Materiały źródłowe

• #1 Diagnosis and treatment of chordoma – PubMed

  https://pubmed.ncbi.nlm.nih.gov/23744869/

  Chordoma is a primary bone cancer arising on the midline from the skull base to the sacrum. Diagnosis is often delayed because of insidious onset and nonspecific symptoms. […] Management centers primarily on radical en bloc surgical resection when possible. Radiation therapy using protons and/or photons is often necessary because complete resection is seldom possible due to critical location and invasion of the cancer cells into surrounding structures. […] The high rate of recurrence is reflected by a median survival of 6 to 7 years.

• #2 Diagnosis and Treatment of Chordoma in: Journal of the National Comprehensive Cancer Network Volume 11 Issue 6 (2013)

  https://jnccn.org/view/journals/jnccn/11/6/article-p726.xml

  Chordoma is a primary bone cancer arising on the midline from the skull base to the sacrum. Diagnosis is often delayed because of insidious onset and nonspecific symptoms. […] Chordomas are typically diagnosed at a locally advanced stage due to insidious onset and nonspecific symptoms. […] Fine-needle aspiration or core needle biopsy is the preferred method of diagnosis before surgical resection. Tumor seeding is a significant concern after biopsy, thus the tract of the biopsy needle should be marked and excised with the tumor. […] Previously, chordomas were diagnosed by their histopathologic features and immunoreactivity for S-100 and epithelial markers (eg, cytokeratins). […] Brachyury has emerged as an integral distinguishing biomarker for chordoma from other chondroid lesions. […] Histologic characteristics predictive of recurrence include number of mitotic figures and Ki-67 labeling index greater than 6%.

• #3 Chordoma: What It Is, Types, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17916-chordoma

  Chordomas are rare. They affect about 1 person for every 1 million people per year. That means that about 300 people receive a chordoma diagnosis each year in the United States. […] The only way healthcare providers can definitively diagnose chordoma is with a biopsy usually a needle biopsy. This involves taking a small sample of the tumor so a specialist can examine it under a microscope. […] Your healthcare provider will ask about your symptoms and medical history. Theyll likely perform a physical exam and a neurological exam. […] If they suspect a tumor, theyll order an imaging test, such as an X-ray, computed tomography (CT) scan or MRI scan. […] Your provider will likely refer you to a bone cancer specialist for a second opinion and confirmation of the diagnosis. You may need additional imaging tests to better determine the location of the chordoma and see if its spread to other areas of your body.

• #4 Symptoms and diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/diagnosis/

  Chordoma is not always easy to diagnose and it can be confused with other types of tumors. Getting the correct diagnosis from the start is critical to being able to find and choose the best treatment. […] Any biopsy or surgery to confirm the diagnosis of chordoma should be done at a center with expertise in chordoma treatment. If not done properly, these procedures can cause the chordoma to spread. […] If you have had an MRI and been given a probable or differential diagnosis of chordoma, it is very important to find doctors who have experience diagnosing and treating chordoma patients to confirm the diagnosis. […] Once you have been given a diagnosis, it can be helpful to get a second opinion to confirm the diagnosis before making treatment decisions. […] It is important to go to a hospital, treatment center, or network of treatment centers where doctors have experience diagnosing and treating chordoma as soon as chordoma is suspected.

• #5 New diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/treatment-guidelines/new-diagnosis/

  If chordoma is suspected or has been diagnosed, the most important thing to do is find a medical center with expertise in treating chordoma patients. […] If you’ve been told you might have chordoma, it is important to be evaluated by a multidisciplinary team of specialists who have substantial experience treating chordoma, even before you know for sure if you have it. […] Chordoma tumors are typically detected through imaging tests, which show organs and other structures inside the body, including tumors. The way the tumor looks on imaging tests can tell a radiologist whether the tumor might be chordoma. […] When a chordoma is suspected, you will need magnetic resonance imaging, also called MRI, to help doctors make a diagnosis and plan for treatment. […] While imaging studies can show the possibility of a chordoma, a definitive diagnosis can only be made by a pathologist who examines a sample of tumor tissue under a microscope.

• #6 Symptoms and diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/diagnosis/

  Chordoma tumors are typically detected through imaging tests, which show organs and other structures inside the body, including tumors. […] When a chordoma is suspected, you will need magnetic resonance imaging, also called MRI, to help doctors make a diagnosis and plan for treatment. […] Imaging tests can show the possibility of a chordoma, but a definitive diagnosis can only be made by a pathologist who examines a sample of tumor tissue under a microscope. […] Tissue samples should be evaluated by a pathologist who has experience diagnosing bone tumors. […] Your pathologist may test your tumor tissue for the presence of a protein called brachyury (also known as TBXT). Nearly all chordomas have high levels of brachyury, which makes it helpful for diagnosis. […] If tissue is not available for testing, the possibility of a biopsy to obtain tissue for this testing should be discussed with a doctor who has experience treating chordoma, carefully weighing the potential risks of biopsy. […] Interpretation of the testing should be done by a pathologist who has experience diagnosing chordoma.

• #7 New diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/treatment-guidelines/new-diagnosis/

  If chordoma is suspected or has been diagnosed, the most important thing to do is find a medical center with expertise in treating chordoma patients. […] If you’ve been told you might have chordoma, it is important to be evaluated by a multidisciplinary team of specialists who have substantial experience treating chordoma, even before you know for sure if you have it. […] Chordoma tumors are typically detected through imaging tests, which show organs and other structures inside the body, including tumors. The way the tumor looks on imaging tests can tell a radiologist whether the tumor might be chordoma. […] When a chordoma is suspected, you will need magnetic resonance imaging, also called MRI, to help doctors make a diagnosis and plan for treatment. […] While imaging studies can show the possibility of a chordoma, a definitive diagnosis can only be made by a pathologist who examines a sample of tumor tissue under a microscope.

• #8 Chordoma | Brain Tumor Center | Stanford Medicine

  https://med.stanford.edu/brain-tumor/conditions/chordoma.html

  A dedicated skull base MRI scan is the first step in diagnosing chordoma. […] However, diagnosing a skull base chordoma is not always easy, as it can be confused with other types of clival tumors. […] Once you have been given a diagnosis, it can be helpful to get a second opinion to confirm the diagnosis before making treatment decisions. […] Biopsy and histologic studies are necessary to confirm the diagnosis, and distinguish chordomas from other lesions. […] However, in experienced hands, an MRI is usually sufficient to suspect the diagnosis of chordoma, obviating the need for undergoing a dedicated procedure for biopsy and proceeding directly with biopsy and surgical resection at the same time. […] It is important to go to a hospital, treatment center, or network of treatment centers where doctors have experience diagnosing and treating chordoma as soon as chordoma is suspected.

• #9 Chordoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/chordoma?lang=us

  Chordomas are uncommon malignant tumors of the axial skeleton that account for 1% of intracranial tumors and 4% of all primary bone tumors. […] MRI and CT scan have complementary roles in tumor evaluation. CT evaluation is needed to assess the degree of bone involvement and to detect patterns of calcification within the lesion. MRI provides excellent anatomical delineation of adjacent structures and is able to characterize the signal of the lesion usually allowing for a confident preoperative diagnosis. MRI is, however, limited in its ability to evaluate calcification and the precise involvement of skull base osteolysis less well than CT, especially for skull base foramina. […] Traditionally, surgical resection has been the first line of treatment when feasible, with radiotherapy offered for recurrent cases. Some advocate the combination of radiation therapy and complete or subtotal surgical resection for select patients.

• #10 Understanding chordoma, a bone tumor in the skull base, sacrum or spine | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/understanding-chordoma-bone-cancer-skull-base-tumor-spine-sacrum.h00-159149190.html

  How is chordoma diagnosed? […] An MRI scan can help rule out other tumor types, but a tissue sample is needed for a definitive chordoma diagnosis. […] Most skull base chordomas grow in a bone called the clivus. This area is difficult to access for biopsy, so a skull base chordoma diagnosis cannot be confirmed until after your neurosurgeon accesses the tumor during surgery, which is also the first step for treatment.

• #11 Chordoma: A Rare Bone Cancer of the Skull Base and Spine | Pacific Neuroscience Institute

  https://www.pacificneuroscienceinstitute.org/blog/brain-tumor/chordoma-a-rare-bone-cancer-of-the-skull-base-and-spine/

  Chordomas are initially diagnosed by cranial imaging, most commonly, computed tomography (CT) scan, and magnetic resonance imaging (MRI). […] The gold standard of imaging is an MRI with IV contrast which uses gadolinium, with thin cuts in the region of the clivus, often called a pituitary protocol. […] Given that there are numerous types of tumors that can occur in this region with widely varying treatment options, an initial biopsy is almost always required to establish a pathological diagnosis. This is often performed by the ENT surgeon during an office or outpatient procedure. The pathological diagnosis of chordoma is usually unambiguous and is necessary to guide the definitive treatment of the patient.

• #12 Chordoma | AdventHealth Neuroscience Institute

  https://www.adventhealthneuroinstitute.com/programs/brain-tumors/chordoma

  When you or a loved one face a chordoma diagnosis, the AdventHealth Neuroscience Institute team has the experience and compassion you and your family need to help you navigate your journey. […] If a chordoma is suspected, your neurosurgeon will use specific tests and procedures to rule out or reach a chordoma diagnosis. […] During a biopsy, a hollow needle is inserted into your skin to collect a small sample of cells at and around the suspected tumor. This cell sample is then sent to a laboratory where pathologists look under a microscope for cancer cells. […] Through imaging tests, such as magnetic resonance imaging (MRI) or computed tomography (CT) scans, your doctor can identify the size and location of the suspected tumor. The images can also help guide a biopsy, if needed.

• #13 Chordoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/chordoma?lang=us

  Chordomas are uncommon malignant tumors of the axial skeleton that account for 1% of intracranial tumors and 4% of all primary bone tumors. […] MRI and CT scan have complementary roles in tumor evaluation. CT evaluation is needed to assess the degree of bone involvement and to detect patterns of calcification within the lesion. MRI provides excellent anatomical delineation of adjacent structures and is able to characterize the signal of the lesion usually allowing for a confident preoperative diagnosis. MRI is, however, limited in its ability to evaluate calcification and the precise involvement of skull base osteolysis less well than CT, especially for skull base foramina. […] Traditionally, surgical resection has been the first line of treatment when feasible, with radiotherapy offered for recurrent cases. Some advocate the combination of radiation therapy and complete or subtotal surgical resection for select patients.

• #14 Chordoma at the skull base, spine, and sacrum: A pictorial essay – Journal of Clinical Imaging Science

  https://clinicalimagingscience.org/chordoma-at-the-skull-base-spine-and-sacrum-a-pictorial-essay/

  Chordomas are rare tumors believed to be arising from the notochord remnant in the axial skeleton. Diagnosis is often difficult since they show overlapping imaging features with other more common disease including metastases. […] Diagnosis is often difficult since there are overlapping imaging features with other differential diagnoses including chondrosarcoma and metastasis. Biopsy may often be required for a definitive diagnosis. […] The hallmark of diagnosing chordomas is physaliphorous cells, which have abundant eosinophilic cytoplasm and intracytoplasmic vacuoles. […] Multiple immunohistochemical markers have been proposed such as cytokeratin, EMA, S100 and Brachyury. However, Brachyury becomes the principal marker for diagnosis since it is present in almost all chordomas but not found in chondrosarcomas. […] Biopsy and multidisciplinary involvement are often required for definitive diagnosis and disease management.

• #15

  https://www.seyitaligumustas.com/en/chordoma

  Chordoma is a malignant and slowly progressive tumor that develops from the notochord remnants of the bone. […] Because of the slow progression of chordoma and vague symptoms, there may be a delay in diagnosis. […] Radiologic evaluation of patients with suspected chordoma begins with plain radiography. […] After clinical and radiologic evaluation of the patient with suspected chordoma, the diagnosis is made by biopsy. […] The patient diagnosed with chordoma should undergo a CT scan of the lungs for staging. […] Patients should be closely monitored because of the high risk of postoperative recurrence of chordoma and the possibility of metastasis to the lung.

• #16 Chordoma Diagnosis & Treatment – NYC | Columbia Neurosurgery in New York City

  https://www.neurosurgery.columbia.edu/patient-care/conditions/chordoma

  A chordoma is a tumor that develops in the bones of the skull or spine. […] At the Spine Hospital at the Neurological Institute of New York, we specialize in chordomas of the spine. […] As with all spine tumors, imaging studies are essential in diagnosing chordomas. Because these tumors often involve bony structures, computed tomography (CT) scans are used to assess the degree of bony involvement. Magnetic resonance (MR) scans also are used to distinguish the tumor from the sensitive structures around it. For both CTs and MRs, an agent like a dye may be administered intravenously so that neurosurgeons can better visualize the tumor against the normal tissue in the background. […] Spinal angiography, which reveals arteries and vascular structures around the tumor, may also be used. This imaging study can help direct the surgical approach to the tumor by showing which blood vessels it affects.

• #17 Chordoma Workup: Imaging Studies, Diagnostic Procedures, Histologic Findings

  https://emedicine.medscape.com/article/250902-workup

  Chordomas show notochordal differentiation and are characterized by nuclear expression of brachyury (TBXT). […] The inherent FDG avidity of chordomas suggests that 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) may be a useful modality for staging, evaluating treatment response, and assessing recurrent or metastatic disease. […] Age at diagnosis, tumor size, and disease stage can influence conditional survival for patients with chordoma. […] The role of MIB-1 immunohistochemical staining (a proliferation marker) as a prognostic indicator in chordoma is controversial, but data suggest that an increased MIB-1 labeling index correlates with recurrence.

• #18

  https://tumorsurgery.org/tumor-education/bone-tumors/types-of-bone-tumors/chordoma.aspx

  Chordomas expand and destroy bone May erode clivus, sella turca, petrous, and sphenoid bones Lesions can be overlooked on plain x-rays, and CT scans and MRI often do not extend below S2 Commonly extends into soft tissue. […] Locally aggressive tumor with high rate of local recurrence Due to its location on axial skeleton, vital structures may be affected causing death Local recurrences are common. Local recurrence is the most important predictor of mortality Extensive resection may be the most determining factor in affording an opportunity to cure. […] Wide en-bloc surgical resection. Wider surgical margins improve the local recurrence rate. Notoriously recur even with a wide en bloc resection Enbloc surgical resection may be associated with a high degree of morbidity Extensive curettage and cryosurgery may be acceptable Total en-bloc resection is suitable in 50% of the cases. Radiotherapy still in debate when is used in conjunction with surgery.

• #19 Symptoms and diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/diagnosis/

  Chordoma tumors are typically detected through imaging tests, which show organs and other structures inside the body, including tumors. […] When a chordoma is suspected, you will need magnetic resonance imaging, also called MRI, to help doctors make a diagnosis and plan for treatment. […] Imaging tests can show the possibility of a chordoma, but a definitive diagnosis can only be made by a pathologist who examines a sample of tumor tissue under a microscope. […] Tissue samples should be evaluated by a pathologist who has experience diagnosing bone tumors. […] Your pathologist may test your tumor tissue for the presence of a protein called brachyury (also known as TBXT). Nearly all chordomas have high levels of brachyury, which makes it helpful for diagnosis. […] If tissue is not available for testing, the possibility of a biopsy to obtain tissue for this testing should be discussed with a doctor who has experience treating chordoma, carefully weighing the potential risks of biopsy. […] Interpretation of the testing should be done by a pathologist who has experience diagnosing chordoma.

• #20 New diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/treatment-guidelines/new-diagnosis/

  If chordoma is suspected or has been diagnosed, the most important thing to do is find a medical center with expertise in treating chordoma patients. […] If you’ve been told you might have chordoma, it is important to be evaluated by a multidisciplinary team of specialists who have substantial experience treating chordoma, even before you know for sure if you have it. […] Chordoma tumors are typically detected through imaging tests, which show organs and other structures inside the body, including tumors. The way the tumor looks on imaging tests can tell a radiologist whether the tumor might be chordoma. […] When a chordoma is suspected, you will need magnetic resonance imaging, also called MRI, to help doctors make a diagnosis and plan for treatment. […] While imaging studies can show the possibility of a chordoma, a definitive diagnosis can only be made by a pathologist who examines a sample of tumor tissue under a microscope.

• #21 Chordoma Workup: Imaging Studies, Diagnostic Procedures, Histologic Findings

  https://emedicine.medscape.com/article/250902-workup

  Evaluation of chordoma revolves around imaging and biopsy. […] No laboratory studies are required for evaluation of chordoma, except as needed for routine preoperative evaluation for patients scheduled to undergo surgical resection. […] Imaging of the clivus usually reveals features adequate for differentiating chordomas from other site-specific lesions. […] Biopsies of chordomas are useful only when other bone lesions remain in the differential diagnosis after imaging studies are performed. […] Fine-needle aspiration (FNA), the preferred method for establishing the preoperative morphologic diagnosis of chordoma, has been reported to lower local recurrence rates when compared with open biopsy. […] Many times, a needle or open biopsy is performed to confirm the diagnosis of chordoma.

• #22 Diagnosis and Treatment of Chordoma in: Journal of the National Comprehensive Cancer Network Volume 11 Issue 6 (2013)

  https://jnccn.org/view/journals/jnccn/11/6/article-p726.xml

  Chordoma is a primary bone cancer arising on the midline from the skull base to the sacrum. Diagnosis is often delayed because of insidious onset and nonspecific symptoms. […] Chordomas are typically diagnosed at a locally advanced stage due to insidious onset and nonspecific symptoms. […] Fine-needle aspiration or core needle biopsy is the preferred method of diagnosis before surgical resection. Tumor seeding is a significant concern after biopsy, thus the tract of the biopsy needle should be marked and excised with the tumor. […] Previously, chordomas were diagnosed by their histopathologic features and immunoreactivity for S-100 and epithelial markers (eg, cytokeratins). […] Brachyury has emerged as an integral distinguishing biomarker for chordoma from other chondroid lesions. […] Histologic characteristics predictive of recurrence include number of mitotic figures and Ki-67 labeling index greater than 6%.

• #23 Chordoma | AdventHealth Neuroscience Institute

  https://www.adventhealthneuroinstitute.com/programs/brain-tumors/chordoma

  When you or a loved one face a chordoma diagnosis, the AdventHealth Neuroscience Institute team has the experience and compassion you and your family need to help you navigate your journey. […] If a chordoma is suspected, your neurosurgeon will use specific tests and procedures to rule out or reach a chordoma diagnosis. […] During a biopsy, a hollow needle is inserted into your skin to collect a small sample of cells at and around the suspected tumor. This cell sample is then sent to a laboratory where pathologists look under a microscope for cancer cells. […] Through imaging tests, such as magnetic resonance imaging (MRI) or computed tomography (CT) scans, your doctor can identify the size and location of the suspected tumor. The images can also help guide a biopsy, if needed.

• #24 Chordoma Workup: Imaging Studies, Diagnostic Procedures, Histologic Findings

  https://emedicine.medscape.com/article/250902-workup

  Evaluation of chordoma revolves around imaging and biopsy. […] No laboratory studies are required for evaluation of chordoma, except as needed for routine preoperative evaluation for patients scheduled to undergo surgical resection. […] Imaging of the clivus usually reveals features adequate for differentiating chordomas from other site-specific lesions. […] Biopsies of chordomas are useful only when other bone lesions remain in the differential diagnosis after imaging studies are performed. […] Fine-needle aspiration (FNA), the preferred method for establishing the preoperative morphologic diagnosis of chordoma, has been reported to lower local recurrence rates when compared with open biopsy. […] Many times, a needle or open biopsy is performed to confirm the diagnosis of chordoma.

• #25 Chordoma spinal cancer diagnosis and treatment | Chordoma UK

  https://chordoma-uk.org/diagnosis-and-treatment

  Skull base tumors can be difficult to reach safely for a biopsy therefore a surgeon may opt for a biopsy to be taken during surgery. This means that a pathologist will be prepared to examine a sample of tumor tissue removed at the start of surgery and give an immediate diagnosis, and the surgical team will proceed with surgery based on that information.

• #26 Chordoma | Brain Tumor Center | Stanford Medicine

  https://med.stanford.edu/brain-tumor/conditions/chordoma.html

  You should avoid having a biopsy or surgery to confirm the diagnosis of chordoma outside of a referral center because, if not done properly, these procedures can cause the chordoma to spread. […] If you cannot travel to see a doctor who has experience with chordoma, have your imaging tests sent to a doctor with chordoma experience for a second opinion before you get treatment or a biopsy.

• #27 Symptoms and diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/diagnosis/

  Chordoma is not always easy to diagnose and it can be confused with other types of tumors. Getting the correct diagnosis from the start is critical to being able to find and choose the best treatment. […] Any biopsy or surgery to confirm the diagnosis of chordoma should be done at a center with expertise in chordoma treatment. If not done properly, these procedures can cause the chordoma to spread. […] If you have had an MRI and been given a probable or differential diagnosis of chordoma, it is very important to find doctors who have experience diagnosing and treating chordoma patients to confirm the diagnosis. […] Once you have been given a diagnosis, it can be helpful to get a second opinion to confirm the diagnosis before making treatment decisions. […] It is important to go to a hospital, treatment center, or network of treatment centers where doctors have experience diagnosing and treating chordoma as soon as chordoma is suspected.

• #28 Diagnosis and Treatment of Chordoma in: Journal of the National Comprehensive Cancer Network Volume 11 Issue 6 (2013)

  https://jnccn.org/view/journals/jnccn/11/6/article-p726.xml

  Chordoma is a primary bone cancer arising on the midline from the skull base to the sacrum. Diagnosis is often delayed because of insidious onset and nonspecific symptoms. […] Chordomas are typically diagnosed at a locally advanced stage due to insidious onset and nonspecific symptoms. […] Fine-needle aspiration or core needle biopsy is the preferred method of diagnosis before surgical resection. Tumor seeding is a significant concern after biopsy, thus the tract of the biopsy needle should be marked and excised with the tumor. […] Previously, chordomas were diagnosed by their histopathologic features and immunoreactivity for S-100 and epithelial markers (eg, cytokeratins). […] Brachyury has emerged as an integral distinguishing biomarker for chordoma from other chondroid lesions. […] Histologic characteristics predictive of recurrence include number of mitotic figures and Ki-67 labeling index greater than 6%.

• #29 Chordoma spinal cancer diagnosis and treatment | Chordoma UK

  https://chordoma-uk.org/diagnosis-and-treatment

  Skull base tumors can be difficult to reach safely for a biopsy therefore a surgeon may opt for a biopsy to be taken during surgery. This means that a pathologist will be prepared to examine a sample of tumor tissue removed at the start of surgery and give an immediate diagnosis, and the surgical team will proceed with surgery based on that information.

• #30 Symptoms and diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/diagnosis/

  Chordoma tumors are typically detected through imaging tests, which show organs and other structures inside the body, including tumors. […] When a chordoma is suspected, you will need magnetic resonance imaging, also called MRI, to help doctors make a diagnosis and plan for treatment. […] Imaging tests can show the possibility of a chordoma, but a definitive diagnosis can only be made by a pathologist who examines a sample of tumor tissue under a microscope. […] Tissue samples should be evaluated by a pathologist who has experience diagnosing bone tumors. […] Your pathologist may test your tumor tissue for the presence of a protein called brachyury (also known as TBXT). Nearly all chordomas have high levels of brachyury, which makes it helpful for diagnosis. […] If tissue is not available for testing, the possibility of a biopsy to obtain tissue for this testing should be discussed with a doctor who has experience treating chordoma, carefully weighing the potential risks of biopsy. […] Interpretation of the testing should be done by a pathologist who has experience diagnosing chordoma.

• #31 New diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/treatment-guidelines/new-diagnosis/

  Tissue samples should be evaluated by a pathologist who has experience diagnosing bone tumors. Your pathologist should test your tumor tissue for the presence of a protein called brachyury (also known as TBXT). Nearly all chordomas have high levels of brachyury, which makes it helpful for diagnosis.

• #32 Chordoma – Wikipedia

  https://en.wikipedia.org/wiki/Chordoma

  In 2015 the first consensus guidelines for the diagnosis and treatment of chordoma were published in The Lancet Oncology. These tumors express brachyury and cytokeratin, which can be detected by immunohistochemistry. […] There are three histological variants of chordoma: conventional, chondroid and dedifferentiated. […] The histological appearance of classical chordoma is of a lobulated tumor composed of groups of cells separated by fibrous septa. The cells have small round nuclei and abundant vacuolated cytoplasm, sometimes described as „physaliferous” because of their cytoplasmic vacuoles, and their resemblance to the physalis plant. Chondroid chordomas histologically show features of both chordoma and chondrosarcoma.

• #33 Azthena logo with the word Azthena

  https://www.news-medical.net/health/What-is-Chordoma.aspx

  Chordoma is most usually diagnosed between the ages of 50 and 60, and it affects men more than women. […] The classical (conventional) appearance of „sheets and cords of round to polygonal” tumor cells filled with occasionally large mucin-filled eosinophilic cytoplasm called physaliphorous cells confirms the diagnosis of chordoma after surgical resection or first biopsy. […] Unfortunately, due to chordoma’s slow growth and the insidious, non-specific symptoms it causes, identification might take months or even years. […] When possible, surgery is the backbone of treatment for primary and/or recurrent chordoma, and the outcome of surgery is directly tied to the patient’s projected outcome.

• #34 Chordoma – Wikipedia

  https://en.wikipedia.org/wiki/Chordoma

  In 2015 the first consensus guidelines for the diagnosis and treatment of chordoma were published in The Lancet Oncology. These tumors express brachyury and cytokeratin, which can be detected by immunohistochemistry. […] There are three histological variants of chordoma: conventional, chondroid and dedifferentiated. […] The histological appearance of classical chordoma is of a lobulated tumor composed of groups of cells separated by fibrous septa. The cells have small round nuclei and abundant vacuolated cytoplasm, sometimes described as „physaliferous” because of their cytoplasmic vacuoles, and their resemblance to the physalis plant. Chondroid chordomas histologically show features of both chordoma and chondrosarcoma.

• #35

  https://www.massgeneral.org/orthopaedics/oncology/conditions-and-treatments/chordoma

  There are four subtypes of chordoma: Conventional (or classic) chordoma is the most common form. […] Poorly differentiated chordoma can be more aggressive and faster growing than conventional chordoma. […] Dedifferentiated chordoma is the most aggressive and fastest growing chordoma. […] Chondroid chordoma is a term used in the past when it was difficult for pathologists to distinguish conventional chordoma from chondrosarcoma (malignant bone tumor of cartilage tissue). […] Once the diagnosis is confirmed and all scans are reviewed, you will have a lengthy visit with your chordoma team to discuss all treatment options.

• #36 Final Diagnosis — Case 689

  https://path.upmc.edu/cases/case689/dx.html

  For these reasons, formulation of an appropriate immunohistochemical panel is important. […] Chordomas are typically immunoreactive for S100, keratins, and epithelial membrane antigen (EMA). […] Chordomas are not typically immunoreactive for CK7, CK20, GFAP, D2-40, MelanA, HMB45, Desmin, CD34 or smooth muscle actin. Staining for Ki67 reveals a low proliferative index. […] The mass was subsequently excised. Histologic sections showed similar morphology, including a myxoid stroma, large cells arranged in cell cords and lobules, 'physaliferous cells,’ rare mitotic activity and no necrosis. […] For sacrococcygeal chordomas, surgical resection is the treatment of choice.

• #37 Chordoma – Wikipedia

  https://en.wikipedia.org/wiki/Chordoma

  In 2015 the first consensus guidelines for the diagnosis and treatment of chordoma were published in The Lancet Oncology. These tumors express brachyury and cytokeratin, which can be detected by immunohistochemistry. […] There are three histological variants of chordoma: conventional, chondroid and dedifferentiated. […] The histological appearance of classical chordoma is of a lobulated tumor composed of groups of cells separated by fibrous septa. The cells have small round nuclei and abundant vacuolated cytoplasm, sometimes described as „physaliferous” because of their cytoplasmic vacuoles, and their resemblance to the physalis plant. Chondroid chordomas histologically show features of both chordoma and chondrosarcoma.

• #38 New diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/treatment-guidelines/new-diagnosis/

  Tissue samples should be evaluated by a pathologist who has experience diagnosing bone tumors. Your pathologist should test your tumor tissue for the presence of a protein called brachyury (also known as TBXT). Nearly all chordomas have high levels of brachyury, which makes it helpful for diagnosis.

• #39 Symptoms and diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/diagnosis/

  Chordoma tumors are typically detected through imaging tests, which show organs and other structures inside the body, including tumors. […] When a chordoma is suspected, you will need magnetic resonance imaging, also called MRI, to help doctors make a diagnosis and plan for treatment. […] Imaging tests can show the possibility of a chordoma, but a definitive diagnosis can only be made by a pathologist who examines a sample of tumor tissue under a microscope. […] Tissue samples should be evaluated by a pathologist who has experience diagnosing bone tumors. […] Your pathologist may test your tumor tissue for the presence of a protein called brachyury (also known as TBXT). Nearly all chordomas have high levels of brachyury, which makes it helpful for diagnosis. […] If tissue is not available for testing, the possibility of a biopsy to obtain tissue for this testing should be discussed with a doctor who has experience treating chordoma, carefully weighing the potential risks of biopsy. […] Interpretation of the testing should be done by a pathologist who has experience diagnosing chordoma.

• #40 Chordoma at the skull base, spine, and sacrum: A pictorial essay – Journal of Clinical Imaging Science

  https://clinicalimagingscience.org/chordoma-at-the-skull-base-spine-and-sacrum-a-pictorial-essay/

  Chordomas are rare tumors believed to be arising from the notochord remnant in the axial skeleton. Diagnosis is often difficult since they show overlapping imaging features with other more common disease including metastases. […] Diagnosis is often difficult since there are overlapping imaging features with other differential diagnoses including chondrosarcoma and metastasis. Biopsy may often be required for a definitive diagnosis. […] The hallmark of diagnosing chordomas is physaliphorous cells, which have abundant eosinophilic cytoplasm and intracytoplasmic vacuoles. […] Multiple immunohistochemical markers have been proposed such as cytokeratin, EMA, S100 and Brachyury. However, Brachyury becomes the principal marker for diagnosis since it is present in almost all chordomas but not found in chondrosarcomas. […] Biopsy and multidisciplinary involvement are often required for definitive diagnosis and disease management.

• #41 Final Diagnosis — Case 689

  https://path.upmc.edu/cases/case689/dx.html

  For these reasons, formulation of an appropriate immunohistochemical panel is important. […] Chordomas are typically immunoreactive for S100, keratins, and epithelial membrane antigen (EMA). […] Chordomas are not typically immunoreactive for CK7, CK20, GFAP, D2-40, MelanA, HMB45, Desmin, CD34 or smooth muscle actin. Staining for Ki67 reveals a low proliferative index. […] The mass was subsequently excised. Histologic sections showed similar morphology, including a myxoid stroma, large cells arranged in cell cords and lobules, 'physaliferous cells,’ rare mitotic activity and no necrosis. […] For sacrococcygeal chordomas, surgical resection is the treatment of choice.

• #42 Final Diagnosis — Case 689

  https://path.upmc.edu/cases/case689/dx.html

  For these reasons, formulation of an appropriate immunohistochemical panel is important. […] Chordomas are typically immunoreactive for S100, keratins, and epithelial membrane antigen (EMA). […] Chordomas are not typically immunoreactive for CK7, CK20, GFAP, D2-40, MelanA, HMB45, Desmin, CD34 or smooth muscle actin. Staining for Ki67 reveals a low proliferative index. […] The mass was subsequently excised. Histologic sections showed similar morphology, including a myxoid stroma, large cells arranged in cell cords and lobules, 'physaliferous cells,’ rare mitotic activity and no necrosis. […] For sacrococcygeal chordomas, surgical resection is the treatment of choice.

• #43 Final Diagnosis — Case 689

  https://path.upmc.edu/cases/case689/dx.html

  For these reasons, formulation of an appropriate immunohistochemical panel is important. […] Chordomas are typically immunoreactive for S100, keratins, and epithelial membrane antigen (EMA). […] Chordomas are not typically immunoreactive for CK7, CK20, GFAP, D2-40, MelanA, HMB45, Desmin, CD34 or smooth muscle actin. Staining for Ki67 reveals a low proliferative index. […] The mass was subsequently excised. Histologic sections showed similar morphology, including a myxoid stroma, large cells arranged in cell cords and lobules, 'physaliferous cells,’ rare mitotic activity and no necrosis. […] For sacrococcygeal chordomas, surgical resection is the treatment of choice.

• #44 Final Diagnosis — Case 689

  https://path.upmc.edu/cases/case689/dx.html

  For these reasons, formulation of an appropriate immunohistochemical panel is important. […] Chordomas are typically immunoreactive for S100, keratins, and epithelial membrane antigen (EMA). […] Chordomas are not typically immunoreactive for CK7, CK20, GFAP, D2-40, MelanA, HMB45, Desmin, CD34 or smooth muscle actin. Staining for Ki67 reveals a low proliferative index. […] The mass was subsequently excised. Histologic sections showed similar morphology, including a myxoid stroma, large cells arranged in cell cords and lobules, 'physaliferous cells,’ rare mitotic activity and no necrosis. […] For sacrococcygeal chordomas, surgical resection is the treatment of choice.

• #45 Symptoms and diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/diagnosis/

  Chordoma is not always easy to diagnose and it can be confused with other types of tumors. Getting the correct diagnosis from the start is critical to being able to find and choose the best treatment. […] Any biopsy or surgery to confirm the diagnosis of chordoma should be done at a center with expertise in chordoma treatment. If not done properly, these procedures can cause the chordoma to spread. […] If you have had an MRI and been given a probable or differential diagnosis of chordoma, it is very important to find doctors who have experience diagnosing and treating chordoma patients to confirm the diagnosis. […] Once you have been given a diagnosis, it can be helpful to get a second opinion to confirm the diagnosis before making treatment decisions. […] It is important to go to a hospital, treatment center, or network of treatment centers where doctors have experience diagnosing and treating chordoma as soon as chordoma is suspected.

• #46 Final Diagnosis — Case 312

  https://path.upmc.edu/cases/case312/dx.html

  FINAL DIAGNOSIS: CHORDOMA […] Chordoma comprises approximately 3 to 4% of primary malignant bone tumors (although some authors feel this should be 1%), and is the 4th most common primary malignancy in bone after osteosarcoma, chondrosarcoma and Ewing’s sarcoma. […] The average age at the time of diagnosis for sacrococcygeal chordoma is 48 years and for cranial lesions is 38 years. […] The definitive treatment for chordoma is wide, intracompartmental excision. […] The average interval between the onset of symptoms and establishment of the correct diagnosis is 12 months with a range of one month to 5 years. […] The differential diagnosis of chordoma on imaging includes metastatic carcinoma, myeloma, giant cell tumor, neurogenic tumors, aneurysmal bone cyst, chondrosarcoma, and tuberculosis of the spine.

• #47 Chordoma at the skull base, spine, and sacrum: A pictorial essay – Journal of Clinical Imaging Science

  https://clinicalimagingscience.org/chordoma-at-the-skull-base-spine-and-sacrum-a-pictorial-essay/

  Chordomas are rare tumors believed to be arising from the notochord remnant in the axial skeleton. Diagnosis is often difficult since they show overlapping imaging features with other more common disease including metastases. […] Diagnosis is often difficult since there are overlapping imaging features with other differential diagnoses including chondrosarcoma and metastasis. Biopsy may often be required for a definitive diagnosis. […] The hallmark of diagnosing chordomas is physaliphorous cells, which have abundant eosinophilic cytoplasm and intracytoplasmic vacuoles. […] Multiple immunohistochemical markers have been proposed such as cytokeratin, EMA, S100 and Brachyury. However, Brachyury becomes the principal marker for diagnosis since it is present in almost all chordomas but not found in chondrosarcomas. […] Biopsy and multidisciplinary involvement are often required for definitive diagnosis and disease management.

• #48 Chordoma – Symptoms, Support and Treatments – UC Health

  https://www.uchealth.com/en/conditions/chordoma

  Methods typically used to diagnose cancer will be used to identify a skull base chordoma. These may include different scans, such as X-rays, CT scans, or MRI. You may also have a blood test or a biopsy. Testing may be done by a specialist, such as a neurologist or a neurosurgeon. […] It is common for a chordoma to be confused with (misdiagnosed as) another type of bone tumor called a chondrosarcoma. Both types of tumors can occur in similar places in the body. These tumors also look similar. Chondrosarcomas tend to respond better to radiation and have a better outlook.

• #49 Chordoma – Symptoms, Support and Treatments – UC Health

  https://www.uchealth.com/en/conditions/chordoma

  Methods typically used to diagnose cancer will be used to identify a skull base chordoma. These may include different scans, such as X-rays, CT scans, or MRI. You may also have a blood test or a biopsy. Testing may be done by a specialist, such as a neurologist or a neurosurgeon. […] It is common for a chordoma to be confused with (misdiagnosed as) another type of bone tumor called a chondrosarcoma. Both types of tumors can occur in similar places in the body. These tumors also look similar. Chondrosarcomas tend to respond better to radiation and have a better outlook.

• #50 Chordoma: What It Is, Types, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17916-chordoma

  Chordomas are rare. They affect about 1 person for every 1 million people per year. That means that about 300 people receive a chordoma diagnosis each year in the United States. […] The only way healthcare providers can definitively diagnose chordoma is with a biopsy usually a needle biopsy. This involves taking a small sample of the tumor so a specialist can examine it under a microscope. […] Your healthcare provider will ask about your symptoms and medical history. Theyll likely perform a physical exam and a neurological exam. […] If they suspect a tumor, theyll order an imaging test, such as an X-ray, computed tomography (CT) scan or MRI scan. […] Your provider will likely refer you to a bone cancer specialist for a second opinion and confirmation of the diagnosis. You may need additional imaging tests to better determine the location of the chordoma and see if its spread to other areas of your body.

• #51 Diagnosing and Treating a Chordoma | Neurological Surgery

  https://neurosurgery.weillcornell.org/condition/chordoma/diagnosing-and-treating-chordoma

  A person suspected of having a chordoma will have an initial examination that includes a detailed medical history, blood and urine tests, and a basic neurological examination to check for alertness, coordination, eye movement, muscle strength, and reflexes. […] It is vital that an individual with a chordoma seek care from a highly trained, experienced multidisciplinary team, including neurosurgeons and neuro-oncologists at a major medical center where the physicians have proven skills in diagnosis and treatment. […] An accurate diagnosis will include extensive testing and expertise. […] One of the tools used to diagnose a chordoma is an MRI scan, which uses magnetic fields and radio-frequency waves to create an image that can show the presence of a tumor and some of its characteristics, such as its size, precise location, and whether it has spread.

• #52 Chordoma Treatment

  https://www.rwjbh.org/treatment-care/neuroscience/neurosurgery/conditions-treated/chordoma/

  After discussing a patients medical history, performing a general physical, and conducting a neurological exam, your physician may suspect a spinal tumor and refer you to a neurologist, who is an expert in the field of brain and spinal tumors. […] A chordoma cannot be diagnosed until a biopsy has been performed by a neuropathologist. Before this can happen, your doctor must first detect and locate a spinal tumor. During an exam, sometimes, a lump can be found at the base of the spine. In other cases, imaging tests need to be performed to locate a tumor; these include computed tomography (CT) scans and magnetic resonance imaging (MRI) scans. These scans can also help a neurologist or neurosurgeon develop treatment strategies. […] The following tests can confirm diagnosis of a chordoma and pinpoint the location of the tumor: Physical exam: A physician may be able to feel a large soft tissue mass in the middle of the sacral area of the spine, or during a rectal examination. Neurological exam: Patients will be examined for loss of sacral nerve sensation, such as lower leg weakness. Your doctor will ask if you have back pain, loss of bowel and/or bladder control, loss of sexual function (from the tumors involvement with the nerves of the sacral area of the spine), and more. Spinal magnetic imaging scan (MRI): These tests are the preferred method for diagnosing tumors of the spinal cord. MRI technology works by using radio waves to produce images of the spinal column, spinal cord, and nerves. Computerized tomography scan (CT): By using a narrow beam of radiation to produce detailed images of the spine, your neurologist can spot any abnormal change in the spinal canal. However, this test is rarely used to diagnose chordomas or other spinal tumors. Biopsy: Once a tumor has been located, the only way to determine its exact type is by taking a small tissue sample and examining it under a microscope. By identifying the tumors type and histologic grade, a treatment plan can be developed.

• #53 Chordoma Workup: Imaging Studies, Diagnostic Procedures, Histologic Findings

  https://emedicine.medscape.com/article/250902-workup

  Evaluation of chordoma revolves around imaging and biopsy. […] No laboratory studies are required for evaluation of chordoma, except as needed for routine preoperative evaluation for patients scheduled to undergo surgical resection. […] Imaging of the clivus usually reveals features adequate for differentiating chordomas from other site-specific lesions. […] Biopsies of chordomas are useful only when other bone lesions remain in the differential diagnosis after imaging studies are performed. […] Fine-needle aspiration (FNA), the preferred method for establishing the preoperative morphologic diagnosis of chordoma, has been reported to lower local recurrence rates when compared with open biopsy. […] Many times, a needle or open biopsy is performed to confirm the diagnosis of chordoma.

• #54 Diagnosing and Treating a Chordoma | Neurological Surgery

  https://neurosurgery.weillcornell.org/condition/chordoma/diagnosing-and-treating-chordoma

  A person suspected of having a chordoma will have an initial examination that includes a detailed medical history, blood and urine tests, and a basic neurological examination to check for alertness, coordination, eye movement, muscle strength, and reflexes. […] It is vital that an individual with a chordoma seek care from a highly trained, experienced multidisciplinary team, including neurosurgeons and neuro-oncologists at a major medical center where the physicians have proven skills in diagnosis and treatment. […] An accurate diagnosis will include extensive testing and expertise. […] One of the tools used to diagnose a chordoma is an MRI scan, which uses magnetic fields and radio-frequency waves to create an image that can show the presence of a tumor and some of its characteristics, such as its size, precise location, and whether it has spread.

• #55 Chordoma Treatment

  https://www.rwjbh.org/treatment-care/neuroscience/neurosurgery/conditions-treated/chordoma/

  After discussing a patients medical history, performing a general physical, and conducting a neurological exam, your physician may suspect a spinal tumor and refer you to a neurologist, who is an expert in the field of brain and spinal tumors. […] A chordoma cannot be diagnosed until a biopsy has been performed by a neuropathologist. Before this can happen, your doctor must first detect and locate a spinal tumor. During an exam, sometimes, a lump can be found at the base of the spine. In other cases, imaging tests need to be performed to locate a tumor; these include computed tomography (CT) scans and magnetic resonance imaging (MRI) scans. These scans can also help a neurologist or neurosurgeon develop treatment strategies. […] The following tests can confirm diagnosis of a chordoma and pinpoint the location of the tumor: Physical exam: A physician may be able to feel a large soft tissue mass in the middle of the sacral area of the spine, or during a rectal examination. Neurological exam: Patients will be examined for loss of sacral nerve sensation, such as lower leg weakness. Your doctor will ask if you have back pain, loss of bowel and/or bladder control, loss of sexual function (from the tumors involvement with the nerves of the sacral area of the spine), and more. Spinal magnetic imaging scan (MRI): These tests are the preferred method for diagnosing tumors of the spinal cord. MRI technology works by using radio waves to produce images of the spinal column, spinal cord, and nerves. Computerized tomography scan (CT): By using a narrow beam of radiation to produce detailed images of the spine, your neurologist can spot any abnormal change in the spinal canal. However, this test is rarely used to diagnose chordomas or other spinal tumors. Biopsy: Once a tumor has been located, the only way to determine its exact type is by taking a small tissue sample and examining it under a microscope. By identifying the tumors type and histologic grade, a treatment plan can be developed.

• #56 Diagnosing and Treating a Chordoma | Neurological Surgery

  https://neurosurgery.weillcornell.org/condition/chordoma/diagnosing-and-treating-chordoma

  If a surgical team decides to perform a needle biopsy of the tumor, they may be prepared to also immediately remove the chordoma once the diagnosis is confirmed by a pathologist. […] Reaching an accurate diagnosis is critical to ensure optimal outcome and require unique, specific tests. […] Tests we offer include: Dynamic hormone testing, High-resolution pituitary MRI, Office endonasal endoscopy, Visual field and OCT testing, CT angiogram.

• #57 Chordoma Treatment

  https://www.rwjbh.org/treatment-care/neuroscience/neurosurgery/conditions-treated/chordoma/

  After discussing a patients medical history, performing a general physical, and conducting a neurological exam, your physician may suspect a spinal tumor and refer you to a neurologist, who is an expert in the field of brain and spinal tumors. […] A chordoma cannot be diagnosed until a biopsy has been performed by a neuropathologist. Before this can happen, your doctor must first detect and locate a spinal tumor. During an exam, sometimes, a lump can be found at the base of the spine. In other cases, imaging tests need to be performed to locate a tumor; these include computed tomography (CT) scans and magnetic resonance imaging (MRI) scans. These scans can also help a neurologist or neurosurgeon develop treatment strategies. […] The following tests can confirm diagnosis of a chordoma and pinpoint the location of the tumor: Physical exam: A physician may be able to feel a large soft tissue mass in the middle of the sacral area of the spine, or during a rectal examination. Neurological exam: Patients will be examined for loss of sacral nerve sensation, such as lower leg weakness. Your doctor will ask if you have back pain, loss of bowel and/or bladder control, loss of sexual function (from the tumors involvement with the nerves of the sacral area of the spine), and more. Spinal magnetic imaging scan (MRI): These tests are the preferred method for diagnosing tumors of the spinal cord. MRI technology works by using radio waves to produce images of the spinal column, spinal cord, and nerves. Computerized tomography scan (CT): By using a narrow beam of radiation to produce detailed images of the spine, your neurologist can spot any abnormal change in the spinal canal. However, this test is rarely used to diagnose chordomas or other spinal tumors. Biopsy: Once a tumor has been located, the only way to determine its exact type is by taking a small tissue sample and examining it under a microscope. By identifying the tumors type and histologic grade, a treatment plan can be developed.

• #58 Chordoma: Symptoms, Treatment & Diagnosis | Pacific Brain Tumor Center

  https://www.pacificneuroscienceinstitute.org/brain-tumor/conditions/chordoma/

  These skull base tumors are best diagnosed by MRI and CT scans which will clearly show the extent of tumor and bony destruction. […] Focused MRIs of the pituitary region, sinuses, temporal bones or internal auditory canals may be indicated to obtain better anatomical detail of a chordoma. […] Other tests may also be needed prior to surgery such as angiography (typically now performed as a CT angiogram or an MR angiogram), visual field tests, an audiogram or pituitary hormonal tests.

• #59 Chordoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430846/

  Most physicians advocate for radiation therapy after any type of chordoma resection due to the high local recurrence rate. […] Due to the high local recurrence of chordomas, most physicians recommend lifelong surveillance with magnetic resonance imaging (MRI) with and without gadolinium contrast. […] It should be stressed that if chordoma is entertained in the differential diagnosis, there should be careful planning of any biopsy as the biopsy tract needs to be included in the ultimate resection of the chordoma to decrease the risk of local recurrence. […] Five-year survival is approximately 50% overall but improved with complete resection with negative margins to a 65% 5-year survival rate. […] Chordomas can be divided into four subtypes: conventional, poorly differentiated, dedifferentiated, and chondroid. […] The management of chordoma is best done with an interprofessional team that includes an orthopedic surgeon, radiologist, neurologist, and pathologist.

• #60 Diagnosing and Treating a Chordoma | Neurological Surgery

  https://neurosurgery.weillcornell.org/condition/chordoma/diagnosing-and-treating-chordoma

  A person suspected of having a chordoma will have an initial examination that includes a detailed medical history, blood and urine tests, and a basic neurological examination to check for alertness, coordination, eye movement, muscle strength, and reflexes. […] It is vital that an individual with a chordoma seek care from a highly trained, experienced multidisciplinary team, including neurosurgeons and neuro-oncologists at a major medical center where the physicians have proven skills in diagnosis and treatment. […] An accurate diagnosis will include extensive testing and expertise. […] One of the tools used to diagnose a chordoma is an MRI scan, which uses magnetic fields and radio-frequency waves to create an image that can show the presence of a tumor and some of its characteristics, such as its size, precise location, and whether it has spread.

• #61 Symptoms and diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/diagnosis/

  Chordoma is not always easy to diagnose and it can be confused with other types of tumors. Getting the correct diagnosis from the start is critical to being able to find and choose the best treatment. […] Any biopsy or surgery to confirm the diagnosis of chordoma should be done at a center with expertise in chordoma treatment. If not done properly, these procedures can cause the chordoma to spread. […] If you have had an MRI and been given a probable or differential diagnosis of chordoma, it is very important to find doctors who have experience diagnosing and treating chordoma patients to confirm the diagnosis. […] Once you have been given a diagnosis, it can be helpful to get a second opinion to confirm the diagnosis before making treatment decisions. […] It is important to go to a hospital, treatment center, or network of treatment centers where doctors have experience diagnosing and treating chordoma as soon as chordoma is suspected.

• #62 New diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/treatment-guidelines/new-diagnosis/

  If chordoma is suspected or has been diagnosed, the most important thing to do is find a medical center with expertise in treating chordoma patients. […] If you’ve been told you might have chordoma, it is important to be evaluated by a multidisciplinary team of specialists who have substantial experience treating chordoma, even before you know for sure if you have it. […] Chordoma tumors are typically detected through imaging tests, which show organs and other structures inside the body, including tumors. The way the tumor looks on imaging tests can tell a radiologist whether the tumor might be chordoma. […] When a chordoma is suspected, you will need magnetic resonance imaging, also called MRI, to help doctors make a diagnosis and plan for treatment. […] While imaging studies can show the possibility of a chordoma, a definitive diagnosis can only be made by a pathologist who examines a sample of tumor tissue under a microscope.

• #63 Chordoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/chordoma/diagnosis-treatment/drc-20580273

  To diagnose chordoma, a healthcare professional might do a physical exam and ask questions about your health history. Other tests and procedures might include imaging tests and taking a sample of tissue for lab testing. […] Imaging tests make pictures of the body. They can show the size and location of a cancer. For chordoma, imaging tests might include CT scans and MRIs. […] A biopsy is a procedure to remove a sample of tissue for testing in a lab. The sample is tested to see if it is cancer. Other tests give more details about the cancer cells. Your healthcare team uses this information to make a treatment plan. […] Deciding how the biopsy should be performed requires careful planning by your healthcare team. Your care team needs to perform the biopsy in a way that won’t cause problems with future surgery to remove the cancer. For this reason, it’s a good idea to seek care at a medical center that sees many people with chordoma.

• #64 Chordoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430846/

  Most physicians advocate for radiation therapy after any type of chordoma resection due to the high local recurrence rate. […] Due to the high local recurrence of chordomas, most physicians recommend lifelong surveillance with magnetic resonance imaging (MRI) with and without gadolinium contrast. […] It should be stressed that if chordoma is entertained in the differential diagnosis, there should be careful planning of any biopsy as the biopsy tract needs to be included in the ultimate resection of the chordoma to decrease the risk of local recurrence. […] Five-year survival is approximately 50% overall but improved with complete resection with negative margins to a 65% 5-year survival rate. […] Chordomas can be divided into four subtypes: conventional, poorly differentiated, dedifferentiated, and chondroid. […] The management of chordoma is best done with an interprofessional team that includes an orthopedic surgeon, radiologist, neurologist, and pathologist.

• #65 Chordoma – Macmillan Cancer Support | Macmillan Cancer Support

  https://www.macmillan.org.uk/cancer-information-and-support/bone-cancer/chordoma

  A bone biopsy means the doctor takes a sample of tissue from the bone to be checked for cancer under a microscope. […] The results of your tests give your cancer doctor information about the stage and grade of the chordoma. […] The stage refers to the size of the chordoma and whether it has spread outside the bone. […] Your test results will be discussed by a team of specialist health care professionals. If your tests show a diagnosis of bone cancer, a team of specialist doctors and other professionals will meet to discuss the best possible treatment for you. This is called a multidisciplinary team (MDT). […] The main treatments for chordoma are surgery and radiotherapy. Occasionally chemotherapy is used. […] Surgery is usually the main treatment for chordoma. If possible, the surgeon will remove all of the tumour. If not, it is often possible to remove part of it. This is called debulking surgery.

• #66 Symptoms and diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/diagnosis/

  Chordoma is not always easy to diagnose and it can be confused with other types of tumors. Getting the correct diagnosis from the start is critical to being able to find and choose the best treatment. […] Any biopsy or surgery to confirm the diagnosis of chordoma should be done at a center with expertise in chordoma treatment. If not done properly, these procedures can cause the chordoma to spread. […] If you have had an MRI and been given a probable or differential diagnosis of chordoma, it is very important to find doctors who have experience diagnosing and treating chordoma patients to confirm the diagnosis. […] Once you have been given a diagnosis, it can be helpful to get a second opinion to confirm the diagnosis before making treatment decisions. […] It is important to go to a hospital, treatment center, or network of treatment centers where doctors have experience diagnosing and treating chordoma as soon as chordoma is suspected.

• #67 Chordoma spinal cancer diagnosis and treatment | Chordoma UK

  https://chordoma-uk.org/diagnosis-and-treatment

  Patients with chordoma should be treated at specialist centres, whether they be private or through the NHS, where a team of knowledgeable physicians and health care workers are involved in a patient`s care. […] Imaging studies can show the possibility of a chordoma, but a definitive diagnosis can only be made by a pathologist who examines a sample of tumor tissue. […] For this reason, your medical team may consider taking a small sample of tissue from the tumor, called a biopsy, prior to surgery if the tumor can be reached safely. […] Tissue samples should be evaluated by a pathologist who has experience diagnosing bone tumors. […] Your pathologist may test your tumor tissue for the presence of a protein called brachyury. Nearly all chordomas have high levels of brachyury, which makes it helpful for diagnosis.

• #68 Chordoma | Brain Tumor Center | Stanford Medicine

  https://med.stanford.edu/brain-tumor/conditions/chordoma.html

  A dedicated skull base MRI scan is the first step in diagnosing chordoma. […] However, diagnosing a skull base chordoma is not always easy, as it can be confused with other types of clival tumors. […] Once you have been given a diagnosis, it can be helpful to get a second opinion to confirm the diagnosis before making treatment decisions. […] Biopsy and histologic studies are necessary to confirm the diagnosis, and distinguish chordomas from other lesions. […] However, in experienced hands, an MRI is usually sufficient to suspect the diagnosis of chordoma, obviating the need for undergoing a dedicated procedure for biopsy and proceeding directly with biopsy and surgical resection at the same time. […] It is important to go to a hospital, treatment center, or network of treatment centers where doctors have experience diagnosing and treating chordoma as soon as chordoma is suspected.

• #69 Chordoma: Practice Essentials, History of the Disease, Epidemiology

  https://emedicine.medscape.com/article/339169-overview

  Chordoma is a clinically and histologically unique malignant neoplasm, and numerous diagnostic considerations must be excluded to establish the correct diagnosis. […] Chordomas often present late in the disease course and tend to encapsulate adjacent neurovascular anatomy, seed resection cavities, recur locally, and respond poorly to radiotherapy and conventional chemotherapy—all of which make them challenging to treat. Extent of surgical resection and adequacy of surgical margins are the most important prognostic factors; thus, patients with chordoma should be cared for by a highly experienced, multidisciplinary surgical team at a quaternary center. […] Identification of molecular factors that are associated with survival contributes to better prognostication of patients with chordoma. Given the rarity of chordoma, often only univariate analyses can be performed. Robust multivariate analyses are scarcer but provide independently significant prognostic factors.

• #70 Symptoms and diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/diagnosis/

  Chordoma is not always easy to diagnose and it can be confused with other types of tumors. Getting the correct diagnosis from the start is critical to being able to find and choose the best treatment. […] Any biopsy or surgery to confirm the diagnosis of chordoma should be done at a center with expertise in chordoma treatment. If not done properly, these procedures can cause the chordoma to spread. […] If you have had an MRI and been given a probable or differential diagnosis of chordoma, it is very important to find doctors who have experience diagnosing and treating chordoma patients to confirm the diagnosis. […] Once you have been given a diagnosis, it can be helpful to get a second opinion to confirm the diagnosis before making treatment decisions. […] It is important to go to a hospital, treatment center, or network of treatment centers where doctors have experience diagnosing and treating chordoma as soon as chordoma is suspected.

• #71 Chordoma | Brain Tumor Center | Stanford Medicine

  https://med.stanford.edu/brain-tumor/conditions/chordoma.html

  A dedicated skull base MRI scan is the first step in diagnosing chordoma. […] However, diagnosing a skull base chordoma is not always easy, as it can be confused with other types of clival tumors. […] Once you have been given a diagnosis, it can be helpful to get a second opinion to confirm the diagnosis before making treatment decisions. […] Biopsy and histologic studies are necessary to confirm the diagnosis, and distinguish chordomas from other lesions. […] However, in experienced hands, an MRI is usually sufficient to suspect the diagnosis of chordoma, obviating the need for undergoing a dedicated procedure for biopsy and proceeding directly with biopsy and surgical resection at the same time. […] It is important to go to a hospital, treatment center, or network of treatment centers where doctors have experience diagnosing and treating chordoma as soon as chordoma is suspected.

• #72 Symptoms and diagnosis | Chordoma Foundation

  https://www.chordomafoundation.org/diagnosis/

  Chordoma is not always easy to diagnose and it can be confused with other types of tumors. Getting the correct diagnosis from the start is critical to being able to find and choose the best treatment. […] Any biopsy or surgery to confirm the diagnosis of chordoma should be done at a center with expertise in chordoma treatment. If not done properly, these procedures can cause the chordoma to spread. […] If you have had an MRI and been given a probable or differential diagnosis of chordoma, it is very important to find doctors who have experience diagnosing and treating chordoma patients to confirm the diagnosis. […] Once you have been given a diagnosis, it can be helpful to get a second opinion to confirm the diagnosis before making treatment decisions. […] It is important to go to a hospital, treatment center, or network of treatment centers where doctors have experience diagnosing and treating chordoma as soon as chordoma is suspected.

• #73 Chordoma Signs & Symptoms | Rush

  https://www.rush.edu/conditions/chordoma

  Chordomas are rare and slow-growing cancers of the bone that can occur anywhere along the spine from the base of the skull to the tailbone. […] Many of these are symptoms of multiple conditions, so its important to be evaluated by your doctor to get an accurate diagnosis. If you are diagnosed with chordoma, your doctor will refer you to a chordoma specialist. […] Ongoing monitoring and management of chordoma is important to make sure the cancer does not recur and if it does, to address it right away when it is most treatable. […] A chordoma diagnosis can be scary, and we encourage you to seek a second opinion to help you explore all possible treatment options.

• #74 Chordoma | Brain Tumor Center | Stanford Medicine

  https://med.stanford.edu/brain-tumor/conditions/chordoma.html

  You should avoid having a biopsy or surgery to confirm the diagnosis of chordoma outside of a referral center because, if not done properly, these procedures can cause the chordoma to spread. […] If you cannot travel to see a doctor who has experience with chordoma, have your imaging tests sent to a doctor with chordoma experience for a second opinion before you get treatment or a biopsy.

• #75 Chordoma of the skull base | Skull Base Surgery | Stanford Medicine

  https://med.stanford.edu/skullbasesurgery/conditions-we-treat/chordoma-of-the-skull-base.html

  You should avoid having a biopsy or surgery to confirm the diagnosis of chordoma outside of a referral center because, if not done properly, these procedures can cause the chordoma to spread. […] If you cannot travel to see a doctor who has experience with chordoma, have your imaging tests sent to a doctor with chordoma experience for a second opinion before you get treatment or a biopsy.

• #76 Chordoma: Practice Essentials, History of the Disease, Epidemiology

  https://emedicine.medscape.com/article/339169-overview

  Despite the low-grade status of chordomas, they have a high recurrence rate and involve significant mortality. Five-year survival is approximately 50% overall but is improved to 65% with complete resection with negative margins. […] Due to high local recurrence of chordomas, most physicians recommend lifelong surveillance with magnetic resonance imaging (MRI) with and without gadolinium contrast. Metastatic chordoma should be on the differential if new lesions arise elsewhere in the body, as up to 20% of chordomas can metastasize.

• #77 Chordoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430846/

  Most physicians advocate for radiation therapy after any type of chordoma resection due to the high local recurrence rate. […] Due to the high local recurrence of chordomas, most physicians recommend lifelong surveillance with magnetic resonance imaging (MRI) with and without gadolinium contrast. […] It should be stressed that if chordoma is entertained in the differential diagnosis, there should be careful planning of any biopsy as the biopsy tract needs to be included in the ultimate resection of the chordoma to decrease the risk of local recurrence. […] Five-year survival is approximately 50% overall but improved with complete resection with negative margins to a 65% 5-year survival rate. […] Chordomas can be divided into four subtypes: conventional, poorly differentiated, dedifferentiated, and chondroid. […] The management of chordoma is best done with an interprofessional team that includes an orthopedic surgeon, radiologist, neurologist, and pathologist.

• #78 Chordoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430846/

  Most physicians advocate for radiation therapy after any type of chordoma resection due to the high local recurrence rate. […] Due to the high local recurrence of chordomas, most physicians recommend lifelong surveillance with magnetic resonance imaging (MRI) with and without gadolinium contrast. […] It should be stressed that if chordoma is entertained in the differential diagnosis, there should be careful planning of any biopsy as the biopsy tract needs to be included in the ultimate resection of the chordoma to decrease the risk of local recurrence. […] Five-year survival is approximately 50% overall but improved with complete resection with negative margins to a 65% 5-year survival rate. […] Chordomas can be divided into four subtypes: conventional, poorly differentiated, dedifferentiated, and chondroid. […] The management of chordoma is best done with an interprofessional team that includes an orthopedic surgeon, radiologist, neurologist, and pathologist.

• #79 Chordoma – Macmillan Cancer Support | Macmillan Cancer Support

  https://www.macmillan.org.uk/cancer-information-and-support/bone-cancer/chordoma

  Radiotherapy uses high-energy rays to destroy the cancer cells. […] Chemotherapy uses anti-cancer (cytotoxic) drugs to destroy cancer cells. Chemotherapy is not often used to treat chordoma. It may occasionally be used to control certain types of chordoma that have spread or come back. […] After you finish treatment, you will have regular check-ups for a few years. This will include chest x-rays. You may also have scans and blood tests. […] Your cancer doctor or specialist nurse will explain more about any possible late effects and what can help to manage them. Always tell them if side effects are not improving.

• #80 Chordoma: Practice Essentials, History of the Disease, Epidemiology

  https://emedicine.medscape.com/article/339169-overview

  Despite the low-grade status of chordomas, they have a high recurrence rate and involve significant mortality. Five-year survival is approximately 50% overall but is improved to 65% with complete resection with negative margins. […] Due to high local recurrence of chordomas, most physicians recommend lifelong surveillance with magnetic resonance imaging (MRI) with and without gadolinium contrast. Metastatic chordoma should be on the differential if new lesions arise elsewhere in the body, as up to 20% of chordomas can metastasize.

• #81 Chordoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430846/

  Most physicians advocate for radiation therapy after any type of chordoma resection due to the high local recurrence rate. […] Due to the high local recurrence of chordomas, most physicians recommend lifelong surveillance with magnetic resonance imaging (MRI) with and without gadolinium contrast. […] It should be stressed that if chordoma is entertained in the differential diagnosis, there should be careful planning of any biopsy as the biopsy tract needs to be included in the ultimate resection of the chordoma to decrease the risk of local recurrence. […] Five-year survival is approximately 50% overall but improved with complete resection with negative margins to a 65% 5-year survival rate. […] Chordomas can be divided into four subtypes: conventional, poorly differentiated, dedifferentiated, and chondroid. […] The management of chordoma is best done with an interprofessional team that includes an orthopedic surgeon, radiologist, neurologist, and pathologist.

• #82 Chordoma: Practice Essentials, History of the Disease, Epidemiology

  https://emedicine.medscape.com/article/339169-overview

  Despite the low-grade status of chordomas, they have a high recurrence rate and involve significant mortality. Five-year survival is approximately 50% overall but is improved to 65% with complete resection with negative margins. […] Due to high local recurrence of chordomas, most physicians recommend lifelong surveillance with magnetic resonance imaging (MRI) with and without gadolinium contrast. Metastatic chordoma should be on the differential if new lesions arise elsewhere in the body, as up to 20% of chordomas can metastasize.

• #83 Chordoma – OrthoInfo – AAOS

  https://orthoinfo.aaos.org/en/diseases–conditions/chordoma/

  Because of their proximity to the brain and spinal cord, chordomas are very difficult to treat. […] Whenever possible, surgery is the preferred treatment option. Surgery is often combined with radiation to treat chordomas. […] Your outcome after treatment depends on a number of factors, including: Your age, The size and location of the tumor, The method of treatment. […] Advances in radiation therapy have allowed patients to receive higher doses of radiation with less damage to surrounding normal tissues, such as the brain and spinal cord. Different types of radiation therapy (including proton beam therapy, hadron therapy, intraoperative radiation, and brachytherapy) may offer better treatment of chordomas.

• #84 Chordoma: Practice Essentials, History of the Disease, Epidemiology

  https://emedicine.medscape.com/article/339169-overview

  Despite the low-grade status of chordomas, they have a high recurrence rate and involve significant mortality. Five-year survival is approximately 50% overall but is improved to 65% with complete resection with negative margins. […] Due to high local recurrence of chordomas, most physicians recommend lifelong surveillance with magnetic resonance imaging (MRI) with and without gadolinium contrast. Metastatic chordoma should be on the differential if new lesions arise elsewhere in the body, as up to 20% of chordomas can metastasize.

• #85 Chordomas | EBSCO Research Starters

  https://www.ebsco.com/research-starters/health-and-medicine/chordomas

  Chordomas are rare malignant bone tumors that primarily develop along the spinal cord, most frequently occurring at the base of the skull, the sacrum, or the coccyx. […] Diagnosis typically involves imaging tests such as CT and MRI scans, followed by a biopsy to confirm the presence of cancerous cells. […] Screening and diagnosis: Most chordomas grow slowly and do not spread, and the gradual development of symptoms often results in a delayed diagnosis. Patients often have symptoms for more than a year before diagnosis. Imaging tests such as computed tomography (CT) scans and magnetic resonance imaging (MRI) can reveal bone destruction and tissue damage. A biopsy of the tumor confirms the diagnosis. […] Survival rates depend on the location of the tumor. Persons with chordomas of the skull base tend to have the best prognosis. Overall, between 68 and 72 percent of persons with chordomas survive at least five years.

• #86 Chordoma | Bone Cancer Research Trust

  https://www.bcrt.org.uk/information/information-by-type/chordoma/

  The complete surgical removal of the chordoma offers the best chance of controlling the cancer. […] If the tumour is present at the base of the skull, surgery can often be carried out using a tube through the nose – which is known as an endoscopy. […] Ultimately, early diagnosis is key to achieving better control of the tumour and reducing the risk of recurrence.

• #87 Chordoma – Colorado Orthopedic News

  https://coloradoorthopedicnews.com/chordoma/

  Chordoma can be fatal, but with early diagnosis and appropriate treatment, many chordoma patients can live for a decade or more and some can be cured. […] Getting an accurate diagnosis and immediate treatment for chordoma can make all the difference in how quickly chordomas grow, the extent to which they can metastasize, the rate of recurrence, the neurological involvement, and most importantly, survival rates. […] The only way your surgeon can really diagnose a chordoma is with a needle biopsy. Determining how the biopsy should be performed requires careful planning by your medical team.

• #88 Chordoma—Current Understanding and Modern Treatment Paradigms

  https://www.mdpi.com/2077-0383/10/5/1054

  Aggressive resection (i.e., wide or marginal when feasible) is the most important prognostic indicator for patients with chordoma. […] Chordoma continues to present a considerable treatment challenge due to a number of factors, including: (1) indolent growth rate and resultant tendency to present late in the disease course, (2) propensity for invading through tissue planes, encapsulating critical nearby anatomy and seeding after resection and (3) its relative insensitivity to conventional radiotherapy and chemotherapy.

• #89 Chordoma – Colorado Orthopedic News

  https://coloradoorthopedicnews.com/chordoma/

  Chordoma can be fatal, but with early diagnosis and appropriate treatment, many chordoma patients can live for a decade or more and some can be cured. […] Getting an accurate diagnosis and immediate treatment for chordoma can make all the difference in how quickly chordomas grow, the extent to which they can metastasize, the rate of recurrence, the neurological involvement, and most importantly, survival rates. […] The only way your surgeon can really diagnose a chordoma is with a needle biopsy. Determining how the biopsy should be performed requires careful planning by your medical team.

• #90 Chordoma: Types, Causes, Symptoms, Treatment, and More

  https://www.verywellhealth.com/chordoma-5197631

  Chordomas are malignant but slow-growing bone tumors that can occur along the spine or lower portion of the skull. […] This article will provide an overview of chordomas and their causes, as well as symptoms, diagnosis, and treatment options. […] Confirming a chordoma diagnosis may require a biopsy and diagnostic imaging. […] A biopsy is a collection of cells from a suspicious area, which are sent for laboratory testing to determine if cancerous cells are present. […] Diagnostic imaging can help doctors visualize the chordoma and see if it has spread to other areas. […] Treatment for chordomas can be complex because they form close to the spinal cord, brain, nerves, or arteries. […] A chordoma diagnosis can be overwhelming. However, there are successful treatments and specialists who can help. […] Early detection offers the best treatment options and improves the overall prognosis.

• #91 Chordoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430846/

  A chordoma is a low-grade, slow-growing but locally invasive and locally aggressive tumor that is a type of sarcoma. […] This activity describes the clinical evaluation of chordoma and explains the role of the health professional team in coordinating the care of this condition. […] Outline the clinical evaluation of a chordoma. […] Describe how enhanced coordination of the interprofessional team can lead to more rapid recognition of chordomas and subsequently their evaluation, enhancing detection of this pathology and allowing for treatment when appropriate. […] Evaluation of chordomas revolves around imaging and biopsy. […] Many times a needle or open biopsy is performed to confirm the diagnosis of chordoma. […] The treatment which provides the longest survival is complete en bloc resection of the tumor with clean margins.

• #92 Chordoma—Current Understanding and Modern Treatment Paradigms

  https://www.mdpi.com/2077-0383/10/5/1054

  Though the diagnosis of chordoma may be suspected based on patient presentation and radiographic imaging, diagnostic certainty requires histopathological evaluation. […] Chordomas pose a considerable treatment challenge due to their midline location, predilection for involving critical neurovascular anatomy, indolent growth patterns, tendency to seed/recur and resistance to traditional chemoradiotherapeutic modalities. […] Surgical resection is currently the mainstay of treatment for chordoma and extent of surgical resection is one of the most important prognostic factors for patients with this disease. […] Recommendations for surgical margins may vary based on the grade of tumor and the presence of metastases and in some cases, radiotherapy and/or palliative procedures may be recommended rather than aggressive resection.

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