent one half of all chordomas, a rare. This study is the first report on unresectable sacral chordomas treated with. Clinical and imaging features of cervical chordoma. The type of surgical procedure depends on the size and location of the tumor. 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. Learningradiology sacrococcygeal, chordoma, sacral, coccyx.
Sacral chordoma is one of the most challenging malignancies to treat. Longterm tumour control in sacral chordoma following high. Jan 28, 2010 adequate surgery still remains the only curative treatment of chordoma. Role of the apparent diffusion coefficient as a predictor of. Longterm tumour control in sacral chordoma following highdose palliative image guided intensitymodulated radiotherapy igimrt. A total of 56 patients were included in this retrospective study. Skull base chordoma has been widely studied in recent years, however, imaging characteristics of this tumor have not been well elaborated. Mri of the pelvis identified a welldefined presacral midline lesion of 9.
Chordoma and chondrosarcoma of the skull base are rare tumors. They may also use a ct scan to see if the tumor has spread. Chordoma is a type of malignant cancerous tumor that most often occurs in the bones of the spine and base of the skull. It accounts for 14% of all primary skeletal tumors and its incidence rate is inferior to 0.
It most often forms where the skull sits atop the spine skull base or at the bottom of the spine sacrum. However, mr imaging is the single best imaging modality for both pre and posttreatment evaluation of intracranial chordoma. The clival region is the second most common location, accounting for 3035% of cases 2,3. Chordoma are rare tumours of the axial skeleton which occur most often at the base of the skull and in the sacrum. If the chordoma affects the lower portion of the spine sacrum, treatment options may include. Chordomas, which arise from notochordal remnants, have slower local growth and metastasize less frequently than other bone and soft tissue malignant tumors 1, 3, 4. Abstractin the present paper, the writers offer another authentic case of chordoma to add to the rather meagre number now reported. Chordoma is a rare slowgrowing neoplasm thought to arise from cellular remnants of the notochord.
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. On ct scan, the typical description of a clival chordoma is a welldefined, hyperdense softtissue mass with. Ct scan of a sacral chordoma demonstrates a lytic lesion of the sacrum with a soft tissue mass. Chordoma accounts for more than 50% of lesions located in the sacral area. 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. Since then, en bloc resection has remained the mainstay of treatment of sacral chordoma worldwide, as reported by a large multicentric study based on the aospine tumor knowledge forum primary spinal tumor database. Surgical resection is the primary treatment of choice, and the quality of surgical margin is the most important risk.
Carbon ion radiotherapy for sacral chordoma the british. The tumor cells have oval to round, occasionally spindled, hyperchromatic nuclei with occasional large. Methods between 2009 and 2015, we treated 57 patients older then 18 years with sacral chordoma. Options may include surgery, radiation therapy, radiosurgery and targeted therapies. Chordoma, computerassisted navigation, resection, sacral. It is located on the midline and confined to the axial skeleton sacral chordoma usually grows on the anterior side of sacrum and presents with destruction of sacral bone by a large soft tissue mass. Aug 23, 2018 this study aimed to analyse the frequency and clinical relevance of sacral insufficiency fractures sifs after highdose carbonion based irradiation of sacral chordomas. This study explores the prognostic role of the apparent diffusion coefficient in chordomas.
An mri is the best way to see a chordoma and how it is affecting the tissue around it. 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. Fourteen 25% of them were treated with radiation only. Sacral chordoma patient discusses cancer treatment duration. Report atypical clival chordoma in an adolescent without. We describe a case of longterm tumour control in a chordoma of the lumbar spine following highdose palliative radiotherapy. Computer navigationaided resection of sacral chordomas. These two reports, however, used ct imaging alone, which was unable to confirm soft tissue margins. Although chordoma are generally slowgrowing lesions, the recurrence rate is high and the location makes it often difficult to treat. We described the clinical outcome of a series of chordoma patients followed at regina elena national cancer centre of rome from 2004 to 2008. Being on the sacrum, that can be very tricky, particularly depending on size. There is a t1 hypo and hyperintense mass centered destroying the sacrum. Plain radiograph of the pelvis showing expansion of the sacrum, bone rarefaction, and large mass of soft tissue with.
Sacral chordoma cleveland clinic journal of medicine. Carbon ion radiotherapy for unresectable sacral chordomas. Symptoms of sacral chordoma are indolent and include pain, numbness, constipation, weakness, and incontinence. The goal of surgery for sacral chordomas is to completely remove the tumor in one piece enbloc with wide. When a chordoma is suspected, you will need magnetic resonance imaging 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. However, this case proved to be diagnostically challenging. Right sagittal reformatted ct shows the mass white arrows is destroying most of the sacrum and coccyx red arrow. The clinical outcome of recurrent sacral chordoma with. Sacral insufficiency fractures after highdose carbonion. Twenty one patients 37% were treated with definitive radiotherapy rt, and 35 patients 63% received postoperative rt using carbon ions, either in.
Twentyfive consecutive patients with sacral 11 patients, spine patients, and. Chordomas of the skull base may be approached directly using the endoscopic endonasal approach eea. To the best of our knowledge, this study is the first to use combined ct and mri for navigationaided resection of sacral chordomas. You will get magnetic resonance imaging and computed tomography scans that.
Imaging is useful not only for diagnostic purposes, but also for selection of operative approaches. Histology the sections show strands and clusters of atypical epithelioid cells within a myxoid matrix. When you have symptoms of chordoma, your doctor will take an image of your spine using an mri to look at the size of the tumor and where it is. Over the next six months, she began to experience progressive sacral pain. Interesting clinical data on advanced disease with molecularly targeted therapies were reported. En bloc sacrectomy is a highly demanding surgical procedure, consisting of a partial or total amputation of the sacrum. Expert recommendations for the diagnosis and treatment of. The tumors are slowgrowing, but can be difficult to treat because of their location near vital structures such as the spinal cord and brain. Imageguided intensitymodulated radiotherapy with photons provided a good solution to deliver 65 gy to the tumour in a technically challenging case, and local control has been sustained over a period of years. In cases of severe trauma it is inadvisable to undertake a lateral projection of the sacral area in the position described in this section. A sagittal ct a image demonstrates a mixed sclerotic and lytic lesion with a pathologic fracture arrow and vertebral body collapse. Treating chordomas can involve extensive surgery, so your doctor will be sure to have a definite diagnosis before planning your individualized treatment. Eight patients had followup mr examination after treatment. Typically the mass projects posteriorly at midline, indenting the pons.
The role of imaging exploring sacral bone tumors is very important. 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. Probably, chordoma is not so rare as the number of reported cases indicates, many such tumors being either overlooked or incorrectly diagnosed. Imaging characteristics of a lumbar spine chordoma.
She was asymptomatic at the time of diagnosis and initially elected to pursue close monitoring only. Highdose definitive radiation therapy is feasible in treating selected patients with unresected mobile spine and sacral chordomas. To check if the tumor is chordoma, your doctor will do a biopsy, taking a small sample from the tumor with a needle. The notochord disappears when the fetus is about 8 weeks old, but some notochord cells remain behind in the bones of the spine and skull base. At imaging, a chordoma typically manifests as a large destructive sacral mass with secondary softtissue extension. Chordomas are difficult to excise completely because preservation of sacral stability and sacral nerve pathways to the rectum and bladder limit the extent of surgery. The purpose is to evaluate the efficacy and toxicity of carbon ion radiotherapy for unresectable sacral chordomas. Surgery is the mainstay but complete excision is sometimes difficult to achieve if the tumour volume is large andor the tumour invades the upper levels of the sacrum e. The mr images were performed with t1 and t2weighted imaging, and gadolinium gdenhanced imaging. The mass shows low attenuation and some internal calcifications.
Radaelli et al 19 reported that among 99 patients with primary sacral chordoma, the 5year and 10year survival rate was 92% and 63%, respectively. If the chordoma affects the lower portion of the spine sacrum, treatment. The educational objectives for this selfassessment module are for the participant to exercise, selfassess, and improve his or her understanding of sacral masses and the key imaging characteristics of various sacral masses. Diffusionweighted imaging may aid in distinguishing aggressive chordoma from nonaggressive chordoma.
Large osteolytic lesion arising in the midline from the lower sacrum. This article is either under construction or in the approval process. They are known to develop anywhere along the spinal axis 32% from the skull base, 32. 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. May 16, 2017 laparoscopic dissection and anatomy of sacral nerve roots and pelvic splanchnic. Aug 12, 2015 she underwent a ctguided biopsy demonstrating chordoma. Pdf computer navigationaided resection of sacral chordomas. Chordoma is a rare, lowgrade, primary malignant bone tumour arising from primitive notochord remnants of the axial skeleton. The spine and sacral chordoma center at rhode island hospital combines extensive experience and technical expertise in the multidisciplinary treatment of sacral chordomas. In this study, 156 patients with skull base chordomas were retrospectively assessed. Sacral chordoma also has poor sensitivity to chemotherapy.
This article discusses various protocols for plainfilm and ct imaging. Spine and sacral chordoma center lifespan brain and. Stateoftheart imaging in human chordoma of the skull. Sixteen patients with residual or recurrent chordoma were divided postoperatively into those. Mr images images demonstrate a t1 hypointense and t2 hyperintense mass arising from the sacrum.
An mri is the best way to see a chordoma and how it is. In this study, 156 patients with skull base chordomas were. Nov 10, 2016 wippold fj 2nd, koeller kk, smirniotopoulos jg. Clinical features and ct and mr findings of metastatic chordomas were analyzed by. In the imaging tests of the primary chordoma, bone destruction, calcifications, osteosclerosis in the nearby bone and the presence of a soft tissue mass with. Of the two patients with sacral chordomas that did not have surgery, one received. Imaging the sacrum and coccyx imaging of the sacrum and coccyx is a common procedure in radiology. Not good news, but not as bad as feared with multiple myeloma. Sixteen patients with residual or recurrent chordoma were divided postoperatively into those with an aggressive tumor, defined as a growing tumor having a.
Some studies have reported that photon radiation therapy may possibly delay recurrence after incomplete resection and may also be able to relieve symptoms caused by recurrences 5, 9, 12. Local surgical resection is the gold standard of treatment for sacral chordomas. Pdf carbon ion radiotherapy for unresectable sacral chordomas. The current study is a retrospective series of patients with sacral chordoma treated with a combination of surgery and radiation at our institution between may 2009 and december 2015. We describe the imaging findings of primary sacral tumors, emphasizing the mri findings.