Materiały źródłowe

• #1 Ewing Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/bone/hp/ewing-treatment-pdq

  Dramatic improvements in survival have been achieved for children and adolescents with cancer. For Ewing sarcoma, the 5-year survival rate has increased from 59% to a range of 80% to 85% for children younger than 15 years and from 20% to 69% for adolescents aged 15 to 19 years. […] Most of these tumors have been treated with regimens designed for Ewing sarcoma, and the consensus was that they were often included in clinical trials for the treatment of Ewing sarcoma, sometimes referred to as translocation-negative Ewing sarcoma. It is now agreed that these tumors are sufficiently different from Ewing sarcoma and that they should be stratified and analyzed separately from Ewing sarcoma, even if they are treated with similar therapy. […] The successful treatment of patients with Ewing sarcoma requires systemic chemotherapy in conjunction with surgery and/or radiation therapy for local tumor control. In general, patients receive chemotherapy before instituting local-control measures.

• #2 Emerging therapies in Ewing sarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11155282/

  There is an unmet need to improve outcomes for patients for Ewing sarcoma, a rare, aggressive sarcoma with a peak incidence in adolescents and young adults (AYA). Current therapy at diagnosis involves multiagent chemotherapy and local therapy, but despite intensification of treatment, those with metastases at diagnosis and recurrent disease have poor outcomes. […] Improved understanding of Ewing sarcoma biology has identified novel targets with promising activity in Ewing sarcoma patients, including tyrosine kinase inhibitors that are now undergoing evaluation as combination and maintenance therapy. Other emerging therapies include those that target the EWSR1::FLI1 fusion oncoprotein, and act on DNA damage, cell cycle and apoptotic pathways. Immunotherapeutic approaches, particularly CAR-T-cell therapy directed at GD2, also hold promise.

• #2 Emerging therapies in Ewing sarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11155282/

  Treatment of Ewing sarcoma requires multimodality therapy that involves intensive multiagent chemotherapy and local therapy (surgery, radiation therapy, or both). […] The current European collaborative trials, Inter-Ewing-1 and iEuroEwing are evaluating the addition of cyclophosphamide and vinorelbine to VDC/IE as maintenance chemotherapy in this setting but with flexible designs that allow evaluation of additional agents as described forthwith. […] Patients with recurrent or refractory disease have poor outcomes with a median overall survival of around 12 months. […] The rEECur phase II/III adaptive multiarm trial is the first to compare regimens in a randomized design. […] Aberrant angiogenesis is crucial for Ewing sarcoma growth and metastasis and preclinical data supports this target in Ewing sarcoma.

• #3 Chemotherapy for Ewing Tumors | American Cancer Society

  https://www.cancer.org/cancer/types/ewing-tumor/treating/chemotherapy.html

  Chemotherapy (chemo) is the use of drugs to treat cancer. The drugs are usually given into a vein (IV) and can affect cancer cells in all parts of the body, which makes this treatment useful for cancers that are likely to have spread. […] Chemo is an important part of treatment for almost all patients with Ewing tumors (Ewing sarcomas). It is typically the first treatment given, followed by surgery and/or radiation therapy. More chemo is often given after surgery and/or radiation. […] Most patients will get chemo for at least 9 weeks before surgery or radiation, and then will get more chemo afterward as well. Usually a total of about 14 to 15 cycles of chemo are given, which can take from about 6 months to close to a year to complete, depending on the schedule. If the tumor has spread to other parts of the body, these same drugs may be given at higher doses. […] Some chemo drugs can affect your (childs) ability to have children (fertility) later in life. Talk to the cancer care team about the possible effects of treatment on fertility, and ask if there are options for preserving fertility, such as sperm banking or ovarian tissue banking.

• #4 Treatment of Ewing Tumors by Stage | American Cancer Society | American Cancer Society

  https://www.cancer.org/cancer/types/ewing-tumor/treating/by-stage.html

  Treatment of a Ewing tumor (Ewing sarcoma) is based mainly on where it is in the body and how far it has spread when it’s first found. […] A localized Ewing tumor is one that still appears to be confined to the area where it started (and maybe also to nearby tissues such as muscle or tendons), based on imaging test and biopsy results. […] If these people don’t get chemotherapy (chemo) as part of their treatment, these small areas of cancer cells would eventually become larger tumors. This is why chemo, which can reach all parts of the body, is an important part of treatment for localized Ewing tumors. […] Once the Ewing tumor has been diagnosed and staged, the first treatment is chemotherapy. It’s called neoadjuvant chemotherapy because it’s given before any surgery or radiation therapy.

• #5 Ewing Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/bone/hp/ewing-treatment-pdq

  Multidrug chemotherapy for Ewing sarcoma always includes vincristine, doxorubicin, ifosfamide, and etoposide. […] The mode of administration and dose intensity of cyclophosphamide within courses differs markedly between protocols. […] For patients with metastatic Ewing sarcoma, standard treatment that uses alternating cycles of vincristine/doxorubicin/cyclophosphamide and ifosfamide/etoposide (VDC/IE) combined with adequate local-control measures applied to both primary and metastatic sites often results in complete or partial responses. However, the overall cure rate is 20%. […] The following chemotherapy regimens have not shown benefit: In the Intergroup Ewing Sarcoma Study, patients with metastatic disease showed no benefit from the addition of ifosfamide and etoposide to a standard regimen of vincristine, doxorubicin, cyclophosphamide, and dactinomycin.

• #5 Ewing Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/bone/hp/ewing-treatment-pdq

  The role of high-dose therapy with busulfan-melphalan (BuMel) followed by stem cell rescue was investigated in the prospective randomized EURO-EWING-INTERGROUP-EE99 trial. […] A retrospective data analysis of 120 patients with multifocal metastatic Ewing sarcoma, patients who received local treatment to both the primary tumor and metastases had better outcomes than patients who received local treatment to the primary tumor only or with no local treatment. […] Radiation therapy may be used (similar to first-line strategies) for patients who relapsed after the beginning of front-line therapy and/or who present only with relapsed pulmonary metastases.

• #6 Treatment of Ewing Tumors by Stage | American Cancer Society | American Cancer Society

  https://www.cancer.org/cancer/types/ewing-tumor/treating/by-stage.html

  In the United States, patients are given a chemo regimen known as VDC/IE (or VAC/IE), which is a combination of vincristine, doxorubicin (Adriamycin), and cyclophosphamide, alternated with ifosfamide and etoposide, although other combinations of the same drugs are also effective. […] If cancer cells are found at or near the edges of the surgery specimen (meaning cancer cells may have been left behind), radiation therapy and chemotherapy (for several months) are used. […] If there are no cancer cells at or near the edges of the surgery specimen, chemotherapy can be used without radiation therapy. […] If surgery is not an option after the initial chemotherapy (because of the tumor location or some other reason), but the tumor is not growing, radiation therapy (along with chemotherapy) is usually the next treatment given.

• #6 Treatment of Ewing Tumors by Stage | American Cancer Society | American Cancer Society

  https://www.cancer.org/cancer/types/ewing-tumor/treating/by-stage.html

  If the Ewing tumor continues to grow despite the initial chemotherapy, a second type of chemotherapy (using different drugs) may be tried. […] Treating metastatic disease is similar in many ways to treating localized disease. Chemotherapy is the first treatment, but it often requires a more intense regimen than would be used if the cancer was localized. […] If the cancer remains in only a few small areas after chemo, the main (primary) tumor and all known areas of metastases may be removed with surgery at this point. […] Chemotherapy, surgery, radiation therapy, or some combination of these may be used to treat recurrent tumors, depending on the situation. […] Doctors are also studying the usefulness of high-dose chemotherapy followed by a stem cell transplant, as well as targeted drugs and immune therapies.

• #7 Metastatic Ewing’s Sarcoma | Nebraska Hematology Oncology – Cancer Care Treatment Blood Disorders Clinical Trials Lincoln Nebraska (NE)

  https://www.yourcancercare.com/types-of-cancer/sarcoma/ewings-sarcoma/metastatic-ewings-sarcoma

  The multi-modality approach to treatment for metastatic Ewings sarcoma requires that patients be treated by a multi-disciplinary team consisting of the primary care physician, an orthopedic surgeon experienced in bone tumors, a pathologist, radiation oncologists, pediatric oncologists, rehabilitation specialists, pediatric nurse specialists, social workers, and others. […] Patients with metastatic Ewings sarcoma have disease that has spread to various parts of the body, necessitating systemic (whole-body) therapy. In many instances it may also be necessary to treat the primary tumor with surgery (with or without radiation therapy) for optimal results. […] The current standard chemotherapy regimen is a combination of drugs which includes: Adriamycin (doxorubicin), Oncovin (vincristine), Cytoxan (cyclophosphamide) and Actinomycin D (dactinomycin).

• #8 Ewing Sarcoma Treatment & Management: Approach Considerations, Treatment Team, Consultations

  https://emedicine.medscape.com/article/990378-treatment

  Treatment lasts 6-9 months and consists of alternating courses of 2 chemotherapeutic regimens: (1) vincristine, doxorubicin, and cyclophosphamide and (2) ifosfamide and etoposide. […] Chemotherapy can be administered on an inpatient or outpatient basis, depending on patient tolerance and proximity to the hospital. […] Chemotherapy interval compression from the standard 3-week therapy to 2 weeks improves outcomes for localized Ewing sarcoma, without increased toxicity, according to a study by the Children’s Oncology Group. […] According to the investigators, patients who received interval compression chemotherapy every 2 weeks had a 5-year event-free survival rate of 73%, compared with 65% in patients who received chemotherapy every 3 weeks. […] An open study within the Childrens Oncology Group (AEWS1031) evaluated the efficacy of adding vincristine, topotecan, and cyclophosphamide to the interval compressed 5-drug backbone for patients with nonmetastatic Ewing sarcoma (NCT01231906).

• #8 Ewing Sarcoma Treatment & Management: Approach Considerations, Treatment Team, Consultations

  https://emedicine.medscape.com/article/990378-treatment

  The addition of vincristine, topotecan, and cyclophosphamide to interval compressed chemotherapy did not improve survival outcomes. […] Management of the primary tumor site is critical to long-term cure. […] Definitive surgical margins are desirable (eg, removal of fibula, limb salvage with extensive margins). […] In the absence of a minimally morbid surgical procedure, local control may be achieved with radiation therapy. […] There are no standardized second-line treatment plans for relapsed or refractory Ewing sarcoma. […] Chemotherapy combinations such as vincristine/irinotecan/temozolomide or gemcitabine/docetaxel have been considered in recurrent Ewing sarcoma. […] Identification and development of targeted therapies for Ewing sarcoma are underway in early clinical trial settings.

• #8 Ewing Sarcoma Treatment & Management: Approach Considerations, Treatment Team, Consultations

  https://emedicine.medscape.com/article/990378-treatment

  Most patients require red blood cell (RBC) and platelet support during therapy. […] A full physical examination is required before each cycle of chemotherapy and any time suspicious signs or symptoms arise between cycles. […] Primary and metastatic sites are evaluated approximately every 10-12 weeks during therapy and every 3-4 months during the first year after therapy. […] Reevaluations are spaced out gradually for 5-6 years after the completion of therapy. […] After 5 years of disease-free remission, no further scanning is indicated; however, the patient should have annual follow-up visits to monitor the function of the primary site and late effects of therapy, preferably in a late-effects clinical setting.

• #9 Ewing Sarcoma | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/ewing-sarcoma

  Most of the time, there are two components in treating children with newly diagnosed Ewing sarcoma: local control, which involves treating the tumor itself, usually through surgery, radiation, or both; and systemic therapy, which treats any tumor cells throughout the body, usually through chemotherapy. Ewing sarcoma can usually only be cured by using local control together with systemic therapy. Your child’s treatment may include (alone or in combination): […] Chemotherapy is usually given over a period of a few days every two weeks for about 12 weeks before it is time for local control of the main tumor. After surgery and/or radiation for local control, chemotherapy is continued for another four to six months in order to eliminate all the cancerous cells in the body. We give chemotherapy cycles every two weeks in Ewing sarcoma because studies show that this schedule (called interval compression) improves outcomes for children with localized Ewing sarcoma.

• #9 Ewing Sarcoma | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/ewing-sarcoma

  Local control of Ewing sarcoma is achieved with surgery and/or radiation therapy. Surgery may be performed after the first 12 weeks of chemotherapy to remove any parts of the tumor that remain. Several forms of surgical intervention may be considered for your child depending on the size and location of the tumor. […] Limb-salvage surgery helps preserve the limb by removing the tumor and wide margins of healthy tissue surrounding the tumor. […] Amputation may be necessary if the tumor cannot be completely removed (for example, if it involves the nerves and blood vessels) or if limb function cannot be preserved through limb-salvage surgery. […] Rotationplasty is a partial amputation that preserves a cancer-free lower leg, attaches it to the thighbone, and uses the ankle as a knee joint especially useful in very young children where limb length can be an issue.

• #10 Ewing sarcoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/ewing-sarcoma/diagnosis-treatment/drc-20351072

  Ewing sarcoma treatment most often includes chemotherapy and surgery. Which treatment you have first will depend on your situation. Other treatment options might include radiation therapy and targeted therapy. […] Chemotherapy is sometimes used as the first treatment for Ewing sarcoma. The medicines may shrink the cancer. That makes it easier to remove the cancer with surgery or target with radiation therapy. […] After surgery or radiation therapy, chemotherapy treatments might be used to kill any cancer cells that might remain. […] The goal of surgery is to remove all the cancer cells. Surgery for Ewing sarcoma might mean removing a small portion of bone and some surrounding tissue. Rarely, it might mean removing the affected arm or leg. […] Radiation therapy might be suggested after surgery to kill cancer cells that remain. Radiation therapy might be used instead of surgery if an operation is not possible or if it is likely to hurt nearby organs.

• #10 Ewing sarcoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/ewing-sarcoma/diagnosis-treatment/drc-20351072

  For advanced Ewing sarcoma, radiation therapy can slow the growth of the cancer and help relieve pain. […] Targeted therapy for cancer is a treatment that uses medicines that attack specific ways that cancer cells can grow. […] Clinical trials are studies of new treatments. These studies provide a chance to try the latest treatments. The risk of side effects might not be known.

• #11

  https://link.springer.com/article/10.1007/s12094-024-03602-5

  Recommended local treatment options include wide excision, definitive RT, or, in selected cases, amputation. […] Data from retrospective analyses suggest that surgery yields better local control and survival than definitive RT in patients with localized disease. […] Since options have not been directly compared in a randomized trial, the choice of local control treatments should be individualized, as it depends on tumor location, and size, response to CTh, anticipated morbidity, the patients age, and patient preferences. […] RT as definitive local treatment is an effective local control option for patients for whom function-preserving surgery is not possible because of tumor location or extent, and for patients with unresectable primary tumors despite induction CTh. […] Although patients with metastasis on diagnosis have a significantly poorer prognosis (5-year OS 15-40%), treatment must be administered with curative intent, first, because it is difficult to predict which patients can be cured, and second, because treatment may relieve pain and prolong PFS. […] Treatment of a local relapse depends on the time of recurrence, the association with distant metastases, and also resectability using the same criteria as for initial presentation. […] Prognosis for ES following systemic relapse is poor, with 5-year OS reported as 8-30%.

• #12 5 Innovative Ewing’s Sarcoma Treatment Options | MD Anderson Cancer Center

  https://www.mdanderson.org/cancer-types/ewings-sarcoma/ewings-sarcoma-treatment.html

  Because Ewing’s sarcoma can be found in a number of places throughout the body, several types of surgery may be used to treat it. […] Surgery to treat Ewing’s sarcoma attempts to remove as much of the tumor as possible without harming surrounding areas. […] At Children’s Cancer Hospital, our specialized orthopedic surgeons are often able to avoid amputation when the tumor is in an arm or leg. […] Radiation therapy (also called radiotherapy) uses high-energy beams to destroy cancer cells. […] Proton therapy delivers high radiation doses directly into the tumor, sparing nearby healthy tissue and vital organs. […] Ewing’s sarcoma is treated in our Children’s Cancer Hospital, Sarcoma and Orthopaedic Center and our Proton Therapy Center.

• #13 Ewing sarcoma | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/ewing-sarcoma

  Surgery for Ewing sarcoma has two goals: To remove the tumor and to restore function at the site of the tumor. […] Most children with Ewing sarcoma of an extremity (arm or leg) can be treated with limb-sparing (also known as limb-salvage) and reconstructive surgery. […] Depending on your child’s individual situation, radiation therapy may be recommended. The radiation destroys or damages cancer cells so they cannot grow or spread. The goal of radiation treatment is to damage as many cancer cells as possible, while limiting harm to nearby healthy cells. […] Regular monitoring by trained clinicians is strongly encouraged to check for possible recurrence of the growth and manage any side effects of treatment. Ewing sarcoma can reoccur even after being successfully treated so it is important for your child continue to be seen regularly by one of the physicians at the Bone and Soft Tissue Tumor Program.

• #14 Ewing sarcoma | NHS inform

  https://www.nhsinform.scot/illnesses-and-conditions/cancer/cancer-types-in-adults/ewing-sarcoma/

  Limb-sparing surgery involves removing the area of bone on an arm or leg where the cancer is growing. A piece of metal (prosthesis) or a bone graft will be used to replace the piece of bone that is removed. […] In some cases, your whole arm or leg may need to be removed (amputation). This may be the only option to prevent the cancer returning if it’s spread into the tissues surrounding the bone. […] For more information about the types of treatment for bone cancer, visit the Cancer Research UK website.

• #14 Ewing sarcoma | NHS inform

  https://www.nhsinform.scot/illnesses-and-conditions/cancer/cancer-types-in-adults/ewing-sarcoma/

  Ewing sarcoma is usually treated with a combination of chemotherapy, surgery and radiotherapy. […] Chemotherapy may be used before surgery to shrink the tumour and make it easier to remove. […] Ewing sarcoma also responds well to radiotherapy. It can be used to shrink the tumour after chemotherapy, and before or after surgery to lower the risk of the cancer returning. […] In some cases, surgically removing Ewing sarcoma can be difficult for example, if it develops in the pelvis. In this case, radiotherapy may be used as the main treatment. […] If surgery is recommended, the type you’ll have will depend on the size of the tumour, where it is in your body, and whether it has grown into the tissues surrounding the bone. […] You may have surgery to remove part or all of the bone affected by cancer. This type of surgery is known as resection.

• #15 Ewing Sarcoma—Diagnosis, Treatment, Clinical Challenges and Future Perspectives

  https://www.mdpi.com/2077-0383/10/8/1685

  Definitive surgery typically follows an initial period of neoadjuvant chemotherapy, unless emergency surgical procedures are mandatory at diagnosis, e.g., in case of spinal cord compression. […] The principles and techniques of surgical resection and reconstruction in primary malignant bone tumors have been defined by Enneking. […] The aim of the tumor resection is a wide resection according to Enneking. […] The majority of patients with surgical local treatment requires bone reconstructive surgery. […] Primary amputation is rarely needed in EwS due to both tumor shrinkage during neoadjuvant chemotherapy and the availability of definitive RT as an acceptable alternative to surgery. […] The surgical margin status is a reliable indicator of tumor left in the patient. […] An adequate surgical margin is one in which there is no viable tumor at the edge of the resection specimen that can be obtained by wide tumor resection, i.e., sufficient safety distance to the reactive zone of the tumor.

• #15 Ewing Sarcoma—Diagnosis, Treatment, Clinical Challenges and Future Perspectives

  https://www.mdpi.com/2077-0383/10/8/1685

  Additional RT following surgery is recommended in any case of positive margins, while European protocols also recommend additional RT for narrow margins and/or poor histological response. […] The radiosensitivity of EwS has been recognized since its first description by James Ewing. […] RT as an active modality for assuring local control is used as definitive RT in inoperable tumors or in combination with surgery, either pre- or postoperatively. […] Ifosfamide was established as an active agent against EwS in the 1980s. […] Subsequent cooperative group trials incorporated IE into VACA-based chemotherapy backbones. […] The five-year OS in these studies improved to 60–70% for patients with non-metastatic disease. […] The combination of gemcitabine and docetaxel has been evaluated in multiple trials, with response rates of 14% to lower doses of each agent, and 66% in a trial that utilized higher doses.

• #16

  https://www.orthobullets.com/pathology/8047/ewings-sarcoma

  Adjuvant radiation is not necessary if margins are adequate and there is good response to chemotherapy. […] Outcomes show a 5-year survival rate of 39% and 82% in those with and without metastases at diagnosis, respectively. […] The standard regimen for chemotherapy includes vincristine, doxorubicin, cyclophosphamide, with some studies suggesting the addition of ifosfamide and etoposide improves survival and decreases failure rates. […] Radiation therapy should include pretreatment tumor volume plus a 2-3 cm margin, with a dose of 56-60 Gy. […] Limb salvage must obtain negative surgical margins, as 5-year survival improves by over 10% with negative margins. […] If positive margins are identified, re-resection should be performed +/- radiation therapy.

• #17 Ewing sarcoma – Macmillan Cancer Support | Macmillan Cancer Support

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

  Chemotherapy uses anti-cancer (cytotoxic) drugs to destroy cancer cells. It is often given before surgery to shrink the tumour and improve the results of the operation. […] Radiotherapy uses high-energy rays to destroy the cancer cells, while doing as little harm as possible to normal cells. Ewing sarcoma responds very well to radiotherapy. It may be used with surgery, either before or after an operation. […] You may be offered some treatments as part of a clinical trial.

• #17 Ewing sarcoma – Macmillan Cancer Support | Macmillan Cancer Support

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

  Ewing sarcoma is rare, so it is treated by a team of doctors and other healthcare professionals at a hospital with a specialist sarcoma treatment centre. […] Your test results are 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 called a multidisciplinary team (MDT) will meet. They will discuss the best possible treatment for you. […] After the MDT meeting, your cancer doctor or specialist nurse will explain the treatment options and possible side effects to you. They will also talk to you about things to consider when making a decision. […] The main treatments for Ewing sarcoma are surgery, radiotherapy and chemotherapy. You usually have a combination of these treatments: […] If possible, surgery is used to remove Ewing sarcoma. Surgery aims to remove the affected area of bone. Some people might have reconstructive surgery at the same time as the operation.

• #18 The Role of Radiation Therapy for Ewing’s Sarcoma

  https://www.texasoncology.com/types-of-cancer/sarcoma/ewings-sarcoma/localized-ewings-sarcoma/the-role-of-radiation-therapy-for-ewings-sarcoma

  Radiation therapy is a local treatment used to eliminate cancer in a specific area. […] Optimal treatment of patients with Ewings sarcoma often requires more than one therapeutic approach. […] Ewings sarcoma is relatively sensitive to radiation, and conventional radiation therapy plays a major role in treatment. […] External beam radiation therapy is most often administered in conjunction with chemotherapy. […] The most common approach for the treatment of localized Ewings sarcoma is to remove as much tumor as possible surgically, deliver local radiation to eradicate microscopic tumor not removed by surgery, and administer systemic combination chemotherapy to eradicate micrometastases. […] Patients who have inoperable tumors or tumors in sites not suitable for surgery are treated with radiation therapy and chemotherapy.

• #19 Ewing sarcoma | Bone Cancer Research Trust

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

  Radiotherapy may be used: before surgery to shrink the cancer, after surgery to destroy any cancer cells that remain in the area after the operation, instead of surgery if the tumour cannot be removed by surgery, in combination with surgery and chemo if the tumour cannot be fully removed or if full removal would limit how well the body works. […] If the Ewing sarcoma is in a limb (arm or leg) the medical team will do their best to save the limb by doing 'limb-sparing surgery’. […] Sometimes, it is not possible to remove a bone tumour from an arm or leg (limb). In this case, limb-sparing surgery will not work and an 'amputation’ may be needed instead. […] If the cancer does return, it may feel like the bottom has fallen out of your world, but there are treatments that you can be given.

• #20 Ewing Sarcoma Treatment | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/treatment/childhood-cancer/solid-tumors/ewing-sarcoma.html

  St. Jude provides the highest quality of care for patients with Ewing sarcoma: […] St. Jude researchers identified important drug combinations that target the Ewing sarcoma tumor cells inability to repair DNA. If the tumor cell cannot repair DNA, then it dies. […] St. Jude researchers are conducting a clinical trial called ONITT that targets the Ewing sarcoma cells vulnerability. […] Radiation treatments, such as intensity-modulated radiation therapy (IMRT) and proton beam radiation therapy, deliver focused radiation to the tumor. […] St. Jude has a team of experts who pioneered limb-salvage procedures (surgery) in children with bone tumors, such as Ewing sarcoma. […] Imaging techniques allow St. Jude to better define the effects of treatment in children with Ewing sarcoma.

• #21 Localized Ewing’s Sarcoma

  https://www.texasoncology.com/types-of-cancer/sarcoma/ewings-sarcoma/localized-ewings-sarcoma

  Localized Ewings sarcoma affects only the bone in which it developed and the tissues next to the bone, such as muscle and tendon. […] The following is a general overview of treatment for localized Ewings sarcoma. Treatment may consist of surgery, radiation therapy, chemotherapy, or all three modalities. […] The multi-modality approach to treatment for Ewings sarcoma requires that patients be treated by a multi-disciplinary team consisting of the primary care physician, an orthopedic surgeon experienced in bone tumors, a pathologist, radiation oncologists, pediatric oncologists, rehabilitation specialists, pediatric nurse specialists, social workers, and others. […] Effective treatment of localized Ewings sarcoma requires both local and systemic therapy. Local therapy consists of surgery alone, radiation therapy alone or both radiation and surgery.

• #21 Localized Ewing’s Sarcoma

  https://www.texasoncology.com/types-of-cancer/sarcoma/ewings-sarcoma/localized-ewings-sarcoma

  The delivery of systemic therapy in addition to local treatment is necessary to maximize a patients chance of cure. […] The ultimate goal of surgery for localized Ewings sarcoma is to remove the cancer without amputation. […] Radiation Therapy is used to prevent local recurrences following complete or incomplete surgery. […] The main improvement in the treatment of localized Ewings sarcoma over the past 30 years has been the advent of combination chemotherapy. […] The most common approach for the treatment of localized Ewings sarcoma is to remove as much tumor as possible surgically, deliver local radiation to eradicate microscopic tumor not removed by surgery and to administer systemic combination chemotherapy to eradicate micrometastases. […] Patients with Ewings sarcoma who have inoperable tumors or tumors in sites not suitable for surgery are generally treated with radiation therapy and chemotherapy.

• #22 Treatments for childhood Ewing sarcoma of the bone | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/bone-childhood/treatment/ewing-sarcoma

  A child diagnosed with Ewing sarcoma of the bone will have a treatment plan created for them by their healthcare team. It will be based on important information about the cancer and about the child. When deciding which treatments to offer as part of the plan, the healthcare team will consider the: […] Localized Ewing sarcoma of the bone in children is usually treated with chemotherapy first. It may then be treated with surgery or radiation therapy. […] Chemotherapy uses anticancer (cytotoxic) drugs to destroy cancer cells. It is used first to treat the tumour. When chemotherapy is used before other treatments, it is called neoadjuvant chemotherapy. Neoadjuvant chemotherapy is given for about 12 weeks. […] If the tumour is not growing and it is resectable, it will be removed by surgery. After surgery, more chemotherapy is given and radiation therapy may or may not be given. When chemotherapy is given after surgery it is called adjuvant chemotherapy.

• #22 Treatments for childhood Ewing sarcoma of the bone | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/bone-childhood/treatment/ewing-sarcoma

  If the tumour is not growing but it is not resectable, it is treated with radiation therapy and more chemotherapy. The tumour may then be reassessed to see if surgery is possible. […] If the tumour is still growing, a different combination of chemotherapy drugs may be given. Surgery or radiation therapy may be used to control the growth of the tumour. […] After chemotherapy (and sometimes radiation therapy), surgery is used to remove the tumour. The goal of surgery is to remove the entire tumour. […] Tumours in the arms or legs are treated with limb-sparing surgery, amputation or rotationplasty. […] Radiation therapy uses high-energy rays or particles to destroy cancer cells. It may be used to treat the tumour when surgery is not possible. Sometimes radiation therapy helps to shrink the tumour and make surgery possible.

• #22 Treatments for childhood Ewing sarcoma of the bone | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/bone-childhood/treatment/ewing-sarcoma

  Metastatic Ewing sarcoma of the bone is usually treated with chemotherapy followed by surgery or radiation therapy or both. […] Chemotherapy is the primary treatment for metastatic Ewing sarcoma of the bone. Chemotherapy is given for 12 to 16 weeks. […] Surgery may be used to remove the primary tumour and metastases in other bones or a lung. It is done after chemotherapy (or radiation therapy, in some cases) if the cancer has spread to only a few small areas. […] The treatments offered for recurrent childhood Ewing sarcoma of the bone are based on: […] Recurrent Ewing sarcoma of the bone may be treated with chemotherapy, radiation therapy, high-dose chemotherapy with stem cell rescue or a combination of these treatments. […] High-dose chemotherapy with stem cell rescue may be used in some cases to treat recurrent childhood Ewing sarcoma as part of a clinical trial.

• #23 Childhood Ewing Sarcoma | Dana-Farber Cancer Institute

  https://www.dana-farber.org/cancer-care/types/childhood-ewing-sarcoma

  The five-year survival rate for children with localized Ewing sarcoma is close to 70 to 80 percent. For children with metastatic disease, the five-year survival rate is 20 to 30 percent. […] Our solid tumor treatment team includes experts in childhood cancer and pediatric orthopedic surgery. We work collaboratively to determine the best treatment plan for your child’s unique needs.

• #24 Potential approaches to the treatment of Ewing’s sarcoma | Oncotarget

  https://www.oncotarget.com/article/12566/text/

  This strategy has markedly improved the patient outcome; current 5-year survival rate for patients with localized ES has increased up to 70%. […] Nevertheless, the 5-year survival rate remains less than 20% for patients with metastatic or recurrent tumors. […] Therefore, novel therapies are urgently needed to improve the treatment; targeted therapies and immunotherapies seem most promising. […] RTKs have been targeted also in ES, although little abnormal expression of RTKs has been detected. […] IGF1R targeting is the most studied targeted therapy in ES. […] Both antibodies and small molecule inhibitors disrupting the IGF1R function are in preclinical and clinical stages of development. […] Anti-IGF1R monoclonal antibodies induce responses in a subset of patients with ES. […] Treatment with the combination of IMC-A12 and the mTOR inhibitor temsirolimus exhibited a great response in five out of 17 (29%) ES patients.

• #24 Potential approaches to the treatment of Ewing’s sarcoma | Oncotarget

  https://www.oncotarget.com/article/12566/text/

  In addition to antibodies, the effects of small molecule inhibitors of IGF1R have been investigated in laboratory and preclinical studies. […] Targeting EWS-FLI1 can be achieved by decreasing EWS-FLI1 expression through transcription impairment, by decreasing EWS-FLI1 activity through targeting the transcriptional modulators to which EWS-FLI1 binds, or by targeting genes that are deregulated by EWS-FLI1 expression. […] EWS-FLI1 is a perfect target for treating ES. […] The interaction of EWS-FLI1 with RHA affects pre-mRNA processing, resulting in splicing isoforms involved in oncogenesis. […] EWS-FLI1 knockdown in ES led to widespread epigenetic changes in promoters, enhancers, and super-enhancers; histone H3K27 acetylation was the most affected mark. […] An HDAC inhibitor, romidepsin, reverses EWS-FLI1 mediated histone deacetylation, decreases EWS-FLI1 mRNA and protein levels, inhibits cell proliferation, and induces TRAIL-dependent apoptosis of ES cells.

• #25 Metastatic Ewing’s Sarcoma | Nebraska Hematology Oncology – Cancer Care Treatment Blood Disorders Clinical Trials Lincoln Nebraska (NE)

  https://www.yourcancercare.com/types-of-cancer/sarcoma/ewings-sarcoma/metastatic-ewings-sarcoma

  Ewings sarcoma that has spread from the initially affected bone to one or more sites in the body, distant from the site of origin, is called metastatic. The most common site to which Ewings sarcoma spreads, or metastasizes, is the lungs. Metastatic Ewings is typically difficult to control, though patients with lung metastases have a better prognosis than patients with other distant metastases. […] The following is a general overview of treatment for metastatic Ewings sarcoma. Treatment may consist of surgery, radiation therapy, chemotherapy, or all three modalities. Multi-modality treatment is treatment using two or more techniques; this is increasingly recognized as an important approach for increasing a patients chance of cure or prolonging survival. In some cases, participation in a clinical trial utilizing new, innovative therapies may provide the most promising treatment.

• #25 Metastatic Ewing’s Sarcoma | Nebraska Hematology Oncology – Cancer Care Treatment Blood Disorders Clinical Trials Lincoln Nebraska (NE)

  https://www.yourcancercare.com/types-of-cancer/sarcoma/ewings-sarcoma/metastatic-ewings-sarcoma

  The multi-modality approach to treatment for metastatic Ewings sarcoma requires that patients be treated by a multi-disciplinary team consisting of the primary care physician, an orthopedic surgeon experienced in bone tumors, a pathologist, radiation oncologists, pediatric oncologists, rehabilitation specialists, pediatric nurse specialists, social workers, and others. […] Patients with metastatic Ewings sarcoma have disease that has spread to various parts of the body, necessitating systemic (whole-body) therapy. In many instances it may also be necessary to treat the primary tumor with surgery (with or without radiation therapy) for optimal results. […] The current standard chemotherapy regimen is a combination of drugs which includes: Adriamycin (doxorubicin), Oncovin (vincristine), Cytoxan (cyclophosphamide) and Actinomycin D (dactinomycin).

• #26 Management of Recurrent Ewing Sarcoma: Challenges and Approaches – Oncology Nurse Advisor

  https://www.oncologynurseadvisor.com/features/challenges-and-approaches-to-recurrent-ewing-sarcoma/

  Ewing sarcoma is a high-grade sarcoma arising in bone or soft tissue and occurs most commonly in adolescent and young adult patients. The primary tumor is treated with surgery and/or radiation, depending on the feasibility of resection. […] Because of the high rate of dissemination even in patients with apparently localized disease, patients are also treated with chemotherapy in an effort to eradicate microscopic disease that could lead to distant metastases and death. Three strategies for initial chemotherapy are most commonly used, depending in part on institutional preferences and patient age. […] Therapy is administered for up to 14 total cycles, and the vast majority of patients achieve remission by the completion of treatment. […] Despite this extensive therapy, at least one-fourth of patients with initially localized disease will relapse after completing all planned therapy. […] In addition, there is no standard management for this group of patients, raising many questions about how best to proceed. In this review, we address common management issues and discuss current and upcoming strategies for the treatment of this complex disease.

• #26 Management of Recurrent Ewing Sarcoma: Challenges and Approaches – Oncology Nurse Advisor

  https://www.oncologynurseadvisor.com/features/challenges-and-approaches-to-recurrent-ewing-sarcoma/

  For the approximately 15%20% of patients with isolated local recurrence, further local control measures with either surgery or radiotherapy may improve outcomes and should strongly be considered when feasible. […] In general, chemotherapy is also used for local recurrences, given the systemic nature of relapsed Ewing sarcoma and the expected high likelihood of developing additional sites of recurrence if therapy is directed only to local sites. […] A variety of commercially available chemotherapy regimens have produced responses in patients with recurrent Ewing sarcoma; however, the superiority of one regimen over another has not yet been established. […] The design of the study includes sequential elimination of the regimens that produce the lowest objective response rate, which is the primary endpoint of the Phase II portion of the study. Once two regimens have emerged, they will be compared in the Phase III portion of the study, which uses PFS as the primary endpoint. This data will be immensely helpful in defining a more standard approach to recurrent Ewing sarcoma.

• #26 Management of Recurrent Ewing Sarcoma: Challenges and Approaches – Oncology Nurse Advisor

  https://www.oncologynurseadvisor.com/features/challenges-and-approaches-to-recurrent-ewing-sarcoma/

  Clinical trials of new strategies for treating patients with recurrent Ewing sarcoma will be essential for improving outcomes. […] Enrollment on clinical trials should strongly be considered when feasible, as a variety of mechanistically novel Phase I to Phase III studies are currently underway and represent the best way to better understand which treatments may be beneficial in the future.

• #27 Ewing Sarcoma Treatment – NCI

  https://www.cancer.gov/types/bone/patient/ewing-treatment-pdq

  Surgery is usually done to remove cancer that is left after chemotherapy or radiation therapy. […] Stem cell transplant is a treatment to replace the blood-forming cells. […] Treatment of recurrent Ewing sarcoma may include: combination chemotherapy, surgery, radiation therapy to bone tumors, as palliative therapy to relieve symptoms and improve quality of life, radiation therapy that may be followed by surgery to remove tumors that have spread to the lungs, stem cell transplant with the child’s own cells.

• #28 Emerging therapies in Ewing sarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11155282/

  TKIs with antiangiogenic activities such as regorafenib and cabozantinib have shown encouraging single-agent results in single-arm and randomized trials versus placebo with 10-26% objective tumour responses and median PFS of 34.5 months in heavily pretreated patients, mostly adults. […] Currently the EEC is testing the combination of lenvatinib with IFOS within the rEECur trial as described above and regorafenib in newly diagnosed patients. […] Several strategies have attempted to directly or indirectly target the EWSR1::FLI1 gene fusion or immediate downstream targets. […] The DNA minor groove-binding alkylating cytotoxic agent trabectedin is highly potent in vitro against Ewing sarcoma cells and other fusion protein-driven sarcomas, with direct modulatory effects on transcriptional targets of EWSR1::FLI1, yet has little to no single agent clinical activity.

• #29

  https://journals.lww.com/co-oncology/fulltext/2024/07000/emerging_therapies_in_ewing_sarcoma.14.aspx

  Aberrant angiogenesis is crucial for Ewing sarcoma growth and metastasis and preclinical data supports this target in Ewing sarcoma. […] TKIs with antiangiogenic activities such as regorafenib and cabozantinib have shown encouraging single-agent results in single-arm and randomized trials versus placebo with 10-26% objective tumour responses and median PFS of 34.5 months in heavily pretreated patients, mostly adults. […] Currently the EEC is testing the combination of lenvatinib with IFOS within the rEECur trial as described above and regorafenib in newly diagnosed patients. […] Several strategies have attempted to directly or indirectly target the EWSR1::FLI1 gene fusion or immediate downstream targets. […] The DNA minor groove-binding alkylating cytotoxic agent trabectedin is highly potent in vitro against Ewing sarcoma cells and other fusion protein-driven sarcomas, with direct modulatory effects on transcriptional targets of EWSR1::FLI1, yet has little to no single agent clinical activity.

• #30

  https://journals.lww.com/co-oncology/fulltext/2024/07000/emerging_therapies_in_ewing_sarcoma.14.aspx

  A phase II study of single agent, Olaparib failed to demonstrate clinical activity; however, strong evidence that PARP inhibitors were also able to selectively potentiate the activity of DNA-damaging cytotoxic agents such as irinotecan and temozolomide in Ewing sarcoma animal models led to several phase I and II combination clinical trials. […] Immunotherapies aim at inducing antitumor immune response by a nontumor-specific immune activity (e.g. immune checkpoint inhibitors) or by targeting tumour-specific features (e.g.: monoclonal antibody, CAR T cells and cancer vaccines). […] The overall disappointing results of these approaches in Ewing sarcoma are at least partially because of the low mutational burden and a resulting lack of high-affinity neoepitopes, the lack of potentially tumour-reactive T cells in the tumour tissue and HLA loss, as well as accumulation of immunosuppressive cells, mainly macrophages, in the tumour microenvironment. […] Development of novel targets remains a challenge in Ewing sarcoma, but improved understanding of biology and the immune microenvironment are leading to more targeted approaches with promising preliminary data in early phase clinical trials.

• #31 How is Ewing’s sarcoma treated? | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/progress-in-ewings-sarcoma-treatment.h00-159460845.html

  Variations of this combination therapy are used for adults, as well, but often adults cannot tolerate a full year of the same intensive treatment given to children. This may partially explain why adult patients with Ewings sarcoma have lower rates of cure than younger patients. Therefore, our researchers are trying to find tolerable and effective treatments for adult patients with Ewings sarcoma. […] In our ongoing clinical trial including adult and pediatric patients with metastatic, relapsed or refractory Ewings sarcoma, we are administering the standard chemotherapy drug vincristine in combination with TK216, a novel inhibitor of ETS proteins. […] Overall, almost half of the patients treated with TK216 experienced clinical benefits either complete response or stable disease and two patients are now in complete remission with no evidence of recurrence for over one and two years. The drug is well-tolerated, with common side effects being fatigue and low red or white blood cell counts. We believe combination therapy with TK216 is a promising option for the subset of patients with metastatic and relapsed Ewings sarcoma.

• #32 Emerging therapies in Ewing sarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11155282/

  The overall disappointing results of these approaches in Ewing sarcoma are at least partially because of the low mutational burden and a resulting lack of high-affinity neoepitopes, the lack of potentially tumour-reactive T cells in the tumour tissue and HLA loss, as well as accumulation of immunosuppressive cells, mainly macrophages, in the tumour microenvironment.

• #33 Role of immunotherapy in Ewing sarcoma | Journal for ImmunoTherapy of Cancer

  https://jitc.bmj.com/content/8/2/e000653

  Ewing sarcoma (ES) is thought to arise from mesenchymal stem cells and is the second most common bone sarcoma in pediatric patients and young adults. Given the dismal overall outcomes and very intensive therapies used, there is an urgent need to explore and develop alternative treatment modalities including immunotherapies. […] In summary, we provide state-of-the-art science in ES tumor immunology and immunotherapy, with rationale and recommendations for future therapeutics development. […] Only 25% of patients with metastatic/recurrent classic ES can be cured by currently available multimodal treatments that include systemic chemotherapy combined with local control either through surgery or radiation. […] There has been some promise with strategies targeted to inactivate or reduce the expression or function of the EWSFLI1 oncoprotein; some of these approaches include inhibitory oligonucleotides and small-molecule inhibitors that can disrupt its transcriptional complex.

• #34 Role of immunotherapy in Ewing sarcoma | Journal for ImmunoTherapy of Cancer

  https://jitc.bmj.com/content/8/2/e000653

  The identification of appropriate tumor antigens is a prerequisite for any immunotherapeutic approach. […] In ES, low or negative HLA class I surface expression theoretically prevents the recognition of intracellular antigens by tumor-specific cytotoxic T lymphocytes (CTL). […] Immunization of patients using peptides, full-length proteins, or tumor cell lysates with or without certain adjuvants is potentially able to induce T cell responses against ES-associated antigens. […] A number of clinical trials have been performed investigating different types of vaccines and adjuvants in patients with ES. […] Oncolytic viruses are used to immunologically target cancer cells based on their: (1) proinflammatory characteristics and (2) their cytolytic properties potentially leading to the release of target antigens and the subsequent induction of antitumor immunity. […] In pediatrics, there have been a few clinical trials using different strains of oncolytic viruses in patients with solid tumors, that have included patients with ES and have demonstrated that intratumoral administration of oncolytic viruses is safe in children; however, objective responses were not observed in these small cohorts.

• #35 Potential approaches to the treatment of Ewing’s sarcoma | Oncotarget

  https://www.oncotarget.com/article/12566/text/

  Cancer immunotherapy aims to establish potent and effective antitumor immune control. […] Immunotherapies for ES include immune cell-based immunotherapies, cancer vaccines, and monoclonal antibodies. […] T cells usually target only cells expressing antigenic sequences presented to the T cell receptor via MHC. […] The abnormal tumor-immune cell interactions in ES may prevent antitumor immunity. […] Therefore, improvement of antigen processing and interactions between ES and T cells has been a focus of ES immunotherapies. […] The transcription factor PAX3 is expressed in ES and functions as a general tumor associated antigen. […] CD99 engagement using anti-CD99 antibody induces massive apoptosis of ES cells through caspase-independent mechanisms and reduces their malignant potential.

• #36 Role of immunotherapy in Ewing sarcoma | Journal for ImmunoTherapy of Cancer

  https://jitc.bmj.com/content/8/2/e000653

  In conclusion, given the dismal outcomes and very intensive therapies used upfront in the treatment of ES, there is a need to explore other treatment modalities in order to attempt to increase response and survival rates and to decrease the toxicities associated with currently available treatments. In this review article, we will focus on immunotherapeutic approaches for classical ES. […] Cellular immunotherapies using engineered T cells have shown impressive clinical activity in hematologic cancers such as B cell malignancies. […] Unfortunately, it has proven difficult to translate these successes to other types of cancer, particularly solid tumors. […] Immune checkpoint inhibitors, such as monoclonal antibodies directed against PD-1 or PD-L1, have shown clinical efficacy in a variety of solid tumors. […] However, a clinical trial investigating PD-1 checkpoint inhibitor pembrolizumab in adults with ES did not result in significant clinical activity.

• #37 UCSF Ewing Sarcoma Clinical Trials for 2025 — San Francisco Bay Area

  https://clinicaltrials.ucsf.edu/ewing-sarcoma

  Ewing sarcoma is a type of cancer that starts in bones or soft tissue. […] A pilot pharmacokinetic trial to determine the safety and efficacy of a flavored, orally administered irinotecan VAL-413 (Orotecan) given with temozolomide for treatment of recurrent pediatric solid tumors including but not limited to neuroblastoma, rhabdomyosarcoma, Ewing sarcoma, hepatoblastoma and medulloblastoma. […] The purpose of this study is to better understand how safe and effective the drug cabozantinib in combination with high-dose ifosfamide is in the treatment of children and adults with relapsed/refractory Ewing sarcoma and osteosarcoma. […] This trial examined the outcome benefit to patients of adding a new chemotherapy drug combination to the established treatment approach for patients with extracranial Ewing sarcoma, that had not spread from the primary site to other places in the body.

• #37 UCSF Ewing Sarcoma Clinical Trials for 2025 — San Francisco Bay Area

  https://clinicaltrials.ucsf.edu/ewing-sarcoma

  This randomized phase III trial studies how well combination chemotherapy with or without ganitumab works in treating patients with newly diagnosed Ewing sarcoma that has spread to other parts of the body. […] Phase 2 to learn about the efficacy of palbociclib in combination with irinotecan and temozolomide when compared with irinotecan and temozolomide alone in the treatment of children, adolescents, and young adults with recurrent or refractory Ewing sarcoma (EWS).

• #38

  https://journals.lww.com/co-oncology/fulltext/2024/07000/emerging_therapies_in_ewing_sarcoma.14.aspx

  Therefore, there remains a significant unmet need to better tailor treatment and identify novel therapies to improve outcome. […] The current European collaborative trials, Inter-Ewing-1 and iEuroEwing are evaluating the addition of cyclophosphamide and vinorelbine to VDC/IE as maintenance chemotherapy in this setting but with flexible designs that allow evaluation of additional agents as described forthwith. […] Patients with recurrent or refractory disease have poor outcomes with a median overall survival of around 12 months. […] The trial is ongoing. In pairwise comparisons, GD was the least effective; imaging response and survival outcomes after TC were marginally better than after IT, whereas in a phase III comparison, IFOS had significantly better progression-free survival (PFS) and overall survival (OS) than TC, making IFOS the most effective regimen, albeit with significant renal and neurological toxicity in a minority.

• #39 Groundbreaking trial launches for everyone with Ewing sarcoma – Cancer Research UK – Cancer Newsfacebook icontwitter iconlinkedin iconfacebook icontwitter iconinstagram iconlinkedin iconyoutube iconfundraising regulator icon

  https://news.cancerresearchuk.org/2024/09/25/inter-ewing-1-groundbreaking-all-ages-ewing-sarcoma-trial/

  Groundbreaking trial launches for everyone with Ewing sarcoma […] Soon after finding out she had cancer, Caitlin was assigned the standard treatment plan for Ewing sarcoma, which usually involves a combination of chemotherapy, radiotherapy and surgery. […] Unfortunately, this treatment protocol hasn’t changed much since the 1990s. That’s why, when Caitlin was offered a place on a brand-new Ewing sarcoma trial on her first day of chemotherapy, she accepted immediately. […] As the largest and most ambitious study ever conducted into Ewing sarcoma, INTER-EWING-1 aims to uncover new insights that could transform treatment options for everyone diagnosed with the disease. […] The first arm of INTER-EWING-1, which Caitlin is joining, will explore whether adding six further cycles of chemotherapy to standard treatment leads to better results.

• #40 Groundbreaking trial launches for everyone with Ewing sarcoma – Cancer Research UK – Cancer Newsfacebook icontwitter iconlinkedin iconfacebook icontwitter iconinstagram iconlinkedin iconyoutube iconfundraising regulator icon

  https://news.cancerresearchuk.org/2024/09/25/inter-ewing-1-groundbreaking-all-ages-ewing-sarcoma-trial/

  The second part of the trial will investigate what dose of radiotherapy treatment produces the best results in patients who cannot have surgery, as well as in those receiving radiotherapy after their tumour has been surgically removed. […] And the final arm will explore whether regorafenib, a medicine that has already shown success treating other types of sarcomas, can improve outcomes for newly diagnosed metastatic Ewing sarcoma patients. […] The introduction of regorafenib in clinical trials marks a huge step forward in tackling Ewing sarcoma, as it’s the first time a targeted drug has ever been trialled as a first treatment option for this cancer type. […] Brennan, who has dedicated her research career to coordinating trials of Ewing sarcoma treatments, believes regorafenib has the potential to overcome the disease’s ‘undruggability’.

• #41 Ewing Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK559183/

  Ewing sarcoma (ES) is a highly metastatic form of sarcoma that ranks as the second most prevalent primary malignant bone tumor, predominantly afflicting adolescents. […] Considerable advancements in local therapy and multiagent adjuvant chemotherapy have substantially improved the 5-year survival rate from less than 20% to over 70%. […] Timely recognition and treatment are imperative to mitigate Ewing sarcoma-related morbidity and mortality risks. […] The standard of care for patients with or without metastasis includes interprofessional treatment with chemotherapy and local therapy, including surgery and radiotherapy. Broadly, systemic therapy is the cornerstone of treatment for all Ewing sarcoma patients. In the United States, this consists of VDC (vincristine/doxorubicin/cyclophosphamide) with alternating IE (ifosfamide/etoposide). After induction chemotherapy, clinicians typically recommend local therapy using radiation, surgery, or a combination of both. Nonmetastatic disease has a 5-year survival rate of 75% to 80%, while metastatic disease is around 30%.

• #42

  https://www.beaumont.org/treatments/ewings-sarcoma-treatment

  Specific treatment for Ewing sarcoma will be determined by your physician based on: […] Treatment may include: surgery, chemotherapy, radiation therapy, amputation, resections for metastases (e.g., pulmonary resections of cancer cells in the lung), rehabilitation including physical and occupational therapy, and psychosocial adaptation, prosthesis fitting and training, supportive care (for the side effects of treatment), antibiotics (to prevent and treat infections), continual follow-up care (to determine response to treatment, detect recurrent disease, and manage late effects of treatment). […] Prognosis for Ewing sarcoma greatly depends on: the extent of the disease, the size and location of the tumor, presence or absence of metastasis, the tumor’s response to therapy, your age and overall health, your tolerance of specific medications, procedures, or therapies, new developments in treatment.

• #42

  https://www.beaumont.org/treatments/ewings-sarcoma-treatment

  Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for a person diagnosed with Ewing sarcoma. Late effects of radiation and chemotherapy, as well as second malignancies, can occur in survivors of Ewing sarcoma. New methods are continually being discovered to improve treatment and to decrease side effects.

• #43 Ewing sarcoma outlook: Is it curable?

  https://www.medicalnewstoday.com/articles/is-ewing-sarcoma-curable

  Ewing sarcoma has an overall 5-year relative survival rate of 62% for all stages combined. […] Ewing sarcoma has an overall 5-year survival rate of 62%, which improves to 80% with a localized tumor. If this cancer spreads to the immediate region, the survival rate is 70%. However, it falls to 39% if it spreads to distant body parts. […] After diagnosis, treatment may include surgery, radiation, chemotherapy, or a combination of treatments.

• #44 Ewing Sarcoma: Symptoms, Treatment & Prognosis

  https://my.clevelandclinic.org/health/diseases/21752-ewings-sarcoma

  Kids who have Ewing sarcoma often have late effects. Late effects are health issues that surface months and years after diagnosis or treatment. These health issues may include second cancers, which are new and different cancers that develop long after they’ve completed cancer treatment. Cancer treatment may cause issues with peoples: […] Ewing sarcoma survival rates vary depending on factors like a child’s age and whether cancerous tumors spread before diagnosis. Based on age, 78% of kids under age 15 and 68% of teenagers ages 15 to 19 were alive five years after diagnosis. […] It’s important to remember that Ewing sarcoma survival rates are estimates based on other peoples experiences with the condition. Their situations may be very different from yours. If you have questions, ask your healthcare provider to explain your or your child’s prognosis.

• #45

  https://link.springer.com/article/10.1007/s12094-024-03602-5

  Ewing sarcoma is a small round-cell sarcoma characterized by gene fusion involving EWSR1 (or another TET family protein like FUS) and an ETS family transcription factor. […] Patient management requires multimodal therapies including intensive chemotherapy in addition to local treatments (surgery and/or radiotherapy). […] In the recurrent/refractory disease setting, different approaches involving systemic treatments and local therapies are also recommended as well as patient inclusion in clinical trials whenever possible. […] Because of the complexity of Ewing sarcoma diagnosis and treatment, it should be carried out in specialized centers and treatment plans should be designed upfront by a multidisciplinary tumor board. […] These guidelines provide recommendations for diagnosis, staging, and multimodal treatment of Ewing sarcoma.

• #46 Ewing Sarcoma – Diagnosis & Disease Information

  https://www.cancertherapyadvisor.com/ddi/ewing-sarcoma/

  Significant advances in the treatment of Ewing sarcoma have improved survival and outcomes, but an optimal prognosis is highly dependent upon an early and accurate diagnosis. […] Treatment planning for children with Ewing sarcoma should be coordinated by an interdisciplinary team of physicians who are experienced in treating children with cancer and overseen by a pediatric oncologist. Treatment of Ewing sarcoma typically consists of chemotherapy, radiation therapy, and/or surgery. […] According to the National Comprehensive Cancer Network guidelines, systemic combination chemotherapy is the first-line treatment for Ewing sarcoma. A combination of chemotherapy agents is administered orally and/or intravenously, with the goal of shrinking the tumor and killing any cancer cells that may have metastasized. In the US, patients with Ewing sarcoma are typically treated with vincristine/doxorubicin/cyclophosphamide (VDC) alternating with ifosfamide/etoposide.

• #47 Ewing Sarcoma: Overview | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions/e/ewing-sarcoma-overview.html

  Your treatment choices depend on the exact type of cancer you have, where it is, test results, and the stage of the cancer. The goal of treatment may be to cure you, control the cancer, or help ease problems caused by the cancer. Talk with your healthcare team about your treatment choices, the goals of treatment, and what the risks and side effects may be. […] Ewing sarcoma may be treated with: […] Chemotherapy […] Surgery […] Radiation therapy […] Stem cell transplant […] Talk with your healthcare providers about your treatment choices. Make a list of questions. Think about the benefits and possible side effects of each choice. Talk about your concerns with your healthcare provider before making a decision. […] Cancer treatment, such as chemotherapy and radiation, can damage normal cells. This can cause side effects like hair loss, mouth sores, and vomiting. […] Talk with your healthcare provider about side effects linked to your treatments and what can be done to manage them. There are often things you can do and medicines you can take to help prevent or control side effects.

• #48 Ewing Sarcoma Medication: Antineoplastic Agents, Uroprotectants

  https://emedicine.medscape.com/article/990378-medication

  Ifosfamide exerts its cytotoxic effect via alkylation of DNA, leading to interstrand and intrastrand DNA crosslinks, DNA-protein crosslinks, and inhibition of DNA replication. […] Vincristine is a plant-derived vinca alkaloid that acts as mitotic inhibitor by binding tubulin. It inhibits microtubule formation in the mitotic spindle, causing metaphase arrest. […] Mesna inactivates acrolein (the urotoxic metabolite of ifosfamide and cyclophosphamide) and prevents urothelial toxicity without affecting cytostatic activity.

• #49 Ewing Sarcoma: Symptoms, Treatment & Prognosis

  https://my.clevelandclinic.org/health/diseases/21752-ewings-sarcoma

  A cancer diagnosis is tough at any age. But it can be especially hard when it’s Ewing sarcoma, which typically affects children and teenagers. If your child has this condition, please remember chemotherapy and other treatments have helped other children live longer cancer-free. Medical researchers are investigating new treatments tailored to Ewing sarcoma. If your child has this condition, ask their healthcare provider about clinical trials that may benefit them.

• #50

  https://journals.lww.com/co-oncology/fulltext/2024/07000/emerging_therapies_in_ewing_sarcoma.14.aspx

  There is an unmet need to improve outcomes for patients for Ewing sarcoma, a rare, aggressive sarcoma with a peak incidence in adolescents and young adults (AYA). Current therapy at diagnosis involves multiagent chemotherapy and local therapy, but despite intensification of treatment, those with metastases at diagnosis and recurrent disease have poor outcomes. […] Improved understanding of Ewing sarcoma biology has identified novel targets with promising activity in Ewing sarcoma patients, including tyrosine kinase inhibitors that are now undergoing evaluation as combination and maintenance therapy. Other emerging therapies include those that target the EWSR1::FLI1 fusion oncoprotein, and act on DNA damage, cell cycle and apoptotic pathways. […] Treatment of Ewing sarcoma requires multimodality therapy that involves intensive multiagent chemotherapy and local therapy (surgery, radiation therapy, or both).

• #51 Ewing Sarcoma—Diagnosis, Treatment, Clinical Challenges and Future Perspectives

  https://www.mdpi.com/2077-0383/10/8/1685

  The therapeutic success of chemotherapy in EwS has been remarkable if you compare today’s survival with that of the 1970s. […] However, no new drugs have been successfully introduced to newly diagnosed patients for almost 40 years. […] A mechanism-based approach would utilize tumor-driving biologic insights that could include activating proto-oncogenes, inactivation of tumor suppressors or use tumor-specific genomic abnormalities to target toxin-bound antibodies or direct an immune-response to the cancer cell. […] Despite the attractive nature of targeting EWSR1-FLI1 fusion proteins, targeting the fusion is considered challenging as these proteins lack enzymatic activity and unlike kinases mostly lack obvious pockets for small molecules to bind. […] Effective clinical therapeutic targeting of EWSR1-FLI1 will require a deeper knowledge of its collaborating macromolecules (e.g., protein or RNA interactions).

• #52 Groundbreaking trial launches for everyone with Ewing sarcoma – Cancer Research UK – Cancer Newsfacebook icontwitter iconlinkedin iconfacebook icontwitter iconinstagram iconlinkedin iconyoutube iconfundraising regulator icon

  https://news.cancerresearchuk.org/2024/09/25/inter-ewing-1-groundbreaking-all-ages-ewing-sarcoma-trial/

  Now, with additional resources, INTER-EWING-1 is also exploring radiotherapy and biomarkers – areas which have previously been overlooked. […] The radiotherapy arm of INTER-EWING-1 has specifically been designed to replicate what Euro Ewing 2012 did for chemotherapy and compare the approaches used in different countries to establish an evidence-based standard of care. […] An important aspect of INTER-EWING-1 is that it will also run in Australia and New Zealand, instead of being limited to Europe. […] By pooling data from around the world, Brennan and her fellow researchers will be better equipped to evaluate the success of regorafenib, different radiotherapy doses and maintenance chemotherapy. […] With Brennan and her team driving forward progress, we’re hopeful that INTER-EWING-1 could revolutionise treatment for Caitlin and all others affected by Ewing sarcoma.

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