Materiały źródłowe

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

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

  Chordoma is a rare bone tumor that forms in your spine or skull base. Surgical removal of the tumor is the first-line treatment, but it can be difficult to completely remove chordomas due to their location near your spinal cord or brainstem. […] The go-to treatment option for chordoma is surgery. Total surgical removal of the tumor (en bloc resection) has the best chance of prolonging survival. However, this is often difficult due to the location of the tumors. Specifically, this isn’t possible for chordomas in the base of your skull. […] Chordomas are generally resistant to radiation therapy and chemotherapy as primary treatments. But your healthcare team might recommend radiation therapy after surgery to lower the chance that the tumor will grow back. […] Researchers are currently studying experimental therapies for chordomas, such as targeted therapy and immunotherapy. There may be clinical trials available that you can participate in. […] Learning you have a rare type of bone cancer can be scary and stressful. Chordomas can be difficult to treat because of their location. But know that your healthcare team will develop an individualized and thorough treatment plan to help treat the chordoma and improve your quality of life.

• #2 Chordoma—Current Understanding and Modern Treatment Paradigms

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7961966/

  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. Chordomas are known to have a propensity for seeding tumor cells throughout a surgical corridor, contributing to tumor recurrence and thus en bloc resection without capsule violation when feasible has often been considered the surgical gold standard. Chordomas occur most commonly in the midline, however, and tend to invade in and around critical neurovascular structures, making en bloc resection a challenge without imparting considerable morbidity. Furthermore, the concept of en bloc resection, in the absence of a description of margins by an experienced pathologist, is imprecise and does not adequately distinguish between margins that contain neoplastic tissue and those that do not. Decision-making regarding surgical approach and extent of resection must be undertaken as a cost-benefit analysis with consideration of a multitude of patient- and tumor-specific factors, including tumor location, the neurovascular anatomy involved and patient functional status, among others, with the goal being to achieve as radical of resection as possible at first presentation while avoiding morbidity.

• #3 Chordoma Treatment & Management: Medical Therapy, Surgical Therapy, Follow-up

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

  Despite the fact that surgery remains the cornerstone of treatment, total resection is not reasonable for all patients with chordoma. For this reason, adjuvant treatment is highly important to ensure local control. If the residual tumor is of small volume, stereotactic body radiation therapy (SBRT) may provide advantages. […] Research is ongoing, and surgery remains the standard treatment for chordoma. Adjuvant radiation therapy is used in cases in which incomplete resection is suspected. Traditional chemotherapy has not been shown to be effective. […] Surgical resection remains the primary mode of treatment for both diagnostic and therapeutic purposes. The prognosis for patients with chordoma generally depends on extent and completeness of tumor excision. […] Radical resection of tumors with clean margins is associated with a longer disease-free interval. If subtotal excision is the only option (generally due to location and proximity to delicate anatomy), added radiation therapy can lengthen the interval to recurrence.

• #4 Chordoma | Brain Tumor Center | Stanford Medicine

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

  Chordoma treatment begins with surgery. […] As stated by The Chordoma Foundation: No matter what stage of the disease you are facing [new, recurrent, advanced, metastatic] or where your tumor is located, the way surgery is performed is very important. […] At Stanford, the preferred surgical treatment for most chordomas of the skull base is the Endoscopic Endonasal Approach (EEA). This innovative, minimally invasive technique uses the nose and nasal cavities as natural corridors to access hard-to-reach or previously inoperable tumors. […] The goal of the Stanford Skull Base team is to perform, whenever possible, a supratotal tumor resection, where the entire tumor is removed along with at least 1 millimeter of healthy tissue around the tumor, and at the same time minimize side effects and surgical complications.

• #5 Northwestern Medicine Chordoma Center | Northwestern Medicine

  https://www.nm.org/conditions-and-care-areas/neurosciences/chordoma-center

  While many surgeons may only perform one chordoma surgery in their lifetime, our chordoma surgeon performs dozens of these surgeries each year. Jean-Paul Wolinsky, MD, is nationally recognized in his excellence in chordoma removal. […] Choosing the best tumor surgeon is the most important decision you can make when facing a chordoma because it is one of the few tumors that can seed its cells to surrounding tissues. If the tumor happens to be ruptured during surgery, tumor cells can spill into surrounding areas and grow uncontrolled. The first surgery is your best chance at a cure. […] Your care team at Northwestern Medicine Chordoma Center works closely with specialists at Northwestern Medicine Proton Center, one of the few proton therapy centers in the Midwest and the first one in Illinois.

• #6 Spinal Chordoma: A Rare Tumor Requiring Complex Surgical Care | Department of Neurological Surgery

  https://neurosurgery.ucsf.edu/news/spinal-chordoma-rare-tumor-requiring-complex-surgical-care

  With an estimated 300 new cases diagnosed each year in the United States, chordoma is a rare tumor that occurs in the brain or spine. […] However in the past year, UCSF neurosurgeons have treated several patients with chordoma, each requiring complex surgical procedures to remove the tumor. […] For spinal chordomas, en bloc resection provides the best prognosis for disease-free survival, said co-author and neurosurgeon Dean Chou, MD. By removing the tumor whole, the spread of residual cells and likelihood of recurrence is greatly reduced. […] Indeed, Chou strongly recommends to any referring physician, If you suspect it may be spinal chordoma even if there is a remote chance get a CT guided biopsy first. To operate on the tumor and remove it piecemeal would impact recurrence and ultimately be detrimental.

• #7 Chordoma—Current Understanding and Modern Treatment Paradigms

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7961966/

  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. Advances in surgical techniques and approaches, image guidance, radiotherapy strategies and the emergence of promising targeted molecular therapies, however, are beginning to change the outlook of the disease. In order to achieve the best possible patient outcomes, it is crucial that patients with chordomas are treated by multidisciplinary teams in experienced quaternary centers, where a well-planned biopsy, appropriate staging, maximal safe surgical resection, molecular sequencing, modern medical therapy, radiotherapy and relevant clinical trials are all accessible and included in the decision-making process. Performance of even some invasive diagnostic procedures outside of an experienced center has been shown to negatively impact patient prognosis.

• #8 Spinal Chordoma: A Rare Tumor Requiring Complex Surgical Care | Department of Neurological Surgery

  https://neurosurgery.ucsf.edu/news/spinal-chordoma-rare-tumor-requiring-complex-surgical-care

  A multidisciplinary surgical team is needed to remove chordomas from the thoracic spine when the tumor is abutting the heart and lungs and invading the spinal canal, said Mummaneni. […] Overall, no matter where in the spine these tumors occur, chordomas require complex and technically demanding surgical approaches. […] According to Chou, a large medical center might see two to five chordoma cases per year, with a neurosurgeon seeing less than 50 chordomas in their entire career. Often, the complicated procedures require expert, multi-disciplinary collaboration across surgical specialties. […] Together with experts across UCSF, our neurosurgeons are continually refining treatment for patients with chordoma. These are very complex operations, said Chou, and you want a team with many years of experience performing these procedures.

• #9 Chordoma | Bone Cancer Research Trust

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

  As with in the majority of primary bone cancers, the main treatment option for chordoma patients is surgery. The slow-growing nature of this tumour type, and its low risk of spreading to other areas of the body, makes the surgical removal of the tumour a beneficial method of treatment. The removal of the tumour alongside a small amount of healthy tissue reduces the risk of the tumour returning at a later date and ensures all cancer cells are removed from the area. This procedure is known as taking a ‘wide-surgical margin’. […] Unfortunately, the location of a chordoma can often make the planning of surgical margins difficult. Chordomas present on the spine and the skull and are therefore frequently located nearby to major nerves, structures and critical organs. 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. The location of the tumour may also mean that it is not always possible to remove a margin of healthy tissue too.

• #10 Chordoma – Diagnosis and treatment – Mayo Clinic

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

  Chordoma treatment depends on the size and location of the cancer, as well as whether it has invaded nerves or other tissue. Options may include surgery, radiation therapy and targeted therapies. […] The goal of surgery for a sacral spine chordoma is to remove all of the cancer and some of the healthy tissue that surrounds it. […] Radiation therapy treats cancer with powerful energy beams. […] Radiation therapy may be used before surgery to shrink a cancer and make it easier to remove. It also can be used after surgery to kill any cancer cells that remain. If surgery isn’t an option, radiation therapy may be recommended instead. […] Targeted therapy for cancer is a treatment that uses medicines that attack specific chemicals in the cancer cells. By blocking these chemicals, targeted treatments can cause cancer cells to die. Targeted therapy is sometimes used to treat chordoma that spreads to other areas of the body or chordoma that comes back after treatment.

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

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

  Chordoma is rare. You will be treated by a team of doctors and other healthcare professionals at a hospital with a specialist sarcoma treatment centre. […] 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. This can help your symptoms by relieving the pressure on the nerves. It can also slow the growth of the tumour.

• #12 Radiation | Chordoma Foundation

  https://www.chordomafoundation.org/treatment-options/radiation/

  The purpose of radiation therapy is to kill or stop the growth of tumor cells within the body. Radiation can serve different roles in the treatment of chordoma, which can vary based on the stage of the disease. […] Radiation is usually recommended after surgery to kill any remaining tumor cells that are left behind in your body after surgery. In some cases, radiation is given both before and after surgery. This method is typically used for sacral tumors and sometimes mobile spine tumors. […] Radiation is sometimes recommended as the primary treatment following a biopsy if the tumor cannot be removed with surgery or if the risks of surgery are unacceptable to you. […] If you experience a recurrence and are able to receive high-dose radiation, radiation with or without surgery is an option. In some cases, high-dose radiation can be given for recurrent tumors as part of a treatment plan with curative intent.

• #13 Radiation | Chordoma Foundation

  https://www.chordomafoundation.org/treatment-options/radiation/

  If you cannot have high-dose radiation, a lower dose may be given to help slow the growth of the recurrent tumor and ease symptoms you might be experiencing. […] If your tumor has spread to other areas of your body, radiation can sometimes be given to one or more of the tumors in those areas. […] Radiation helps stimulate the immune system to cause it to kill cancer cells, which may help some systemic therapies work better. […] Low-dose radiation can be used to help slow tumor progression and ease any symptoms you might be experiencing. […] Chordomas require very high doses of radiation to be controlled. Specifically, a total dose of at least 74 GyE (Grey Equivalents) is recommended for proton and photon radiation. […] Lower doses of radiation are sometimes given to help slow the growth of recurrent or advanced tumors and ease symptoms like pain and nerve damage. Radiation may also be given along with drug therapies to help increase their effectiveness. […] Any time you are considering treatment it is a good idea to talk with your medical team about the role that radiation therapy should play in your treatment.

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

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

  There are newer methods of radiotherapy that are sometime used to treat chordoma. Stereotactic radiotherapy, image-guided radiotherapy (IGRT) and intensity-modulated radiotherapy (IMRT) all allow doctors to direct higher doses of radiation more accurately at the tumour and minimise the side effects of treatment. […] Proton beam therapy can be effective in treating chordoma. It is a different type of radiotherapy that uses high-energy protons rather than high-energy (photon) radiation to deliver a dose of radiotherapy.

• #15 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. However, as there is not much space around these tumours and nearby critical structures, the tumour is often removed and radiotherapy is carried out to destroy any remaining cancer cells in the area. Therefore surgery followed by radiotherapy treatment offers the best possible control of the chordoma and lowers the probability of the tumour returning at a later date. […] Radiotherapy is used following surgery to ensure all cancer cells in the area are destroyed. Radiotherapy is not usually used on its own for the treatment of chordoma, as a very high dose of radiotherapy is needed to kill the cancerous chordoma cells. […] Proton Beam Therapy (PBT) is a type of radiotherapy that uses beams of 'protons’ (energised particles), instead of beams of X-rays (photons), that are used in conventional radiotherapy. It is more targeted than conventional radiotherapy so does less damage to the healthy tissue surrounding the tumour and other organs. This is advantageous for some primary bone cancers where the cancer is close to a critical part of the body such as the spinal cord.

• #16 Chordoma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/chordoma/cdc-20355401

  Chordoma usually grows slowly. It can be difficult to treat because a chordoma can grow very close to the spinal cord and other important structures, such as arteries, nerves or the brain. This cancer often comes back after treatment. […] Diagnosis treatment March 14, 2025 […] Chordoma Current understanding and modern treatment paradigms. Journal of Clinical Medicine. 2021; doi:10.3390/jcm10051054. […] Stereotactic radiosurgery (SRS) and stereotactic body radiotherapy (SBRT). RadiologyInfo.org. https://www.radiologyinfo.org/en/info/stereotactic. Accessed Jan. 29, 2025.

• #17 Spinal chordoma and chondrosarcoma treatment experiences – a 20-year retrospective study from databases of two medical centers | Scientific Reports

  https://www.nature.com/articles/s41598-024-74317-4

  When achieving negative margins is challenging, RT utilizing the heavy charged particle carbon ion, known for its sharper lateral penumbra, is seen as a promising treatment option. […] Additionally, hypofractionated stereotactic body radiation therapy (SBRT) and stereotactic radiosurgery (SRS) are under consideration as potential therapies for unresectable vertebral chordoma and chondrosarcoma due to advancements in geometric accuracy. […] The efficacy of therapy was found to be significantly influenced by complete surgical resection, corroborating findings from previous studies. […] While total excision is considered the optimal treatment approach, it is essential to weigh the risks of perioperative complications, including damage to nearby structures, neurological impairments, wound complications such as dehiscence or infection, medical morbidity stemming from surgical trauma, and potential long-term device failure.

• #18 The Benefits of Proton Therapy for Chordomas – New York Proton Center

  https://www.nyproton.com/benefits-of-proton-therapy-for-chordomas/

  Proton therapy is an effective treatment for chordomas, malignant tumors that occur in the spine or the base of skull. […] Dr. Chhabra recently led a Proton Collaborative Group (PCG) multicenter study on the efficacy of high dose proton therapy for chordoma, with results described in a paper just published in The Green Journal. The study of 100 chordoma patients demonstrated that high dose proton therapy achieved excellent efficacy and outcomes with low rates of toxicity. […] In addition to being the optimal treatment for patients with an initial diagnosis of chordoma, proton therapy is typically the only type of radiation that can safely retreat recurrent chordoma.

• #19 Chordoma—Current Understanding and Modern Treatment Paradigms

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7961966/

  By most accounts, conventional radiotherapy is ineffective as a stand-alone treatment or when coupled with intralesional resections for chordoma. The effectiveness of even modern radiotherapy in chordoma is a matter of controversy, with several recent series demonstrating little benefit. Emerging evidence suggests, however, that modern radiotherapy may have a role as an adjuvant to aggressive resections; particularly when performed early in the disease course and some evidence exists that high-dose stereotactic radiotherapy provides durable local control even when used as a definitive treatment for patients unable to undergo an aggressive surgical resection. […] Chordoma is notoriously insensitive to traditional chemotherapeutic agents but ongoing research into the molecular pathways underlying chordoma pathophysiology have led to a number of promising targeted molecular agents. Imatinib and sunitinib, for example, are both tyrosine kinase inhibitors with activity against PDGFR, KIT receptors, vascular endothelial growth factor receptors and other molecular pathway elements known to be overexpressed in chordoma. Both have shown modest efficacy in early clinical trials, although further study is needed. Overexpression of EGFR and c-MET in some chordomas has led to the use of several agents with anti-EGFR activity, including erlotinib and lapatinib, both of which have been suggested to have some degree of clinical efficacy by small series and/or case reports.

• #20 New Hope in Chordoma Research

  https://www.cancerresearch.org/blog/july-2019/chordoma-foundation-joan-levy-interview

  To determine whether immunotherapy might be an effective option for this hard-to-treat disease, the Cancer Research Institute launched a partnership with the Chordoma Foundation to identify and fund promising chordoma research involving cancer immunology and immunotherapy with the aim of aiding the development of better treatments for these patients. […] Currently, chordoma is treated primarily by surgery with or without radiation. There have been improvements in surgical techniques, and there have been improvements in the types of radiation that is used. However, there are still no systemic treatments that have been approved for chordoma patients. […] Chemotherapy has been tried in chordoma and in the majority of patients who are classified as having conventional chordoma, the chemotherapy agents typically do not demonstrate any clinically significant activity.

• #21 Chordoma Causes, Symptoms, and Treatments

  https://www.upmc.com/services/orthopaedics/conditions/chordoma

  Chemotherapy usually isn’t effective against chordomas. The exception is dedifferentiated chordomas, which grow faster than other forms of chordoma and are more sensitive to chemotherapy. […] The outcome of your treatment will depend on many factors. They include: If the entire tumor was able to be removed. The method of treatment. Where the tumor was located. Your age and overall health.

• #22

  https://link.springer.com/article/10.1007/s44194-022-00017-8

  NCCN guidelines recommend that targeted therapy can be used for the systemic treatment of chordoma. Imatinib, dasatinib, sorafenib, sunitinib, and lapatinib have shown significant clinical efficacy. […] Imatinib is a specific tyrosine kinase inhibitor targeting PDGFR, and it is the first targeted therapeutic drug for myeloma. NCCN recommends imatinib monotherapy for the first-line treatment of chordoma with local recurrence or metastasis. […] Dasatinib is a PDGFR and Src inhibitor, which is recommended by NCCN guidelines for the first-line treatment of locally recurrent and metastatic chordoma. […] Lapatinib can be used in the treatment of locally recurrent or metastatic drug-resistant chordomas. […] Erlotinib is the most commonly used EGFR inhibitor, and NCCN guidelines recommend a single drug for the treatment of recurrent refractory chordoma.

• #23 Chordoma Guidelines: Guidelines Summary

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

  Dedifferentiated chordomas are treated according to soft tissue sarcoma management guidelines; chemotherapy can be provided when clinically indicated. […] For local recurrence, surgical excision should be performed with or without radiation therapy and/or chemotherapy. […] For metastatic disease, options include chemotherapy and/or surgical excision and/or radiation therapy and/or best supportive care. […] Treatment options may include surgical excision and/or radiation therapy and/or systemic therapy, along with best supportive care. […] Systemic therapy options may include use of imatinib, dasatinib, sunitinib, cisplatin or sirolimus, erlotinib, lapatinib (for epidermal growth factor receptor [EGFR]-positive chordoma), and sorafenib, given alone or in combination. […] Treatment options may include surgery, radiation therapy, and other local treatments.

• #24

  https://link.springer.com/article/10.1007/s44194-022-00017-8

  Sorafenib is a kind of tyrosine kinase inhibitor targeting VEGFR and PDGFR. […] Thalidomide is also a VEGFR inhibitor. It can be used as a second-line treatment after the treatment failure of the combination therapy of imatinib and rapamycin. […] The immune microenvironment of a chordoma has interactions with chordoma in many circumstances, affecting the invasiveness of tumors. New treatments targeting the immune microenvironment have been developed. […] Studies have reported that after the use of pembrolizumab in patients with chordoma who failed the standard treatment, the tumor subsided significantly. […] Cisplatin is an alkylating agent, which acts on the DNA of chordoma cells, affects the expression of genetic material of chordoma cells, and works in synergy with imatinib. NCCN guidelines recommend that the combined treatment of imatinib and cisplatin can be used for drug-resistant and relapsed refractory chordomas.

• #25 Chordoma – NCI

  https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-bone-tumors/chordoma

  When possible, chordomas are removed with surgery. Radiation therapy is sometimes used after surgery, or alone if surgery is not possible. Chordomas can be difficult to treat because they grow on the spine, near important tissues like nerves and blood vessels. It can be hard to get all of the tumor out of your spine without hurting the healthy parts of your body. […] New research is being done using immunotherapy to treat chordomas and to find other treatments. Immunotherapy is a cancer treatment that uses the bodys immune system to fight the disease. […] It is important to talk with a team of specialists to decide what the right treatment is for you. You can contact MyPART to get help finding specialists.

• #26 New Hope in Chordoma Research

  https://www.cancerresearch.org/blog/july-2019/chordoma-foundation-joan-levy-interview

  Many of the trials in chordoma are exploring the combination of different types of immunotherapy and radiation, like the combination of brachyury vaccine and radiation as well as the combination of checkpoint immunotherapy and radiation. […] I think that the collaboration will enable many people in the chordoma research community who are interested in immunology and immunotherapy to really think about how they could translate some of the basic scientific findings to the clinic. […] I think the investigators will be looking forward to this research opportunity to bring new therapeutics, like immunotherapy, to chordoma patients, especially those with advanced disease, who don’t have the systemic treatment options.

• #27 Chordoma: The Quest for Better Treatment Options

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5315072/

  Surgery, when feasible, should aim for margin-negative resection without penetration of the capsule, with adjuvant high-dose radiotherapy for optimal local tumor control. […] Patients with residual disease after surgery or unresectable disease at the time of diagnosis should consider high-dose radiotherapy at a center with expertise in delivering such doses for chordoma. […] To date, there has been no randomized, controlled trial of a therapeutic agent in locally advanced and/or metastatic chordoma. […] The combination of the rarity of the disease and the diversity of upfront clinical management practice makes clinical trial design and enrollment difficult. […] Despite rational selection of agents based on potential therapeutic targets identified by extensive preclinical work, radiographic response rates have been very low. […] The recent activity seen in other tumor types treated with immunotherapy suggests that therapeutic cancer vaccines, immune checkpoint inhibitors, or a combination of these agents may be potential therapeutic options for chordoma.

• #28

  https://link.springer.com/article/10.1007/s44194-022-00017-8

  Chordoma is a rare bone tumor often present in the skull base and spine. In addition, it is not sensitive to radiotherapy that surgical resection is of great significance for the treatment of chordoma. […] Targeted therapy and immunotherapy can improve the prognosis of chordoma patients to some extent. This review focuses on the clinical trials related to targeted therapy, immunotherapy, and chemotherapy of chordoma. […] According to the latest NCCN guidelines, the initial treatment is surgery with or without radiotherapy, and the adjuvant treatment is radiotherapy or reoperation for the positive margin of the tumor. For patients with local recurrence and metastasis recurrence, treatments like surgery, radiotherapy, and systemic treatment can be considered, including chemotherapy, targeted therapy, and immunotherapy. The current clinical trials of targeted therapy and immunotherapy have achieved certain clinical benefits, and the safety is acceptable, which brings a new dawn to the treatment of patients.

• #29 Oncology Central | Largest Study of Chordoma and New Treatment Options

  https://www.oncology-central.com/largest-genomics-study-chordoma-reveals-potential-new-treatment-option/

  The largest genomics study to date of chordoma, a rare bone cancer of the spine and skull, has discovered a group of patients who possess mutations in genes known to be targets of existing drug-PI3K inhibitors. […] The team suggests a further clinical trial investigating P13K inhibitors could be beneficial for chordoma patients who possess these mutations. […] The only effective treatment options currently are surgery and radiation therapy. […] We have shown that a particular group of chordoma patients could be treated with PI3K inhibitors, based on their mutations. […] In the future we hope to target chordoma from three angles: trialing PI3K inhibitors in chordoma; developing a therapeutic approach to switching off the extra copy of the T gene and studying the function of LYST as a cancer gene and its role in the development of chordoma.

• #30 Chordoma Treatment & Management: Medical Therapy, Surgical Therapy, Follow-up

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

  Currently, there is no approved medical treatment for individuals with diagnosed chordoma. Surgical en bloc resection with negative margins is the only curative treatment for this disease. High-dose irradiation, particularly with protons and carbon ions, is a therapeutic alternative in cases of inoperable tumor. Clinical trials exploring additional therapeutic modalities are ongoing. […] In a systematic review of 33 studies that reported on use of molecular targeted therapy (MTT) for advanced chordoma, the investigators extracted and analyzed data on clinical outcomes, such as median overall survival, progression-free survival, response rate, and adverse events. They found that imatinib and erlotinib were the most frequently used molecular targeted inhibitors for chordoma, and that for PDGFR-positive and/or EGFR-positive chordomas, clinical benefits were achieved with acceptable adverse events. They stated that monotherapy is preferred as first-line treatment, and combined drug therapy as second-line treatment. Tyrosine kinase inhibitor (TKI) monotherapy is recommended for first-line management, and combination therapy (2 TKIs or 1 TKI plus 1 mTOR inhibitor) may be the choice for drug-resistant chordoma. Brachyury vaccine is a promising therapeutic strategy; additional clinical trials must be undertaken to evaluate its safety and efficacy.

• #31 Signs of effective treatment of rare spine cancer emerge from clinical trial at Providence Saint John’s Health Center’s Cancer Institute Pilot study shows promise in treating chordoma – Saint John’s Cancer Institute

  https://www.saintjohnscancer.org/about-us/news/news/signs-of-effective-treatment-of-rare-spine-cancer-emerge-from-clinical-trial-at-providence-saint-johns-health-centers-cancer-institute-pilot-study-shows-promise-in-treating-chordoma/

  Promising results of a pilot study at Providence Saint Johns Health Center to treat a rare form of cancer of the spine and skull base were published today by the journal Clinical Cancer Research. […] The trial studied pemetrexed, a multi-pronged anti-folate chemotherapy commonly used in treating a variety of cancers. […] While no drugs have been approved by the Food and Drug Administration to treat chordoma, pemetrexed appears to be an effective drug in our study. […] The purpose of the study was to test the safety and tolerability of high doses of pemetrexed in patients with progressive chordoma, monitor any new side effects, understand how the drug might affect the growth of tumor cells, and collect tumor and blood samples for identifying biomarkers of response. […] The median progression-free survival was 10 months, and 6-month PFS was 67%. […] Given the limitations of available systemic therapy options, this early evidence of activity of pemetrexed is very encouraging and provides opportunities for further development of combinations to improve upon this backbone.

• #32 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. The team should involve radiologists to suggest the initial diagnosis (through CT/CAT, MRI PET Scanning), pathologists to make the diagnosis on a small sample of tissue (biopsy) removed from the tumour, surgeons to operate, and oncologists to treat after surgery with radiotherapy and drug therapies as appropriate. A rehabilitation team also plays an important role in a patients recovery. […] Your first treatments will have a big impact on both quality of life after treatment and the chances of the tumor coming back (recurrence). In most cases, surgery is recommended as the first treatment. Radiation therapy is generally recommended after surgery to kill any remaining tumor cells and reduce the chance of recurrence. A radiation oncologist should be part of your Multidisciplinary Team even before surgery to plan with your surgeons for any radiation treatment you will receive after surgery.

• #33 Northwestern Medicine Chordoma Center | Northwestern Medicine

  https://www.nm.org/conditions-and-care-areas/neurosciences/chordoma-center

  Chordomas are rare tumors that occur in the bones of the skull base and spine. They are malignant, potentially fatal tumors that tend to grow slowly and spread to other parts of the body if left untreated. […] At the Northwestern Medicine Chordoma Center, part of the Lou and Jean Malnati Brain Tumor Institute of Robert H. Lurie Comprehensive Cancer Center of Northwestern University at Northwestern Memorial Hospital, we provide advanced treatment options for patients from around the world who have chordomas. Our patients are cared for by a team of experts, including neurosurgeons, orthopaedic surgeons, radiation oncologists, medical oncologists, reconstructive/plastic surgeons, oncology social workers and rehabilitation specialists. […] Our physicians are internationally recognized for their expertise in diagnosing and treating chordomas. We understand the importance of seamless, coordinated care for patients facing this complex disease.

• #34 Chordoma – Brown Neurosurgery

  https://brownneurosurgery.com/portfolio/chordoma/

  The Brown University Chordoma Center at Rhode Island Hospital combines extensive experience and technical expertise in the multidisciplinary treatment of chordoma arising from the spine and skull base. Chordoma is a rare and aggressive bone tumor. They arise from remnants of embryonic cells present at birth and continue to grow anywhere along the spine from the skull base to the sacrum. Although slow-growing, they can be life-threatening if not treated properly. Effective treatment is challenging and often requires the removal of the entire tumor in one piece (en-bloc resection technique) while operating near critical neural and vascular structures. […] Our center is led by the chairman of the Brown / Lifespan Neurosurgery Department, Dr. Ziya L. Gokaslan, a world-renowned neurosurgeon with unique expertise in spinal chordoma treatment. Our team of neurosurgeons, general surgeons, plastic surgeons, oncologists, and radiation oncologists work together to ensure patients receive personalized specialty care to ensure an optimal treatment outcome. […] “Chordomas are best treated with complete removal, with en bloc resection of the tumor. Those are some of the techniques I developed over the years to be able to do that in any location in the spinal column.” – Ziya L. Gokaslan, MD

• #35 Chordoma Treatment & Management: Medical Therapy, Surgical Therapy, Follow-up

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

  Patients with chordoma who are admitted to inpatient rehabilitation facilities after surgical tumor resection experience improvement in multiple functional domains and a high rate of acute hospital transfer, but this is comparable to that seen with other cancer rehabilitation populations. […] For the present, the mainstay of therapy for chordoma remains primary surgical excision, with radiation therapy added for incompletely resected tumors. The near future will likely see the confirmation of potential therapeutic targets, such as signaling molecules in the pathways mentioned above. Inhibitors of these pathways may then be used to arrest the progression of disease, especially for patients with incomplete resection or recurrent/metastatic disease. […] Although these molecular studies continue to explore new treatment modalities for chordoma, the most significant dilemma remains the choice between a radical surgical procedure with the potential for serious morbidity and a subtotal resection with increased potential for recurrence. The general health of the patient should be considered during planning for the surgical procedure.

• #36 Northwestern Medicine Chordoma Center | Northwestern Medicine

  https://www.nm.org/conditions-and-care-areas/neurosciences/chordoma-center

  In treating tumors near the spine, proton therapy uses highly targeted treatment to precisely deliver the radiation dose to the tumor. This level of precision concentrates energy on destroying the tumor while limiting damage to surrounding healthy tissue. […] Northwestern Medicine Chordoma Center offers comprehensive post-operative care services, including physical and occupational therapy, through Northwestern Medicine Marianjoy Rehabilitation Hospital and the Shirley Ryan AbilityLab, formerly known as Rehabilitation Institute of Chicago (RIC). Your rehabilitation team will work closely with your medical and surgical team to help ensure the best recovery possible. […] Northwestern Medicine physicians are working to develop new treatments for chordoma, including use of 3-D printing for spine reconstruction and other new technologies for the removal of spinal tumors. There are also new chemotherapeutic options for patients through clinical trials, as well as the use of radiation therapy in the form of proton beam, the newest type of stereotactic radiosurgery, available at only a few research centers in the country.

• #37 Frequently Asked Questions | Northwestern Medicine

  https://www.nm.org/conditions-and-care-areas/neurosciences/chordoma-center/chordoma/frequently-asked-questions

  Chordomas, however, need a significantly higher dose of radiation therapy to be destroyed a dose too toxic for the body to endure. […] Chemotherapy is also not an effective first-line treatment for chordomas. Patients with reoccurring tumors may, however, be candidates for clinical trials. […] Treating a chordoma requires a complex, well-managed care plan. It is critical that patients with chordomas get their treatment at centers with specialists who are experienced in treating these tumors. […] Yes. Some chordomas can reoccur, even after the entire tumor and its surrounding tissue have been removed. Treatment options may then involve removing the new tumor, radiation therapy or chemotherapy. […] Since these tumors can reoccur sometimes years later we recommend lifelong monitoring for patients. Monitoring plans are tailored specifically for each patient; often this involves an MRI evaluation every 6 months.

• #38 Chordoma: New Updates and Breakthroughs 2024

  https://oncodaily.com/oncolibrary/cancer-types/76224

  Recent advances in molecular biology have led to the development of targeted therapies and immunotherapies for this tumor. […] The prognosis for chordoma patients varies significantly based on several factors, including the tumors location, size, histological subtype, and the extent of surgical resection. The overall 5-year survival rate is approximately 50%. […] Complete surgical resection combined with high-dose radiation therapy offers the best chance for long-term survival. […] Chordoma treatment, which often involves surgery and radiation therapy, can lead to a range of complications and side effects. […] Effective pain management strategies include medications, physical therapy, and, in some cases, nerve blocks. […] Survivorship care plans, addressing both physical and emotional needs, are crucial for managing the long-term effects of treatment.

• #39 Chordoma—Current Understanding and Modern Treatment Paradigms

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7961966/

  Aggressive resection (i.e., wide or marginal when feasible) is the most important prognostic indicator for patients with chordoma. Data from epidemiologic studies indicate that the overall mean survival for patients with chordoma is 6.29 years, with 67.6% surviving at 5 years, 39.9% at 10 years and 13.1% at 20 years. These data were collected over 20 years ago, however and only 84% of these patients underwent surgery. Furthermore, the tumor location/extent and surgical margins achieved in these patients are unclear. With modern, aggressive surgery, 10-year overall survival rates of 95% have been reported for skull base chordomas and 58-100% for chordomas of the mobile spine and sacrum.

• #40 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. […] Even among conventional chordomas, however, biologically distinct tumor types appear to be present, with some chordomas behaving more aggressively than others despite apparently conventional histology.

• #41 Chordoma | Brain Tumor Center | Stanford Medicine

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

  When the entire tumor is successfully removed with surgery, patients statistically live longer and the tumor is less likely to recur, or grow back. […] Chordomas have a high local recurrence rate, meaning they can come back after successful treatment usually in the same place as the first tumor. […] Even in cases where all visible tumor has been removed, it is still likely that microscopic chordoma cells were left behind during surgery. Therefore, radiation therapy is generally recommended after surgery to prevent any remaining cells from re-growing or spreading to other parts of the body. […] Radiation treatments will typically begin within three to six months after surgery. […] Chemotherapy uses drugs to stop cancer cells from growing. Modern chemotherapy and immunotherapy for chordomas is under investigation at Stanford Cancer Center.

• #42 Chordomas | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/bone-cancer/types/chordomas

  Your treatment depends on where the chordoma is and how big it is. It’s important your chordoma is treated as soon as possible. This helps reduce the chance of it coming back (recurring). […] You might have an operation to remove your tumour. Or you might have surgery to remove as much of the tumour as possible. This is called debulking surgery. […] You might have a type of radiotherapy called external radiotherapy. It directs radiation beams at the cancer cells to destroy it from outside of the body. […] After surgery some people go onto have radiotherapy. The aim of radiotherapy after surgery is to kill off any cancer cells that have been left behind. This is to lower the risk of the chordoma coming back. […] If you can’t have surgery, you might have external radiotherapy as your main treatment. The aim is to shrink the tumour and relieve any symptoms you might be getting. […] Treatment for local recurrence may include one of more of the following: surgery, radiotherapy, a cancer drug such as imatinib, treatment using the extreme cold to destroy cancer cells (cryotherapy), radiofrequency ablation (RFA).

• #43 Chordoma: The Quest for Better Treatment Options

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5315072/

  Chordoma is an extremely rare cancer, with an incidence of about one case per million persons per year in the USA and Europe (about 300 and 450 cases per year, respectively). […] The primary treatment for chordoma is surgical excision with wide local margins, when possible. Radiotherapy also plays a significant role in the adjuvant setting and when surgery is not possible. […] Unfortunately, in the advanced and/or metastatic setting, where the role of surgery and/or radiation is less clear, treatment options are very limited. […] Surgery is the mainstay of treatment for primary and/or recurrent chordoma when feasible, and the outcome of surgery is closely related to the expected outcome for a given patient. […] Radiotherapy also plays a key role in the management of patients with localized chordoma, particularly in the adjuvant setting after full resection or subtotal resection and as the primary treatment in unresectable disease.

• #44 Chordoma Guidelines: Guidelines Summary

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

  Systemic therapy options may include chemotherapy, targeted therapy, and immunotherapy. […] Medical therapy options are limited, and no drugs are approved for treatment of advanced chordoma. […] Several targeted therapies have shown modest activity in patients with recurrent disease. […] Imatinib and sorafenib are the agents with greatest evidence of efficacy in advanced chordoma and represent reasonable palliative treatment options to slow disease progression or alleviate symptoms. […] Cytotoxic chemotherapy is generally inactive, and evidence is insufficient to recommend it. […] Palliative care should be considered as part of the active management of all patients and should include pain and symptom control, discussion about a patients concerns and wishes, conversation about advanced directives, and evaluation of patient and family psychosocial needs. […] Both NCCN and ESMO guidelines state that biopsy is required to confirm the diagnosis prior to any surgical procedure and should be performed at a specialized center that will provide definitive treatment.

• #45

  https://link.springer.com/article/10.1007/s40487-016-0016-0

  Patients with residual disease after surgery or unresectable disease at the time of diagnosis should consider high-dose radiotherapy at a center with expertise in delivering such doses for chordoma. […] To date, there has been no randomized, controlled trial of a therapeutic agent in locally advanced and/or metastatic chordoma. […] Despite rational selection of agents based on potential therapeutic targets identified by extensive preclinical work, radiographic response rates have been very low. Thus, the impact of these agents cannot be fully assessed, which has precluded their approval for use in advanced chordoma by the FDA or other regulatory agencies. […] The initial management of chordoma is fairly well defined. Patients should have maximal tumor debulking and adjuvant high-dose radiotherapy when possible. When surgery is not possible, high-dose radiotherapy achieves reasonable disease control in most cases.

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