Materiały źródłowe

• #1 Rhabdomyosarcoma: Current Therapy, Challenges, and Future Approaches to Treatment Strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10650215/

  Rhabdomyosarcoma (RMS) is a rare pediatric sarcoma affecting skeletal muscle in children and young adults. It is responsible for 3% of all childhood malignant tumors and is the third most prevalent pediatric extracranial solid tumor. Despite advances in diagnostic and treatment methods and clinical trials to improve pediatric RMS survival rates, children with high-risk RMS and recurrent disease have 5-year survival rates of less than 30% and 17%, respectively. The cure rate remains low and the current RMS therapies continue to pose potential life-threatening toxicities, which can lead to lifelong morbidity. The treatment strategies for RMS include multi-agent chemotherapies after surgical resection with or without radiotherapy. […] The treatment strategies for rhabdomyosarcoma include multi-agent chemotherapies after surgical resection with or without ionization radiotherapy. In this comprehensive review, we first provide a detailed clinical understanding of rhabdomyosarcoma including its classification and subtypes, diagnosis, and treatment strategies. Later, we focus on chemotherapy strategies for this childhood sarcoma and discuss the impact of three mechanisms that are involved in the chemotherapy response including apoptosis, macro-autophagy, and the unfolded protein response. Finally, we discuss in vivo mouse and zebrafish models and in vitro three-dimensional bioengineering models of rhabdomyosarcoma to screen future therapeutic approaches and promote muscle regeneration.

• #2 Rhabdomyosarcoma: Current Therapy, Challenges, and Future Approaches to Treatment Strategies

  https://www.mdpi.com/2072-6694/15/21/5269

  Rhabdomyosarcoma (RMS) is a rare pediatric sarcoma affecting skeletal muscle in children and young adults. It is responsible for 3% of all childhood malignant tumors and is the third most prevalent pediatric extracranial solid tumor. Despite advances in diagnostic and treatment methods and clinical trials to improve pediatric RMS survival rates, children with high-risk RMS and recurrent disease have 5-year survival rates of less than 30% and 17%, respectively. The cure rate remains low and the current RMS therapies continue to pose potential life-threatening toxicities, which can lead to lifelong morbidity. The treatment strategies for RMS include multi-agent chemotherapies after surgical resection with or without radiotherapy. Here, we focus on chemotherapy strategies and discuss the impact of apoptosis, autophagy, and the UPR that are involved in the chemotherapy response.

• #3 Understanding adult rhabdomyosarcoma: Types, prognosis and treatment | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/understanding-adult-rhabdomyosarcoma–types–prognosis-and-treatment.h00-159773289.html

  Rhabdomyosarcoma is a rare type of soft tissue sarcoma that develops in the skeletal muscles. But rhabdomyosarcoma can affect adults, too. And adult rhabdomyosarcoma is often more difficult to treat. […] Typically, we treat alveolar rhabdomyosarcoma and embryonal rhabdomyosarcoma similarly to how we treat pediatric cancers. But we may modify the treatment when they’re diagnosed in adults. This could mean changing the medicine or adjusting the dosage. […] Treatment for almost all patients with rhabdomyosarcoma child or adult, regardless of subtype typically involves: chemotherapy, surgery and/or radiation therapy. […] So, we usually do chemotherapy first to shrink the main tumor that we can see. Surgery and/or radiation may follow, depending on the cancer’s stage and grade. After that, we’ll give the patient more chemotherapy to eliminate metastasis and help prevent recurrence.

• #4 Pediatric Rhabdomyosarcoma Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/988803-treatment

  Treatment in patients with rhabdomyosarcoma (RMS) involves a combination of surgery, chemotherapy, and radiation therapy. Because the treatment plan is complicated and prolonged and because many medical issues are unique to pediatric oncology, all patients should be referred (at least initially) to a center with personnel who are skilled in caring for children with cancer. […] At present, patients are categorized according to their risk, which takes into account the location of the tumor and the histologic and surgical results. Low-risk patients are those who have the best prognosis, whereas intermediate-risk or high-risk patients have an increased risk of having relapses and incurable disease. […] Although all patients require chemotherapy, regimens vary depending on the stage and group.

• #5 Rhabdomyosarcoma in childhood, adolescence, and adulthood: Treatment – UpToDate

  https://www.uptodate.com/contents/rhabdomyosarcoma-in-childhood-adolescence-and-adulthood-treatment

  Rhabdomyosarcoma in childhood, adolescence, and adulthood: Treatment […] The treatment of RMS has evolved considerably. Cure rates have risen, largely due to the use of combined modality therapy in trials conducted by large international cooperative groups, such as the Intergroup Rhabdomyosarcoma Study Group (IRSG), which is also known as the Soft Tissue Sarcoma Committee of the Children’s Oncology Group (COG). […] This topic review will provide an overview of treatment for RMS. […] TREATMENT IN CHILDHOOD AND ADOLESCENCE […] Overview of risk-adapted therapy — In the past, fewer than 20 percent of patients with rhabdomyosarcoma (RMS) were cured with surgery alone, implying that micrometastatic disease was present at diagnosis in the majority of these patients. By contrast, with the use of modern combined modality therapy, over 70 percent of children with localized RMS can be cured of their disease.

• #6 Understanding adult rhabdomyosarcoma: Types, prognosis and treatment | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/understanding-adult-rhabdomyosarcoma–types–prognosis-and-treatment.h00-159773289.html

  Adult rhabdomyosarcoma is curable. At MD Anderson, we use a multi-modality treatment approach with the intent to cure the disease. […] We are trying to determine the best standard treatment for older adults with rhabdomyosarcoma. […] Im also leading a clinical trial to see if T-cell therapy can be used to treat patients with advanced or metastatic rhabdomyosarcoma. […] Any patient diagnosed with adult rhabdomyosarcoma or suspected of having this diagnosis should seek care at a sarcoma center that specializes in seeing and treating this disease.

• #7 Childhood Rhabdomyosarcoma Treatment – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/patient/rhabdomyosarcoma-treatment-pdq

  Childhood rhabdomyosarcoma is a disease in which malignant (cancer) cells form in muscle tissue. […] Treatment for childhood rhabdomyosarcoma is based in part on the stage and sometimes on the amount of cancer that remains after surgery to remove the tumor. […] After childhood rhabdomyosarcoma has been diagnosed, treatment is based in part on the stage of the cancer and sometimes it is based on whether all the cancer was removed by surgery. […] The treatment of newly diagnosed childhood rhabdomyosarcoma often includes surgery, radiation therapy, and chemotherapy. […] Three types of standard treatment are used: Surgery, Radiation therapy, Chemotherapy. […] New types of treatment are being tested in clinical trials. […] Children with rhabdomyosarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.

• #8 Treating Rhabdomyosarcoma | Treatment for Rhabdomyosarcoma | American Cancer Society

  https://www.cancer.org/cancer/types/rhabdomyosarcoma/treating.html

  If you or your child has been diagnosed with rhabdomyosarcoma (RMS), your treatment team will discuss the options with you. Its important to weigh the benefits of each treatment option against the possible risks and side effects. […] The types of treatment used for rhabdomyosarcoma (RMS) include: Surgery for Rhabdomyosarcoma, Chemotherapy for Rhabdomyosarcoma, Radiation Therapy for Rhabdomyosarcoma, High-Dose Chemotherapy and Stem Cell Transplant for Rhabdomyosarcoma. […] RMS is almost always treated with both local therapy (surgery and/or radiation therapy) as well as chemotherapy. But the details of treatment can vary based on a number of factors, including the location and extent of the tumor and which risk group the patient falls into. […] All children and adults with RMS will be treated with surgery to remove the tumor if it can be done without causing major damage or disfigurement. If this isnt possible, chemotherapy and/or radiation therapy may be used first to try to shrink the tumor. If it shrinks enough, surgery can be done at this point. The goal of surgery is to remove the tumor completely, but this isnt always possible.

• #9 Rhabdomyosarcoma: Symptoms, Diagnosis, Treatment, and Prognosis

  https://www.webmd.com/cancer/multiple-myeloma/rhabdomyosarcoma-cancer

  Rhabdomyosarcoma Treatment […] There are two main ways doctors treat rhabdomyosarcoma. Some treatments are meant to get rid of as much of the tumor as possible. This could mean doing surgery to take the tumor out, giving you radiation treatments to shrink the tumor, or both. Other treatments, such as chemotherapy, aim to kill cancer cells or stop them from growing. […] Your medical team will talk with you about your treatment options. What they recommend will depend on your risk group, the size of the tumor, whether its in a place where doctors can fully remove it through surgery, and other factors. […] Doctors usually treat RMS with some combination of these treatments: Surgery. Depending on where the tumor is, doctors might do surgery to take it out. They might be able to get all of it or just some of it. How complicated the operation is can depend on how big the tumor is and other factors.

• #10 Rhabdomyosarcoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/rhabdomyosarcoma/diagnosis-treatment/drc-20390966

  Rhabdomyosarcoma treatment most often combines chemotherapy, surgery and radiation therapy. […] Which treatments your healthcare team suggests depends on where the cancer is and the size of the cancer. Treatment also will depend on how fast the cancer cells are likely to grow and whether the cancer has spread to other parts of the body. […] The goal of surgery is to remove all the cancer cells. But that’s not always possible if the rhabdomyosarcoma has grown around or near organs. If the surgeon can’t safely remove all the cancer, your healthcare team will use other treatments to kill cancer cells that might be left. This might include chemotherapy and radiation. […] Chemotherapy treats cancer with strong medicines. Many chemotherapy medicines exist. Treatment often involves a combination of medicines. Most chemotherapy medicines are given through a vein. Some come in pill form.

• #11 Rhabdomyosarcoma | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/r/rhabdomyosarcoma

  Rhabdomyosarcoma Treatment may involve surgery, radiation therapy and chemotherapy alone or in combination. […] In some situations, the doctor may recommend proton therapy instead of traditional radiation therapy. Proton therapy targets the tumor while avoiding organs and healthy tissue. This means fewer short-term side effects and long-term complications from radiation. Cincinnati Childrens is one of only a few pediatric hospitals in the country that offer proton therapy. […] Your child may receive treatment as an inpatient and / or as an outpatient, depending on the stage of the disease and the phase of therapy. We will do periodic blood tests to check white blood cells, red blood cells and platelet counts, and test for any signs of returning tumor. Every few months, we will obtain imaging of the lungs, liver and bones.

• #12 Rhabdomyosarcoma Treatment | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/pediatrics/cancer-care/types/rhabdomyosarcoma/treatment

  Your childs care plan depends on the type and stage of rhabdomyosarcoma. Typically, people with low-risk rhabdomyosarcoma need less therapy than those in the higher-risk groups. Alveolar rhabdomyosarcoma may need more-intensive treatment than the embryonal type. Our team will explain how aggressive your childs cancer is and the combination of treatments needed to treat it. […] At MSK Kids, we have a large arsenal of therapies to choose from to treat your childs rhabdomyosarcoma, which include: […] Chemotherapy first to shrink the tumor, followed by surgery in some patients to remove the tumor completely. […] Intensity-modulated radiation therapy (IMRT), a very precise form of radiation therapy. Radiation beams of different intensities are aimed at the tumor from multiple angles to kill cancer cells while sparing nearby healthy tissue. We have found that varying the dose of IMRT in different areas of the tumor reduces side effects in some younger patients.

• #13 Treating Rhabdomyosarcoma | Treatment for Rhabdomyosarcoma | American Cancer Society

  https://www.cancer.org/cancer/types/rhabdomyosarcoma/treating.html

  If you or your child has been diagnosed with rhabdomyosarcoma (RMS), your treatment team will discuss the options with you. Its important to weigh the benefits of each treatment option against the possible risks and side effects. […] The types of treatment used for rhabdomyosarcoma (RMS) include: Surgery for Rhabdomyosarcoma, Chemotherapy for Rhabdomyosarcoma, Radiation Therapy for Rhabdomyosarcoma, High-Dose Chemotherapy and Stem Cell Transplant for Rhabdomyosarcoma. […] RMS is almost always treated with both local therapy (surgery and/or radiation therapy) as well as chemotherapy. But the details of treatment can vary based on a number of factors, including the location and extent of the tumor and which risk group the patient falls into. […] All children and adults with RMS will be treated with surgery to remove the tumor if it can be done without causing major damage or disfigurement. If this isnt possible, chemotherapy and/or radiation therapy may be used first to try to shrink the tumor. If it shrinks enough, surgery can be done at this point. The goal of surgery is to remove the tumor completely, but this isnt always possible.

• #14 Rhabdomyosarcoma: Current Therapy, Challenges, and Future Approaches to Treatment Strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10650215/

  The mainstay of treatment for RMS involves multi-agent systemic chemotherapy in order to eradicate disseminated disease, along with surgical resection of the primary tumor with or without addition of the ionizing radiation therapy for the control of local disease. The timing of systemic chemotherapy remains controversial; however, most North American centers will administer chemotherapy in the neoadjuvant setting. Surgical resection has been shown to increase survival in Group I and II diseases, whereas those with Group III disease may experience increased morbidity without improved survival rate. […] Complete surgical resection with circumferential margins greater than 0.51 cm is the preferred treatment method for localized RMS. Adequate negative margins are required unless the surgical excision threatens adjacent organs, leads to the loss of significant function, results in poor cosmesis, or is not technically feasible.

• #15 Rhabdomyosarcoma Treatment | St. Jude Care & Treatment

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

  Rhabdomyosarcoma is a type of cancerous tumor that develops in soft tissue, such as muscles. But it can occur anywhere in the body. […] Rhabdomyosarcoma is the most common type of soft tissue sarcoma in children. About 350 new cases are diagnosed each year in the United States. More than 70% of children with rhabdomyosarcoma that has not spread survive long-term. […] Surgery is used to biopsy the tumor. A small piece is removed and examined in the lab to help diagnose your child’s disease. Then the surgeon may remove as much of the tumor as possible. […] Chemotherapy (chemo) is given to all rhabdomyosarcoma patients after surgery. Chemotherapy uses powerful medicines to kill cancer cells or stop them from spreading. […] Radiation therapy may be used to treat these tumors. Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or stop them from growing.

• #16 Rhabdomyosarcoma | Children’s Hospital of Philadelphia

  https://gps.chop.edu/condition/rhabdomyosarcoma

  Rhabdomyosarcoma, while rare, is the most common type of soft tissue sarcoma (a tumor that is malignant, or that spreads) in children. […] There are many treatment options available for soft tissue tumors like rhabdomyosarcoma, and some children will need a combination of these therapies. At Childrens Hospital of Philadelphia (CHOP), experts from the Bone and Soft Tissue Tumor Program take a team approach to treatment. Orthopaedists, oncologists and other specialists collaborate to provide your child with individualized care and the best possible outcomes. […] Surgery is usually necessary for rhabdomyosarcoma in children. Our surgeons will remove the cancer using the most advanced surgical techniques available. […] More than 90 percent of children with rhabdomyosarcoma can be treated with limb-sparing (also known as limb-salvage) and reconstructive surgery.

• #17 Rhabdomyosarcoma: Symptoms and Treatment | Doctor

  https://patient.info/doctor/rhabdomyosarcoma-pro

  Rhabdomyosarcoma treatment and management […] Eradication of the gross primary tumour, which is often accomplished using a combination of surgery and/or external beam ionising radiation. […] Outcome is optimised with the use of multimodality therapy. All patients require chemotherapy and at least 85% also benefit from radiotherapy, with favourable outcome even for those patients with non-resectable disease. […] Surgery is recommended for all lesions, provided it is feasible, and as much of the tumour should be removed as possible. […] The most active chemotherapy agents against rhabdomyosarcoma cells are vincristine, cyclophosphamide, actinomycin D, doxorubicin, isophosphamide and etoposide. […] This is given postoperatively, and occasionally pre-operatively to shrink tumour size, and commonly in head, neck and pelvic tumours.

• #18 Rhabdomyosarcoma: Current Therapy, Challenges, and Future Approaches to Treatment Strategies

  https://www.mdpi.com/2072-6694/15/21/5269

  The mainstay of treatment for RMS involves multi-agent systemic chemotherapy in order to eradicate disseminated disease, along with surgical resection of the primary tumor with or without addition of the ionizing radiation therapy for the control of local disease. The timing of systemic chemotherapy remains controversial; however, most North American centers will administer chemotherapy in the neoadjuvant setting. Surgical resection has been shown to increase survival in Group I and II diseases, whereas those with Group III disease may experience increased morbidity without improved survival rate. Complete surgical resection with circumferential margins greater than 0.5–1 cm is the preferred treatment method for localized RMS. […] To ensure that the margins of resection are adequately evaluated, a close cooperation between surgeon and pathologist is warranted. Ideally, the pathologist should be provided with adequate information on the location and orientation of the taken biopsies with the fresh tissue being directly sent to the laboratory. One of the emerging new techniques that could improve resection accuracy is fluorescence-guided surgery that allows for the visualization of tumors in real-time using, most commonly, indocyanine green dye.

• #19 Rhabdomyosarcoma > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/rhabdomyosarcoma

  Treatment includes surgery, radiation therapy, chemotherapy […] Treatment of rhabdomyosarcoma varies depending on the location, size, and genetic features of the tumor, says Juan Vasquez, MD, assistant professor of pediatrics (hematology/oncology) at Smilow Cancer Hospital. […] Chemotherapy is an important part of treatment to help cure disease and prevent the cancer from coming back. […] For rhabdomyosarcoma that is located in the arms or legs, surgery is often used to remove the tumor. After surgery, radiation therapy may be used to help kill any remaining cancer cells. […] In cases when surgery is not possible, radiation therapy, in conjunction with chemotherapy, may be used to eliminate the tumor. […] Treatment for rhabdomyosarcoma may vary, based on the location, size, and type of the tumor. Options include:

• #20 Rhabdomyosarcoma: Current Therapy, Challenges, and Future Approaches to Treatment Strategies

  https://www.mdpi.com/2072-6694/15/21/5269

  In the event that there are positive margins, patients may undergo radiation therapy or further surgical resection of the tumor. Re-excision of recurrent RMS has been shown to increase 5-year survival rates from 8% to 37% compared to the patients without aggressive re-excision. […] Currently approved chemotherapeutic agents for the treatment of RMS include cyclophosphamide, actinomycin-D, doxorubicin, etoposide, ifosfamide, irinotecan, melphalan, temsirolimus, vincristine, and volasertib. In North America, chemotherapy for the pediatric patients consists of a backbone of vincristine, actinomycin D, and cyclophosphamide (VAC). In comparison, in Europe, VAC therapy is substituted for a regimen consisting of ifosfamide, vincristine, and actinomycin D (IVA), which has produced similar treatment outcomes.

• #21 Rhabdomyosarcoma: Current Therapy, Challenges, and Future Approaches to Treatment Strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10650215/

  In the event that there are positive margins, patients may undergo radiation therapy or further surgical resection of the tumor. Re-excision of recurrent RMS has been shown to increase 5-year survival rates from 8% to 37% compared to the patients without aggressive re-excision. […] Currently approved chemotherapeutic agents for the treatment of RMS include cyclophosphamide, actinomycin-D, doxorubicin, etoposide, ifosfamide, irinotecan, melphalan, temsirolimus, vincristine, and volasertib. […] In North America, chemotherapy for the pediatric patients consists of a backbone of vincristine, actinomycin D, and cyclophosphamide (VAC). In comparison, in Europe, VAC therapy is substituted for a regimen consisting of ifosfamide, vincristine, and actinomycin D (IVA), which has produced similar treatment outcomes.

• #22 Rhabdomyosarcoma Cancer Symptoms and Treatment | UPMC

  https://www.upmc.com/services/neurosurgery/brain/conditions/brain-tumors/rhabdomyosarcoma

  At UPMC, the preferred surgical treatment for rhabdomyosarcoma in 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. Benefits of EEA include: […] EEA has proven safe and effective in children. More than 100 children have been treated by UPMC skull base surgeons using EEA more than at any other neurosurgery center in the world. […] UPMC’s neurosurgical team may recommend a combination of surgical and non-surgical approaches to treat rhabdomyosarcoma. […] Surgery is one of the most common methods for treating rhabdomyosarcoma. Surgery requires removal of the cancerous tumor and nearby tissue. […] Rhabdomyosarcoma cancers of the skull base may be approached directly using Endoscopic Endonasal Approach (EEA). This state-of-the-art, minimally invasive approach allows surgeons to access the tumor through the natural corridor of the nose, without making an open incision. Surgeons then remove the tumor through the nose and nasal cavities.

• #23 Rhabdomyosarcoma | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/rhabdomyosarcoma

  There are many treatment options available for soft tissue tumors like rhabdomyosarcoma, including chemotherapy, surgery, and radiation therapy. Most children with rhabdomyosarcoma will need a combination of these therapies. At CHOP, experts at the Bone and Soft Tissue Tumor Program collaborate with specialists such as urologists and radiation oncologists, depending on the tumors location, to provide your child with individualized care and the best possible outcomes. […] In addition to treatment of the primary tumor, almost all children with rhabdomyosarcoma will require treatment with chemotherapy to target cancer cells that may have already spread but have not yet been detected. Chemotherapy refers to medicines that help fight cancer. […] Surgery is often recommended for rhabdomyosarcoma in children. Our surgeons will remove the cancer using the most advanced surgical techniques available.

• #24 Treating Rhabdomyosarcoma | Treatment for Rhabdomyosarcoma | American Cancer Society

  https://www.cancer.org/cancer/types/rhabdomyosarcoma/treating.html

  Even if the tumor appears to have been removed completely, all patients with RMS need chemotherapy. Without it, its very likely that the cancer will come back in distant parts in the body because small amounts of cancer have almost always reached other parts of the body when the cancer is first found (even thought they can’t be seen on imaging tests). […] If cancer is left behind after surgery or if the cancer has some less favorable traits and it hasnt spread to distant sites (as is the case most of the time), radiation therapy will also be given. […] Many of these treatments can be used again if the cancer continues to grow or if it comes back later on. […] RMS is not common, and treating it can be complex, so its very important for patients to be diagnosed and treated by a team of doctors who have experience with RMS.

• #25 Chemotherapy for rhabdomyosarcoma | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/rhabdomyosarcoma/treatment/chemotherapy

  Chemotherapy uses anticancer (cytotoxic) drugs to destroy cancer cells. Almost all children with rhabdomyosarcoma (RMS) will have chemotherapy. The type of drugs, doses and schedules of chemotherapy depend on the risk group. […] Chemotherapy is given for different reasons. Your child may have chemotherapy to: […] destroy cancer cells that may be at a distant site after surgery and reduce the risk that the cancer will come back (recur) (called adjuvant chemotherapy) […] shrink a tumour before other treatments such as surgery or radiation therapy (called neoadjuvant chemotherapy) […] relieve pain or control the symptoms of advanced RMS (called palliative chemotherapy). […] Chemotherapy is usually a systemic therapy. This means that the drugs travel through the blood to reach and destroy cancer cells all over the body, including those that may have broken away from the primary tumour in the muscle.

• #26 Recent Advances and Challenges in the Treatment of Rhabdomyosarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7409313/

  Multi-drug chemotherapy regimens significantly improved the outcome in patients with localized RMS, although no marked improvement has been observed in patients with metastatic RMS. […] Standard chemotherapy regimens for RMS in North America include vincristine, actinomycin D, and cyclophosphamide (VAC), whereas those in Europe include ifosfamide, vincristine, and actinomycin D (IVA). […] High-dose chemotherapy was thought to improve the outcomes compared to standard chemotherapy in patients with metastatic RMS. However, it was reported that a significant improvement was not observed in patients treated with high-dose chemotherapy compared to that in those treated with standard chemotherapy, although an increased incidence of treatment-related adverse events (AEs) was observed in patients with high-dose chemotherapy.

• #27 Rhabdomyosarcoma: Current Therapy, Challenges, and Future Approaches to Treatment Strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10650215/

  In the event that there are positive margins, patients may undergo radiation therapy or further surgical resection of the tumor. Re-excision of recurrent RMS has been shown to increase 5-year survival rates from 8% to 37% compared to the patients without aggressive re-excision. […] Currently approved chemotherapeutic agents for the treatment of RMS include cyclophosphamide, actinomycin-D, doxorubicin, etoposide, ifosfamide, irinotecan, melphalan, temsirolimus, vincristine, and volasertib. […] In North America, chemotherapy for the pediatric patients consists of a backbone of vincristine, actinomycin D, and cyclophosphamide (VAC). In comparison, in Europe, VAC therapy is substituted for a regimen consisting of ifosfamide, vincristine, and actinomycin D (IVA), which has produced similar treatment outcomes.

• #28 Rhabdomyosarcoma: Current Therapy, Challenges, and Future Approaches to Treatment Strategies

  https://www.mdpi.com/2072-6694/15/21/5269

  In the event that there are positive margins, patients may undergo radiation therapy or further surgical resection of the tumor. Re-excision of recurrent RMS has been shown to increase 5-year survival rates from 8% to 37% compared to the patients without aggressive re-excision. […] Currently approved chemotherapeutic agents for the treatment of RMS include cyclophosphamide, actinomycin-D, doxorubicin, etoposide, ifosfamide, irinotecan, melphalan, temsirolimus, vincristine, and volasertib. In North America, chemotherapy for the pediatric patients consists of a backbone of vincristine, actinomycin D, and cyclophosphamide (VAC). In comparison, in Europe, VAC therapy is substituted for a regimen consisting of ifosfamide, vincristine, and actinomycin D (IVA), which has produced similar treatment outcomes.

• #29 Recent Advances and Challenges in the Treatment of Rhabdomyosarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7409313/

  Rhabdomyosarcoma, the most common soft tissue sarcoma noted in childhood, requires multimodality treatment, including chemotherapy, surgical resection, and/or radiation therapy. The standard chemotherapy regimen for patients with rhabdomyosarcoma is the combination of vincristine, actinomycin, and cyclophosphamide/ifosfamide. […] In various type of malignancies, new treatments, such as molecular targeted drugs and immunotherapies, have shown superior clinical outcomes compared to those of standard treatments. Therefore, it is necessary to assess the benefits of these treatments in patients with rhabdomyosarcoma. […] Standard treatment of RMS comprises chemotherapy (vincristine, actinomycin D, and cyclophosphamide/ifosfamide), radiation therapy, and surgical tumor excision. […] Several basic and clinical studies have been carried out on various treatment strategies, including modified or novel chemotherapy protocols, molecular targeted drug therapy, immunotherapy, and new therapeutic approaches for RMS.

• #30 Rhabdomyosarcoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/rhabdomyosarcoma/diagnosis-treatment/drc-20390966

  For rhabdomyosarcoma, chemotherapy is often used after surgery or radiation therapy. It can help kill cancer cells that might be left. Chemotherapy also can be used before other treatments. The chemotherapy can help shrink a cancer to make it easier to do surgery or radiation therapy. […] Radiation therapy treats cancer with powerful energy beams. The energy can come from X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points on your body. […] For rhabdomyosarcoma, radiation therapy might be recommended after surgery. It can help kill cancer cells that might be left. Radiation therapy also can be used instead of surgery. Radiation therapy might be preferred if the cancer is in an area where surgery isn’t possible because of nearby organs. […] 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. Ask your healthcare professional if you might be able to be in a clinical trial.

• #31 Chemotherapy for rhabdomyosarcoma | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/rhabdomyosarcoma/treatment/chemotherapy

  Chemotherapy uses anticancer (cytotoxic) drugs to destroy cancer cells. Almost all children with rhabdomyosarcoma (RMS) will have chemotherapy. The type of drugs, doses and schedules of chemotherapy depend on the risk group. […] Chemotherapy is given for different reasons. Your child may have chemotherapy to: […] destroy cancer cells that may be at a distant site after surgery and reduce the risk that the cancer will come back (recur) (called adjuvant chemotherapy) […] shrink a tumour before other treatments such as surgery or radiation therapy (called neoadjuvant chemotherapy) […] relieve pain or control the symptoms of advanced RMS (called palliative chemotherapy). […] Chemotherapy is usually a systemic therapy. This means that the drugs travel through the blood to reach and destroy cancer cells all over the body, including those that may have broken away from the primary tumour in the muscle.

• #32 Recent Advances and Challenges in the Treatment of Rhabdomyosarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7409313/

  Based on the results, the authors concluded that addition of doxorubicin in the standard chemotherapy regimen did not lead to a significant improvement in the clinical outcome of RMS. […] These studies indicated that the chemotherapy regimen for RMS should be based on the VAC or IVA regimens, and that addition of doxorubicin or high-dose chemotherapy was thought to have little benefit for patients with RMS. […] In a phase 3 clinical trial, 371 patients with high-risk RMS were randomly assigned to standard chemotherapy group and standard chemotherapy with maintenance chemotherapy (low-dose of vinorelbine and cyclophosphamide) group. […] The five-year disease-free survival (DFS) rates were 78% for the patients with maintenance chemotherapy and 70% for the patients without maintenance chemotherapy.

• #33 New Role for Maintenance Chemo in Rhabdomyosarcoma?

  https://www.cancernetwork.com/view/new-role-maintenance-chemo-rhabdomyosarcoma

  Six months of maintenance chemotherapy improved OS in children with high-risk rhabdomyosarcoma. […] Adding 6 months of maintenance therapy after initial treatment improved overall survival (OS) in patients with high-risk rhabdomyosarcoma (RMS), according to a new study. […] Maintenance therapy represents a novel, well-tolerated, effective strategy in patients with high-risk rhabdomyosarcoma, said lead study author Gianni Bisogno, MD, PhD, from the University Hospital of Padova in Italy and Chair of the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG), who reported the results (abstract LBA 2) at a press conference during the 2018 American Society of Clinical Oncology (ASCO) Annual Meeting, held June 15 in Chicago. […] This study establishes the new standard of treatment for patients with high-risk rhabdomyosarcoma, he said, noting that the same approach can be investigated for other childhood solid tumors.

• #34 Chemotherapy for rhabdomyosarcoma | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/rhabdomyosarcoma/treatment/chemotherapy

  low blood cell counts […] low white blood cell count (neutropenia) […] infection […] low platelet count (thrombocytopenia) […] low red blood cell count (anemia) […] hair loss […] fatigue […] sore mouth and throat […] nausea and vomiting […] loss of appetite […] taste changes […] diarrhea […] constipation. […] Other side effects can develop months or years after treatment for RMS.

• #35 Radiation Therapy for Rhabdomyosarcoma | American Cancer Society

  https://www.cancer.org/cancer/types/rhabdomyosarcoma/treating/radiation-therapy.html

  Intensity-modulated radiation therapy (IMRT): IMRT is an advanced form of 3D therapy. […] Brachytherapy (internal radiation therapy): Another approach is to insert a radioactive source into or near the tumor for a short time. […] The side effects of radiation therapy depend on where the radiation is aimed, the dose of radiation, and the person’s age. […] Short-term side effects can include: Fatigue, Increased risk of infections, Effects on the skin in areas that receive radiation, ranging from hair loss and mild sunburn-like changes to more severe skin reactions, Nausea, vomiting, and diarrhea (from radiation to the abdomen or pelvis), Damage to the bladder, which might cause urinary problems (from radiation to the abdomen or pelvis), Mouth sores and loss of appetite (from radiation to the head and neck area). […] Long-term side effects can be more serious, especially in growing children, so doctors try to limit them as much as possible. […] To limit the risk of serious long-term effects from radiation, doctors use the lowest dose of radiation therapy that is still effective.

• #36 Radiation Therapy for Rhabdomyosarcoma | American Cancer Society

  https://www.cancer.org/cancer/types/rhabdomyosarcoma/treating/radiation-therapy.html

  Radiation therapy uses high-energy radiation to kill cancer cells. It is often an effective way to kill cancer cells that cant be removed with surgery. When radiation therapy is used to help treat rhabdomyosarcoma (RMS), it is typically given along with chemotherapy. […] Radiation is most often used when some of the main tumor is still left after surgery (clinical group II or III), or if removing the tumor completely would mean loss of an important organ, like the eye or bladder, or would be disfiguring. It is not usually needed for children with embryonal rhabdomyosarcoma (ERMS) that can be removed completely by surgery (clinical group I). […] Usually radiation therapy is given to any area of remaining disease after 6 to 12 weeks of chemotherapy. An exception is when a tumor near the meninges (linings of the brain) has grown into the skull bones, into the brain itself, or into the spinal cord. These patients are usually given radiation therapy right away (along with chemotherapy).

• #37 Recent Advances and Challenges in the Treatment of Rhabdomyosarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7409313/

  Trabectedin, a synthetic alkaloid isolated from the marine ascidian, Ecteinascidia turbinate, reduces disease progression and mortality rates for various types of soft tissue sarcoma. […] Although these recent clinical trials did not report significant improvements in the outcomes of RMS, gradual improvement has been noted on modification of the standard chemotherapy regimens. […] RT is one of the standard treatment modalities used in the management of RMS. […] Although patients with low-risk RMS who undergo excision with wide margins do not require RT, most RMS patients require RT, and the radiation doses are determined according to the risk. […] Proton RT is considered beneficial for pediatric patients with malignancies. Additional investigations of the clinical outcomes and long-term safety of proton RT in patients with RMS are warranted to determine the efficacy of proton RT.

• #38 Diagnosis and treatment of rhabdomyosarcomas | Łomiak | Oncology in Clinical Practice

  https://journals.viamedica.pl/oncology_in_clinical_practice/article/view/86149

  Surgery is the basic therapeutic option for RMS patients, regardless of the risk group to which they belong. Local treatment must be considered first after diagnosis with the intent of complete resection of the tumor and obtaining microscopically radical surgical margins. […] Supplementary radiotherapy is indicated in all patients with RMS stages 1-3 according to IRSG TNM and CG IIII except for ARMS without the FOX01 rearrangement (then a decision should be taken based on risk and benefit analysis). […] Adding neo- and adjuvant chemotherapy to the treatment of patients without metastases allowed to obtain 60-90% percent of 5-year survival. […] The intensity (2- or 3-drug treatments) and the duration of treatment (6, 12, or 24 months) depend on the risk group. […] After completion of the treatment, the patient should be observed carefully. The recommended procedure includes the physical examination and imaging studies in the form of CT or MRI of the primary localization and CT of the chest, abdominal cavity, and pelvis using a contrast agent.

• #39 Childhood Rhabdomyosarcoma Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Health Information Library | PeaceHealth

  https://www.peacehealth.org/medical-topics/id/ncicdr0000062792

  Local control remains a significant problem in children with rhabdomyosarcoma. The predominant site of treatment failure in patients with initially localized rhabdomyosarcoma has been local recurrence. […] For children with low-risk rhabdomyosarcoma, local control was not diminished with reduced doses of RT after surgical resection. […] The COG recommends the use of RT for all patients with FOXO1 fusion-positive disease. […] RT is an effective method for achieving local control of the tumor for patients with microscopic or gross residual disease after biopsy, initial surgical resection, or chemotherapy. […] For patients with initially unresectable retroperitoneal/pelvic tumors, complete surgical removal after induction chemotherapy, with or without RT, offers a significant survival advantage.

• #40 Radiation Therapy for Rhabdomyosarcoma | American Cancer Society

  https://www.cancer.org/cancer/types/rhabdomyosarcoma/treating/radiation-therapy.html

  Radiation therapy uses high-energy radiation to kill cancer cells. It is often an effective way to kill cancer cells that cant be removed with surgery. When radiation therapy is used to help treat rhabdomyosarcoma (RMS), it is typically given along with chemotherapy. […] Radiation is most often used when some of the main tumor is still left after surgery (clinical group II or III), or if removing the tumor completely would mean loss of an important organ, like the eye or bladder, or would be disfiguring. It is not usually needed for children with embryonal rhabdomyosarcoma (ERMS) that can be removed completely by surgery (clinical group I). […] Usually radiation therapy is given to any area of remaining disease after 6 to 12 weeks of chemotherapy. An exception is when a tumor near the meninges (linings of the brain) has grown into the skull bones, into the brain itself, or into the spinal cord. These patients are usually given radiation therapy right away (along with chemotherapy).

• #41 Radiation Therapy for Rhabdomyosarcoma | American Cancer Society

  https://www.cancer.org/cancer/types/rhabdomyosarcoma/treating/radiation-therapy.html

  If the cancer has spread to another part of the body, radiation might be given to certain areas of known cancer spread to reduce any symptoms it is causing. […] This type of treatment is given by a doctor called a radiation oncologist. Before treatments start, the radiation team takes careful measurements with imaging tests such as MRI scans to determine the correct angles for aiming the beams and the proper dose of radiation. […] Radiation is usually given 5 days a week for many weeks. Each treatment is much like getting an x-ray, although the dose of radiation is much stronger. […] Modern radiation therapy techniques help doctors aim the treatment at the tumor more accurately than they could in the past. […] Three-dimensional conformal radiation therapy (3D-CRT): 3D-CRT uses the results of imaging tests such as MRI and special computers to precisely map the location of the tumor.

• #42 Rhabdomyosarcoma | Diagnosis & Treatment | UH Rainbow Babies & Children’s Hospital | University Hospitals | Cleveland, Ohio | University Hospitals

  https://www.uhhospitals.org/rainbow/services/pediatric-cancer-and-blood-disorders/conditions-and-treatments/rhabdomyosarcoma/diagnosis-and-treatments

  The UH Proton Therapy Center is Clevelands first provider of proton therapy, an advanced type of radiation therapy used in the treatment of rhabdomyosarcoma and other types of cancers. Available only at select hospitals across the country, proton therapy delivers most of its energy to a very narrow field at the location of the tumor, resulting in less damage to surrounding healthy tissue. In comparison to other forms of radiation therapy, proton therapy can result in better quality of life during treatment and may reduce the risk of patients developing radiation-induced secondary cancers later in life. […] Immunotherapy utilizes a persons own immune system to help kill cancer cells. Most immunotherapeutic approaches are relatively new and in the clinical trial phase of development. These approaches offer hope for young cancer patients to receive effective treatments that may prevent the long-term, damaging side effects associated with more traditional cancer treatment methods such as chemotherapy and radiation.

• #43 Optimizing Rhabdomyosarcoma Treatment in Adolescents and Young Adults

  https://www.mdpi.com/2072-6694/14/9/2270

  Although the excellent chemoresponsiveness of RMS is an advantage in treatment, it may lead to overestimating the effectiveness of systemic chemotherapy and misjudging the role of neoadjuvant and adjuvant chemotherapy. […] The timing and intensity of local treatments for patients with AYA RMS should be reappraised, especially because maintaining dose intensity in chemotherapy is difficult. […] The COG recommends modulating the local radiation dosage depending on the local disease status: 50.4 Gy for gross residual disease, 41.4 Gy for minimal residual disease, and 0–36 Gy for completely resected disease, although the dosages depend on the timing of radiation (before or after neoadjuvant chemotherapy) and the risk factors of the original disease. […] Each AYA patient with RMS should have the right to select a treatment strategy based on his/her own priorities. Therefore, a medical team consisting of specialists in each aspect of the strategy is needed to deliver a treatment optimized for individual AYA patients with RMS based on their expertise.

• #44 Radiation Therapy for Rhabdomyosarcoma | American Cancer Society

  https://www.cancer.org/cancer/types/rhabdomyosarcoma/treating/radiation-therapy.html

  Intensity-modulated radiation therapy (IMRT): IMRT is an advanced form of 3D therapy. […] Brachytherapy (internal radiation therapy): Another approach is to insert a radioactive source into or near the tumor for a short time. […] The side effects of radiation therapy depend on where the radiation is aimed, the dose of radiation, and the person’s age. […] Short-term side effects can include: Fatigue, Increased risk of infections, Effects on the skin in areas that receive radiation, ranging from hair loss and mild sunburn-like changes to more severe skin reactions, Nausea, vomiting, and diarrhea (from radiation to the abdomen or pelvis), Damage to the bladder, which might cause urinary problems (from radiation to the abdomen or pelvis), Mouth sores and loss of appetite (from radiation to the head and neck area). […] Long-term side effects can be more serious, especially in growing children, so doctors try to limit them as much as possible. […] To limit the risk of serious long-term effects from radiation, doctors use the lowest dose of radiation therapy that is still effective.

• #45 Radiation Therapy for Rhabdomyosarcoma | American Cancer Society

  https://www.cancer.org/cancer/types/rhabdomyosarcoma/treating/radiation-therapy.html

  Intensity-modulated radiation therapy (IMRT): IMRT is an advanced form of 3D therapy. […] Brachytherapy (internal radiation therapy): Another approach is to insert a radioactive source into or near the tumor for a short time. […] The side effects of radiation therapy depend on where the radiation is aimed, the dose of radiation, and the person’s age. […] Short-term side effects can include: Fatigue, Increased risk of infections, Effects on the skin in areas that receive radiation, ranging from hair loss and mild sunburn-like changes to more severe skin reactions, Nausea, vomiting, and diarrhea (from radiation to the abdomen or pelvis), Damage to the bladder, which might cause urinary problems (from radiation to the abdomen or pelvis), Mouth sores and loss of appetite (from radiation to the head and neck area). […] Long-term side effects can be more serious, especially in growing children, so doctors try to limit them as much as possible. […] To limit the risk of serious long-term effects from radiation, doctors use the lowest dose of radiation therapy that is still effective.

• #46 Pediatric Rhabdomyosarcoma Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/988803-treatment

  Treatment in patients with rhabdomyosarcoma (RMS) involves a combination of surgery, chemotherapy, and radiation therapy. Because the treatment plan is complicated and prolonged and because many medical issues are unique to pediatric oncology, all patients should be referred (at least initially) to a center with personnel who are skilled in caring for children with cancer. […] At present, patients are categorized according to their risk, which takes into account the location of the tumor and the histologic and surgical results. Low-risk patients are those who have the best prognosis, whereas intermediate-risk or high-risk patients have an increased risk of having relapses and incurable disease. […] Although all patients require chemotherapy, regimens vary depending on the stage and group.

• #47 Rhabdomyosarcoma: Symptoms, Prognosis & Treatment

  https://my.clevelandclinic.org/health/diseases/6226-rhabdomyosarcoma

  Rhabdomyosarcoma is a rare soft tissue cancer that typically affects children and teenagers but can also affect adults. Oncologists can treat and sometimes cure rhabdomyosarcoma, but the condition can come back. […] There are different types of rhabdomyosarcomas, some of which are aggressive and more difficult to treat. In some cases, treatment often puts the condition into remission, but rhabdomyosarcoma can come back (cancer recurrence). […] Treatment varies depending on the condition type. Rhabdomyosarcoma is a rare disease; if you or your child has it, ask the cancer care team about participating in a clinical trial. In general, oncologists use the following treatments: Surgery, Radiation therapy, Chemotherapy. […] Sometimes, treatment can cure rhabdomyosarcoma. This is called remission, which means that you dont have symptoms and tests dont detect signs of cancer. In many cases, remission is permanent, but rhabdomyosarcoma can come back. […] Risk group classifications are low risk, intermediate risk and high risk. Your childs oncology team uses risk group classification to plan treatment, assess the chance the tumor will come back after treatment and establish a prognosis, or what you can expect to happen after treatment.

• #48 RMS2021: Using Molecular Risk Stratification and Liposomal Irinotecan in Children with Intermediate-risk and High-risk Rhabdomyosarcoma | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/clinical-trials/rms2021-rhabdomyosarcoma.html

  Rhabdomyosarcoma (RMS) is a type of cancerous tumor found in soft tissue, such as muscles. The standard treatment for newly diagnosed rhabdomyosarcoma is chemotherapy, surgery, and radiation therapy (the use of high-dose x-rays to kill cancer cells). […] We hope to find the most effective treatment for each risk group of rhabdomyosarcoma patients. The treatment they will receive is based on the risk of their cancer returning. […] Low-risk rhabdomyosarcoma: these patients will receive the standard of care treatment (vincristine, dactinomycin, and cyclophosphamide, known as VAC), along with surgery and radiation therapy. […] Intermediate-risk rhabdomyosarcoma: find out if adding a drug called liposomal irinotecan (also called Onivyde), along with the standard (VAC) chemotherapy/radiation/surgery, will be more effective than standard therapy alone. […] High-risk rhabdomyosarcoma: find out if alternating standard (VAC) chemotherapy with VLIT chemotherapy (vincristine/liposomal irinotecan/temozolomide), along with surgery and radiation therapy, will be more effective.

• #49 Rhabdomyosarcoma | Soft tissue sarcoma | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/soft-tissue-sarcoma/types/rhabdomyosarcoma

  Knowing the type of rhabdomyosarcoma helps your specialist decide on the best treatment for you. […] The type of treatment you might have depends on the level of risk of your cancer. […] Depending on the level of risk, you will have surgery. Then you may have chemotherapy on its own. Or chemotherapy alongside radiotherapy. […] You are likely to have surgery if it is possible to remove the sarcoma. […] The chemotherapy drugs most commonly used are: vincristine, actinomycin D, cyclophosphamide, ifosfamide. […] If surgery is not possible you might have radiotherapy to the sarcoma. […] Your treatment depends on where in the body the rhabdomyosarcoma is. Surgery is usually part of the treatment. Chemotherapy tends to work well with this type of sarcoma. […] Chemotherapy after surgery aims to help stop the cancer from coming back. This is called adjuvant treatment.

• #50 Rhabdomyosarcoma in Children – In Treatment | CureSearch

  https://curesearch.org/Rhabdomyosarcoma-In-Treatment/

  All children with rhabdomyosarcoma require a local control measure, which means direct treatment specifically to the site of the tumor. […] Local control can be done by surgery alone, radiation alone, or a combination of both surgery and radiation. […] The best local control option depends on the location of the tumor, how easily it can be removed with surgery, and the results of any surgery performed. […] If embryonal rhabdomyosarcoma is removed completely by surgery, and the pathologist determines the margins are clean, or negative (no cancer cells are present at the edges of the removed mass), then no further treatment measures may be necessary. In many other cases, combination with radiation therapy may be used to minimize the risk of tumor recurrence or relapse. […] For this reason, chemotherapy should be used to treat all patients with RMS with the aim of eliminating micrometastatic tumor cells and decrease the likelihood of recurrence. Many different types of chemotherapy are used, and usually in combination. The specific combination selected depends on cell type, primary tumor location and extent of the disease.

• #51 RMS2021: Using Molecular Risk Stratification and Liposomal Irinotecan in Children with Intermediate-risk and High-risk Rhabdomyosarcoma | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/clinical-trials/rms2021-rhabdomyosarcoma.html

  Rhabdomyosarcoma (RMS) is a type of cancerous tumor found in soft tissue, such as muscles. The standard treatment for newly diagnosed rhabdomyosarcoma is chemotherapy, surgery, and radiation therapy (the use of high-dose x-rays to kill cancer cells). […] We hope to find the most effective treatment for each risk group of rhabdomyosarcoma patients. The treatment they will receive is based on the risk of their cancer returning. […] Low-risk rhabdomyosarcoma: these patients will receive the standard of care treatment (vincristine, dactinomycin, and cyclophosphamide, known as VAC), along with surgery and radiation therapy. […] Intermediate-risk rhabdomyosarcoma: find out if adding a drug called liposomal irinotecan (also called Onivyde), along with the standard (VAC) chemotherapy/radiation/surgery, will be more effective than standard therapy alone. […] High-risk rhabdomyosarcoma: find out if alternating standard (VAC) chemotherapy with VLIT chemotherapy (vincristine/liposomal irinotecan/temozolomide), along with surgery and radiation therapy, will be more effective.

• #52 Rhabdomyosarcoma: Current Therapy, Challenges, and Future Approaches to Treatment Strategies

  https://www.mdpi.com/2072-6694/15/21/5269

  The current literature fails to show a therapeutic advantage for higher doses of cyclophosphamide in children with intermediate-risk tumors. However, the COG does show improvement in the patients with disease relapse who use irinotecan and vincristine as part of their chemotherapy regimen. […] Radiation therapy plays an important role in the treatment of patients with COG group II (microscopic residual) or group III (gross residual) diseases. […] Although the 5-year survival of the patients with low-risk disease has approached 90%, children with metastatic disease have an overall survival rate of 25–30% at 3 years, despite the use of high dose of chemotherapy and stem cell rescue treatments. […] Thus, there are several agents that are currently under investigation to improve treatments for this cohort with poor survival outcomes. For example, targeted therapeutic agents such as cixutumumab, crizotinib, pazopanib, sorafenib, and temsirolimus are currently being studied for their role in RMS treatment.

• #53 Chemotherapy for rhabdomyosarcoma | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/rhabdomyosarcoma/treatment/chemotherapy

  The most common chemotherapy drugs used to treat RMS are: […] vincristine […] dactinomycin (Cosmegen) […] cyclophosphamide (Procytox) […] irinotecan […] topotecan […] ifosfamide (Ifex) […] etoposide (Vepesid) […] doxorubicin. […] A targeted therapy drug, temsirolimus (Torisel), may be given with chemotherapy for intermediate-risk RMS as part of a clinical trial. […] Cyclophosphamide and ifosfamide can irritate the bladder. When these chemotherapy drugs are used, mesna (Uromitexan) is also given to protect the bladder. […] Side effects can happen with any type of treatment for RMS, but every child’s experience is different. […] Side effects of chemotherapy will depend mainly on the type of drug, the dose, how it’s given and your child’s overall health. Some side effects of chemotherapy drugs used for RMS are:

• #54 Pediatric Rhabdomyosarcoma Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/988803-treatment

  A study by Weigel et al that enrolled 109 patients, mostly 20 years of age or younger, with stage IV (metastatic) rhabdomyosarcoma, looked to improve outcome of patients with metastatic rhabdomyosarcoma by dose intensification with interval compression, use of the most active agents determined in phase II window studies, and use of irinotecan as a radiation sensitizer. […] In a phase II trial of 87 patients with rhabdomyosarcoma who had experienced a first relapse or disease progression and whose prognosis was unfavorable, temsirolimus (Torisel) proved superior to bevacizumab (Avastin) as add-on therapy. […] A randomized, controlled study by Bisogno et al demonstrated that the addition of 6 months of maintenance chemotherapy with low-dose vinorelbine/cyclophosphamide improved the 5-year overall survival rate for patients with high-risk RMS from 73.7% to 86.5%, or an absolute 13% increase.

• #55 RMS2021: Using Molecular Risk Stratification and Liposomal Irinotecan in Children with Intermediate-risk and High-risk Rhabdomyosarcoma | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/clinical-trials/rms2021-rhabdomyosarcoma.html

  Rhabdomyosarcoma (RMS) is a type of cancerous tumor found in soft tissue, such as muscles. The standard treatment for newly diagnosed rhabdomyosarcoma is chemotherapy, surgery, and radiation therapy (the use of high-dose x-rays to kill cancer cells). […] We hope to find the most effective treatment for each risk group of rhabdomyosarcoma patients. The treatment they will receive is based on the risk of their cancer returning. […] Low-risk rhabdomyosarcoma: these patients will receive the standard of care treatment (vincristine, dactinomycin, and cyclophosphamide, known as VAC), along with surgery and radiation therapy. […] Intermediate-risk rhabdomyosarcoma: find out if adding a drug called liposomal irinotecan (also called Onivyde), along with the standard (VAC) chemotherapy/radiation/surgery, will be more effective than standard therapy alone. […] High-risk rhabdomyosarcoma: find out if alternating standard (VAC) chemotherapy with VLIT chemotherapy (vincristine/liposomal irinotecan/temozolomide), along with surgery and radiation therapy, will be more effective.

• #56 Rhabdomyosarcoma: Current Therapy, Challenges, and Future Approaches to Treatment Strategies

  https://www.mdpi.com/2072-6694/15/21/5269

  The current literature fails to show a therapeutic advantage for higher doses of cyclophosphamide in children with intermediate-risk tumors. However, the COG does show improvement in the patients with disease relapse who use irinotecan and vincristine as part of their chemotherapy regimen. […] Radiation therapy plays an important role in the treatment of patients with COG group II (microscopic residual) or group III (gross residual) diseases. […] Although the 5-year survival of the patients with low-risk disease has approached 90%, children with metastatic disease have an overall survival rate of 25–30% at 3 years, despite the use of high dose of chemotherapy and stem cell rescue treatments. […] Thus, there are several agents that are currently under investigation to improve treatments for this cohort with poor survival outcomes. For example, targeted therapeutic agents such as cixutumumab, crizotinib, pazopanib, sorafenib, and temsirolimus are currently being studied for their role in RMS treatment.

• #57 Rhabdomyosarcoma: Current Therapy, Challenges, and Future Approaches to Treatment Strategies

  https://www.mdpi.com/2072-6694/15/21/5269

  The current literature fails to show a therapeutic advantage for higher doses of cyclophosphamide in children with intermediate-risk tumors. However, the COG does show improvement in the patients with disease relapse who use irinotecan and vincristine as part of their chemotherapy regimen. […] Radiation therapy plays an important role in the treatment of patients with COG group II (microscopic residual) or group III (gross residual) diseases. […] Although the 5-year survival of the patients with low-risk disease has approached 90%, children with metastatic disease have an overall survival rate of 25–30% at 3 years, despite the use of high dose of chemotherapy and stem cell rescue treatments. […] Thus, there are several agents that are currently under investigation to improve treatments for this cohort with poor survival outcomes. For example, targeted therapeutic agents such as cixutumumab, crizotinib, pazopanib, sorafenib, and temsirolimus are currently being studied for their role in RMS treatment.

• #58 Childhood Rhabdomyosarcoma Treatment – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/patient/rhabdomyosarcoma-treatment-pdq

  Treatment for childhood rhabdomyosarcoma may cause side effects. […] Treatment options for progressive or recurrent childhood rhabdomyosarcoma are based on many factors, including where in the body the cancer has come back, what type of treatment the child had before, and the needs of the child. […] Treatment of progressive or recurrent rhabdomyosarcoma may include one or more of the following: Surgery, Radiation therapy, Chemotherapy.

• #59 Childhood Rhabdomyosarcoma Treatment (PDQ®): Treatment – Patient Information [NCI] | Kaiser Permanente

  https://healthy.kaiserpermanente.org/health-wellness/health-encyclopedia/he.childhood-rhabdomyosarcoma-treatment-pdq%C2%AE-treatment-patient-information-nci.ncicdr0000258466

  Treatment options for progressive or recurrent childhood rhabdomyosarcoma are based on many factors, including where in the body the cancer has come back, what type of treatment the child had before, and the needs of the child. […] Treatment of progressive or recurrent rhabdomyosarcoma may include one or more of the following: Surgery, Radiation therapy, Chemotherapy, A clinical trial of combination chemotherapy with or without temsirolimus, A clinical trial of targeted therapy or immunotherapy (sirolimus, ipilimumab, nivolumab, or pembrolizumab).

• #60 Childhood Rhabdomyosarcoma Treatment (PDQ®): Treatment – Patient Information [NCI] – Boris Bentsianov, MD

  https://www.brooklynentdoc.com/patient-education/health-library?DOCHWID=ncicdr0000258466

  Treatment of progressive or recurrent rhabdomyosarcoma may include one or more of the following: Surgery, Radiation therapy, Chemotherapy, a clinical trial of combination chemotherapy with or without temsirolimus, a clinical trial of targeted therapy or immunotherapy, and new therapies being studied in early stage clinical trials should be considered for patients with recurrent rhabdomyosarcoma.

• #61 Pediatric Rhabdomyosarcoma Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/988803-treatment

  A study by Weigel et al that enrolled 109 patients, mostly 20 years of age or younger, with stage IV (metastatic) rhabdomyosarcoma, looked to improve outcome of patients with metastatic rhabdomyosarcoma by dose intensification with interval compression, use of the most active agents determined in phase II window studies, and use of irinotecan as a radiation sensitizer. […] In a phase II trial of 87 patients with rhabdomyosarcoma who had experienced a first relapse or disease progression and whose prognosis was unfavorable, temsirolimus (Torisel) proved superior to bevacizumab (Avastin) as add-on therapy. […] A randomized, controlled study by Bisogno et al demonstrated that the addition of 6 months of maintenance chemotherapy with low-dose vinorelbine/cyclophosphamide improved the 5-year overall survival rate for patients with high-risk RMS from 73.7% to 86.5%, or an absolute 13% increase.

• #62 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20230726/Researchers-discover-potential-new-treatment-for-rhabdomyosarcoma.aspx

  Researchers from the Leibniz Institute on Aging in Jena and the Brandenburg University of Technology Cottbus-Senftenberg have discovered a novel treatment approach for soft tissue tumors, which are frequently occurring in children and are often malignant. […] A team led by Prof. Julia von Maltzahn has discovered a new treatment approach for rhabdomyosarcoma that could be used as a complemental treatment supporting traditional chemotherapy and radiation therapy. […] Based on these promising results, we believe that we found an effective, new therapeutic approach for the treatment of embryonal rhabdomyosarcomas, which could be used in the future – in addition to traditional chemotherapy and radiotherapy – to stop the growth and spreading of the tumors. […] All indications are suggesting that TRPS1 is a promising therapeutic target with high potential to transform tumor cells into muscle cells, a cell type which cannot undergo cell divisions anymore, thus helping to stop tumor growth.

• #63 Recent Advances and Challenges in the Treatment of Rhabdomyosarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7409313/

  According to several basic and clinical studies, the immune checkpoint axis is considered a strong therapeutic target in various malignancies. […] Currently, only a few clinical studies have reported the efficacy of immune checkpoint inhibitors in patients with RMS. […] As the sample population was small, additional studies with a large number of study patients are needed to evaluate the efficacy of immune checkpoint inhibitors. […] Recent basic studies have reported promising therapeutic approaches, including ferroptotic agents, oncolytic virus, and tumor-targeting bacterial therapy. […] To minimize the incidence of treatment-related complications due to damage to normal cells, high specificity of tumor-targeting treatment is warranted.

• #64 Rhabdomyosarcoma Treatment in Mumbai, India | Nanavati Max Hospital

  https://www.nanavatimaxhospital.org/our-specialities/cancer-centre/conditions-treatments/rhabdomyosarcoma

  Newer techniques can be used to treat rhabdomyosarcoma. These include immunotherapy and targeted therapy. Immunotherapy is an upcoming modality that focuses on the patients immune system to fight against cancerous cells. Targeted therapy helps to limit the spread of cancerous cells by killing the cells while causing little or no harm to the normal cells. A few examples of targeted therapy include mTOR inhibitors such as Sirolimus and tyrosine inhibitors such as palbociclib.

• #65 A trial of treatment for children and adults with rhabdomyosarcoma (FAR-RMS) | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/find-a-clinical-trial/a-trial-of-treatment-for-children-and-adults-with-rhabdomyosarcoma-far-rms

  Your doctor will tell you about the treatment group you might be suitable to join. And how this treatment may differ from standard treatment. […] Regorafenib is a type of targeted drug called a cancer growth blocker. It stops signals that the cancer cells use to divide and grow. It also stops them from forming new blood vessels that they need to divide and grow. […] The researchers want to find out which method of giving radiotherapy works best.

• #66 Rhabdomyosarcoma | Diagnosis & Treatment | UH Rainbow Babies & Children’s Hospital | University Hospitals | Cleveland, Ohio | University Hospitals

  https://www.uhhospitals.org/rainbow/services/pediatric-cancer-and-blood-disorders/conditions-and-treatments/rhabdomyosarcoma/diagnosis-and-treatments

  Immunotherapies that have potential in the treatment rhabdomyosarcoma include: Vaccine therapy: This treatment uses a substance or group of substances to stimulate the immune system to find the tumor and kill it. Vaccine therapy is being studied to treat metastatic rhabdomyosarcoma. Immune checkpoint inhibitor therapy: This therapy utilizes the bodys immune system to kill cancer cells. Certain immune checkpoint inhibitors are being studied in the treatment of childhood rhabdomyosarcoma that returns or progresses after initial treatment. […] For the side effects of cancer and cancer treatments, your childs care team will provide medicines and other treatments to relieve pain, fever, infection, nausea and vomiting. Managing and even preventing side effects is an important component of good cancer treatment.

• #67 Freezing cancer to death: A promising new therapy for rhabdomyosarcoma – Children’s Cancer Research Fund

  https://childrenscancer.org/freezing-cancer-to-death-a-promising-new-therapy-approach-for-rhabdomyosarcoma/

  Freezing cancer to death: A promising new therapy for rhabdomyosarcoma […] But Alex Huang, MD, PhD at Case Western Reserve University thinks the method could boost immunotherapy effectiveness for kids with rhabdomyosarcoma. […] Called cryoablation, it involves inserting a wand-like needle through a patients skin and directly into the tumor. […] It can also shrink the tumor, but the tumor usually comes back, Huang said. […] This caused him to question whether cryoablation could treat tumors like rhabdomyosarcoma beyond pain relief, and he asked Huang to help him find out. […] His investigations revealed that after cryoablation treatment, the STING pathway (the part of the immune system that says, please come help) drove the rest of the immune system to fight cancer cells. […] This is remarkable because rhabdomyosarcoma typically prevents the STING pathway from doing its job, indicating that cryoablation is helping the immune system find and attack the cancer cells.

• #68 Freezing cancer to death: A promising new therapy for rhabdomyosarcoma – Children’s Cancer Research Fund

  https://childrenscancer.org/freezing-cancer-to-death-a-promising-new-therapy-approach-for-rhabdomyosarcoma/

  Freezing cancer to death: A promising new therapy for rhabdomyosarcoma […] But Alex Huang, MD, PhD at Case Western Reserve University thinks the method could boost immunotherapy effectiveness for kids with rhabdomyosarcoma. […] Called cryoablation, it involves inserting a wand-like needle through a patients skin and directly into the tumor. […] It can also shrink the tumor, but the tumor usually comes back, Huang said. […] This caused him to question whether cryoablation could treat tumors like rhabdomyosarcoma beyond pain relief, and he asked Huang to help him find out. […] His investigations revealed that after cryoablation treatment, the STING pathway (the part of the immune system that says, please come help) drove the rest of the immune system to fight cancer cells. […] This is remarkable because rhabdomyosarcoma typically prevents the STING pathway from doing its job, indicating that cryoablation is helping the immune system find and attack the cancer cells.

• #69 Freezing cancer to death: A promising new therapy for rhabdomyosarcoma – Children’s Cancer Research Fund

  https://childrenscancer.org/freezing-cancer-to-death-a-promising-new-therapy-approach-for-rhabdomyosarcoma/

  However, freezing and thawing tumor cells makes cancer cells expand, explode and spill their contents that flag them as cancer. […] The STING pathway recognizes the pieces of the cells as bad and rouses the immune system to attack the tumor cells anywhere it can find in the body, making tumors shrink or disappear for some patients. […] It has whats called an abscopal effect, Huang said, adding that cryoablations abscopal effect has already been shown to help adult cancer patients. […] Huang thinks he can effectively get rhabdomyosarcoma to attack itself by developing a vaccine in combination with turning the STING pathway switch on in rhabdomyosarcoma tumors. […] Switching the STING pathway on in tumors will make them more susceptible to immunotherapies because the body will be able to recognize the cancer.

• #70 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20230726/Researchers-discover-potential-new-treatment-for-rhabdomyosarcoma.aspx

  Researchers from the Leibniz Institute on Aging in Jena and the Brandenburg University of Technology Cottbus-Senftenberg have discovered a novel treatment approach for soft tissue tumors, which are frequently occurring in children and are often malignant. […] A team led by Prof. Julia von Maltzahn has discovered a new treatment approach for rhabdomyosarcoma that could be used as a complemental treatment supporting traditional chemotherapy and radiation therapy. […] Based on these promising results, we believe that we found an effective, new therapeutic approach for the treatment of embryonal rhabdomyosarcomas, which could be used in the future – in addition to traditional chemotherapy and radiotherapy – to stop the growth and spreading of the tumors. […] All indications are suggesting that TRPS1 is a promising therapeutic target with high potential to transform tumor cells into muscle cells, a cell type which cannot undergo cell divisions anymore, thus helping to stop tumor growth.

• #71 Understanding adult rhabdomyosarcoma: Types, prognosis and treatment | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/understanding-adult-rhabdomyosarcoma–types–prognosis-and-treatment.h00-159773289.html

  Rhabdomyosarcoma is a rare type of soft tissue sarcoma that develops in the skeletal muscles. But rhabdomyosarcoma can affect adults, too. And adult rhabdomyosarcoma is often more difficult to treat. […] Typically, we treat alveolar rhabdomyosarcoma and embryonal rhabdomyosarcoma similarly to how we treat pediatric cancers. But we may modify the treatment when they’re diagnosed in adults. This could mean changing the medicine or adjusting the dosage. […] Treatment for almost all patients with rhabdomyosarcoma child or adult, regardless of subtype typically involves: chemotherapy, surgery and/or radiation therapy. […] So, we usually do chemotherapy first to shrink the main tumor that we can see. Surgery and/or radiation may follow, depending on the cancer’s stage and grade. After that, we’ll give the patient more chemotherapy to eliminate metastasis and help prevent recurrence.

• #72 Optimizing Rhabdomyosarcoma Treatment in Adolescents and Young Adults

  https://www.mdpi.com/2072-6694/14/9/2270

  Rhabdomyosarcoma (RMS) is the most common form of soft tissue sarcoma in children, but can also develop in adolescents and young adults (AYA). The mainstay of treatment is multi-agent chemotherapy, ideally with concomitant local treatment, including surgical resection and/or radiation therapy. […] Although most treatment decisions for RMS in AYA are based on scientific evidence accumulated through clinical studies of pediatric RMS, treatment outcomes are significantly inferior in AYA patients than in children. […] Clinical trials for RMS in AYA are the best way to develop an optimal treatment. However, a well-designed clinical trial requires a great deal of time and resources, especially when targeting such a rare population. Until clinical trials are designed and implemented, and their findings duly analyzed, we must provide the best possible practice for RMS treatment in AYA patients based on our own expertise in manipulating the dosage schedules of various chemotherapeutic agents and administering local treatments in a manner appropriate for each patient.

• #73 Rhabdomyosarcoma in adults: Case series | IJWH

  https://www.dovepress.com/rhabdomyosarcoma-in-adults-case-series-and-literature-review-peer-reviewed-fulltext-article-IJWH

  There are no definitive, optimal regimens for the management of rhabdomyosarcoma in adults. […] All patients require multimodal treatment planning (surgery, radiotherapy, and chemotherapy) and risk stratification. […] Combination chemotherapy, which is used to reduce the size of the primary tumor and to eradicate gross or microscopic metastatic foci, is the mainstay treatment in children. […] Surgery is the primary treatment for patients with pleomorphic rhabdomyosarcoma (74% vs 34% for non-pleomorphic histology), and the event-free survival rate was 37% for patients who underwent complete resection compared to 0% in patients with unresectable tumors. […] In terms of radiotherapy, this treatment is beneficial to patients with advanced disease. […] After surgery, chemotherapy and/or radiotherapy will be continued for this patient.

• #74 Rhabdomyosarcoma in adults: Case series | IJWH

  https://www.dovepress.com/rhabdomyosarcoma-in-adults-case-series-and-literature-review-peer-reviewed-fulltext-article-IJWH

  The prognosis of adults is significantly worse than that of children, and the reasons for this continued deterioration in outcome remain largely unknown. […] The expression of multidrug resistance protein is more obvious in adult patients, and the lower tolerance of adults to intensive treatment may be the reason of poor prognosis in adults with rhabdomyosarcoma. […] Additional studies on the management plan and the pathogenesis of rhabdomyosarcoma in adults are needed.

• #75 Rhabdomyosarcoma in adults: Case series | IJWH

  https://www.dovepress.com/rhabdomyosarcoma-in-adults-case-series-and-literature-review-peer-reviewed-fulltext-article-IJWH

  The prognosis of adults is significantly worse than that of children, and the reasons for this continued deterioration in outcome remain largely unknown. […] The expression of multidrug resistance protein is more obvious in adult patients, and the lower tolerance of adults to intensive treatment may be the reason of poor prognosis in adults with rhabdomyosarcoma. […] Additional studies on the management plan and the pathogenesis of rhabdomyosarcoma in adults are needed.

• #76 Treating Rhabdomyosarcoma | Treatment for Rhabdomyosarcoma | American Cancer Society

  https://www.cancer.org/cancer/types/rhabdomyosarcoma/treating.html

  Adults with cancer also typically have the option to participate in clinical trials as a way to get state-of-the art cancer treatment. […] Be sure to talk to your cancer care team about any method you are thinking about using. They can help you learn what is known (or not known) about the method, which can help you make an informed decision. […] Before treatment, the doctors and other members of the team will help you understand the tests that will need to be done. […] Your cancer care team will be your first source of information and support, but there are other resources for help when you need it.

• #77 Rhabdomyosarcoma Treatment | St. Jude Care & Treatment

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

  St. Jude provides the highest quality of care for patients with rhabdomyosarcoma: […] A study by St. Jude and Washington University Pediatric Cancer Genome Project study found drugs that killed rhabdomyosarcoma tumor cells growing in the lab. These drugs may help chemotherapy work better. […] St. Jude researchers are using a new combination of drugs after all therapy has been finished. They want to see if this new combination can improve the survival of children with high-risk features. […] The St. Jude medical team is using new types of imaging such as diffusion-weighted MRI to see how tumors respond to treatment. […] St. Jude scientists are trying to find the most effective treatment with the least amount of therapy that will cure children with low-risk rhabdomyosarcoma. […] Rhabdomyosarcoma treatment at St. Jude may involve proton therapy. St. Jude has the world’s first proton therapy center dedicated solely to the treatment of children. Proton therapy can deliver high radiation doses directly to tumors. This treatment spares normal tissues and reduces the side effects of traditional x-ray therapy. Because it is so precise, proton therapy reduces a child’s risk of having serious side effects, including other cancers later in life.

• #78 UCSF Rhabdomyosarcoma Clinical Trials for 2025 — San Francisco Bay Area

  https://clinicaltrials.ucsf.edu/rhabdomyosarcoma

  Rhabdomyosarcoma is a cancer affecting soft tissues in the body. UCSF is conducting trials to find effective chemotherapy for low-risk rhabdomyosarcoma. Additionally, UCSF is exploring the safety of a flavored oral drug combined with temozolomide for children with recurring solid tumors. […] This phase III trial aims to maintain excellent outcomes in patients with very low risk rhabdomyosarcoma (VLR-RMS) while decreasing the burden of therapy using treatment with 24 weeks of vincristine and dactinomycin (VA) and examines the use of centralized molecular risk stratification in the treatment of rhabdomyosarcoma. […] This phase III trial compares the safety and effect of adding vinorelbine to vincristine, dactinomycin, and cyclophosphamide (VAC) for the treatment of patients with high risk rhabdomyosarcoma (RMS).

• #79 UCSF Rhabdomyosarcoma Clinical Trials for 2025 — San Francisco Bay Area

  https://clinicaltrials.ucsf.edu/rhabdomyosarcoma

  This randomized phase III trial studies how well combination chemotherapy (vincristine sulfate, dactinomycin, cyclophosphamide alternated with vincristine sulfate and irinotecan hydrochloride or vinorelbine) works compared to combination chemotherapy plus temsirolimus in treating patients with rhabdomyosarcoma (cancer that forms in the soft tissues, such as muscle), and has an intermediate chance of coming back after treatment (intermediate risk). […] This phase II trial studies how well cabozantinib-s-malate works in treating younger patients with sarcomas, Wilms tumor, or other rare tumors that have come back, do not respond to therapy, or are newly diagnosed. […] This phase I/II trial tests the safety, best dose, and whether elimusertib works in treating patients with solid tumors that have come back (relapsed) or does not respond to treatment (refractory).

• #80 A trial of treatment for children and adults with rhabdomyosarcoma (FAR-RMS) | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/find-a-clinical-trial/a-trial-of-treatment-for-children-and-adults-with-rhabdomyosarcoma-far-rms

  This trial is looking at new ways of treating rhabdomyosarcoma in children and adults. […] The main treatments include chemotherapy, surgery and radiotherapy. Often you have a combination of these. […] Researchers are looking for ways to improve treatment for people with rhabdomyosarcoma. To do this they are looking at whether changes to chemotherapy and radiotherapy can improve treatment outcomes. […] The trial is trying to answer a number of questions about the treatment of RMS. These include looking at: new combinations of chemotherapy as initial treatment (induction), the timing, dose and extent of radiotherapy, chemotherapy to keep the cancer under control after initial chemotherapy. This is called maintenance treatment, a new combination of drugs for sarcoma that has come back (relapsed).

• #81 Treating Rhabdomyosarcoma | Treatment for Rhabdomyosarcoma | American Cancer Society

  https://www.cancer.org/cancer/types/rhabdomyosarcoma/treating.html

  The treatment for RMS can often be effective, but it can also cause serious side effects. Its important to discuss all treatment options as well as their possible side effects with the cancer care team so you can make an informed decision. […] If time allows, getting a second opinion from another doctor experienced with your childs type of tumor is often a good idea. […] The treatment team will also help you take care of side effects and can help you work closely with nutritionists, psychologists, social workers, and other professionals to understand and deal with medical problems, stress, and other issues related to the treatment. […] Today, most children and teens with cancer are treated at specialized childrens cancer centers. These centers offer the most up-to-date-treatment by conducting clinical trials (studies of promising new therapies).

• #82 Rhabdomyosarcoma | Diagnosis & Treatment | UH Rainbow Babies & Children’s Hospital | University Hospitals | Cleveland, Ohio | University Hospitals

  https://www.uhhospitals.org/rainbow/services/pediatric-cancer-and-blood-disorders/conditions-and-treatments/rhabdomyosarcoma/diagnosis-and-treatments

  Immunotherapies that have potential in the treatment rhabdomyosarcoma include: Vaccine therapy: This treatment uses a substance or group of substances to stimulate the immune system to find the tumor and kill it. Vaccine therapy is being studied to treat metastatic rhabdomyosarcoma. Immune checkpoint inhibitor therapy: This therapy utilizes the bodys immune system to kill cancer cells. Certain immune checkpoint inhibitors are being studied in the treatment of childhood rhabdomyosarcoma that returns or progresses after initial treatment. […] For the side effects of cancer and cancer treatments, your childs care team will provide medicines and other treatments to relieve pain, fever, infection, nausea and vomiting. Managing and even preventing side effects is an important component of good cancer treatment.

• #83 Rhabdomyosarcoma | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/r/rhabdomyosarcoma

  Rhabdomyosarcoma Treatment may involve surgery, radiation therapy and chemotherapy alone or in combination. […] In some situations, the doctor may recommend proton therapy instead of traditional radiation therapy. Proton therapy targets the tumor while avoiding organs and healthy tissue. This means fewer short-term side effects and long-term complications from radiation. Cincinnati Childrens is one of only a few pediatric hospitals in the country that offer proton therapy. […] Your child may receive treatment as an inpatient and / or as an outpatient, depending on the stage of the disease and the phase of therapy. We will do periodic blood tests to check white blood cells, red blood cells and platelet counts, and test for any signs of returning tumor. Every few months, we will obtain imaging of the lungs, liver and bones.

• #84 Embryonal rhabdomyosarcoma | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/embryonal-rhabdomyosarcoma/

  Researchers have been learning more about treatments known as targeted therapies and immunotherapies. Targeted therapy is a treatment that targets specific characteristics within cancer cells. It does this to stop them from growing and spreading. Immunotherapy is a treatment that helps a persons immune system fight and kill cancer cells. […] Your doctor may offer you an opportunity to take part in a trial. The trial might investigate new diagnosis methods, drugs, or treatments. Some studies also look at the care and well-being of patients. Your doctor or nurse can give you more information on taking part in a clinical trial. […] After treatment, you will have regular follow-up appointments for several years. You should receive a follow-up schedule from your sarcoma clinical nurse specialist. The follow-ups will usually include: A chance to discuss symptoms. An examination to look for any signs of ERMS returning. This may include scans such as CT or MRI scans. A chest x-ray and bone scans to rule out any secondary cancers occurring in the lungs or bone marrow. […] If the cancer does reappear, it is important to get treated as quickly as possible. This could involve further surgery and/or radiotherapy; your treatment will be assessed on an individual basis.

• #85 Rhabdomyosarcoma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/rhabdomyosarcoma/symptoms-causes/syc-20390962

  Rhabdomyosarcoma treatment often involves surgery, chemotherapy and radiation therapy. Treatment depends on where the cancer starts, how large it grows and whether it spreads to other parts of the body. […] Research into diagnosis and treatment have greatly improved the outlook for people diagnosed with rhabdomyosarcoma. More and more people are living for years after a rhabdomyosarcoma diagnosis. […] Complications of rhabdomyosarcoma and its treatment include: Cancer that spreads. Rhabdomyosarcoma can spread from where it started to other parts of the body. When cancer spreads, it might require more-intense treatments. This can make recovery harder. Rhabdomyosarcoma most often spreads to the lungs, lymph nodes and bones. […] Long-term side effects. Rhabdomyosarcoma and its treatments can cause many side effects, both short and long term. Your healthcare team can help you manage the side effects that happen during treatment. And the team can give you a list of side effects to watch for in the years after treatment.

• #86 Rhabdomyosarcoma in childhood, adolescence, and adulthood: Treatment – UpToDate

  https://www.uptodate.com/contents/rhabdomyosarcoma-in-childhood-adolescence-and-adulthood-treatment

  These improved outcomes are the direct result of multimodality therapeutic protocols that have been developed by large international cooperative groups, such as the Intergroup Rhabdomyosarcoma Study Group (IRSG, or the Soft Tissue Sarcoma Committee of the Children’s Oncology Group [COG]). Modern treatment for RMS includes chemotherapy for primary cytoreduction and eradication of both macroscopic and microscopic metastatic disease; surgery, if feasible; and radiation therapy (RT) to control microscopic local residual disease.

• #87 Childhood Rhabdomyosarcoma Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Health Information Library | PeaceHealth

  https://www.peacehealth.org/medical-topics/id/ncicdr0000062792

  All patients require chemotherapy, and at least 85% of patients also benefit from radiation therapy, with favorable outcomes even for patients with nonresectable disease. […] The extent of disease after the primary surgical procedure is correlated with outcome. […] Rhabdomyosarcoma is usually curable in children with localized disease who receive combined-modality therapy, with more than 70% of patients surviving 5 years after diagnosis. […] The prognosis for children or adolescents with rhabdomyosarcoma is related to many clinical and biological factors, including age, site of origin, tumor size, resectability, histopathological subtype, and response to therapy. […] Because treatment and prognosis partly depend on the histology and molecular characterization of the tumor, it is necessary that the tumor tissue be reviewed by expert pathologists with experience in the evaluation and diagnosis of tumors in children.

• #88 Rhabdomyosarcoma: Current Therapy, Challenges, and Future Approaches to Treatment Strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10650215/

  Rhabdomyosarcoma (RMS) is a rare pediatric sarcoma affecting skeletal muscle in children and young adults. It is responsible for 3% of all childhood malignant tumors and is the third most prevalent pediatric extracranial solid tumor. Despite advances in diagnostic and treatment methods and clinical trials to improve pediatric RMS survival rates, children with high-risk RMS and recurrent disease have 5-year survival rates of less than 30% and 17%, respectively. The cure rate remains low and the current RMS therapies continue to pose potential life-threatening toxicities, which can lead to lifelong morbidity. The treatment strategies for RMS include multi-agent chemotherapies after surgical resection with or without radiotherapy. […] The treatment strategies for rhabdomyosarcoma include multi-agent chemotherapies after surgical resection with or without ionization radiotherapy. In this comprehensive review, we first provide a detailed clinical understanding of rhabdomyosarcoma including its classification and subtypes, diagnosis, and treatment strategies. Later, we focus on chemotherapy strategies for this childhood sarcoma and discuss the impact of three mechanisms that are involved in the chemotherapy response including apoptosis, macro-autophagy, and the unfolded protein response. Finally, we discuss in vivo mouse and zebrafish models and in vitro three-dimensional bioengineering models of rhabdomyosarcoma to screen future therapeutic approaches and promote muscle regeneration.

• #89 Rhabdomyosarcoma: Current Therapy, Challenges, and Future Approaches to Treatment Strategies

  https://www.mdpi.com/2072-6694/15/21/5269

  Rhabdomyosarcoma (RMS) is a rare pediatric sarcoma affecting skeletal muscle in children and young adults. It is responsible for 3% of all childhood malignant tumors and is the third most prevalent pediatric extracranial solid tumor. Despite advances in diagnostic and treatment methods and clinical trials to improve pediatric RMS survival rates, children with high-risk RMS and recurrent disease have 5-year survival rates of less than 30% and 17%, respectively. The cure rate remains low and the current RMS therapies continue to pose potential life-threatening toxicities, which can lead to lifelong morbidity. The treatment strategies for RMS include multi-agent chemotherapies after surgical resection with or without radiotherapy. Here, we focus on chemotherapy strategies and discuss the impact of apoptosis, autophagy, and the UPR that are involved in the chemotherapy response.

• #90 Understanding adult rhabdomyosarcoma: Types, prognosis and treatment | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/understanding-adult-rhabdomyosarcoma–types–prognosis-and-treatment.h00-159773289.html

  Adult rhabdomyosarcoma is curable. At MD Anderson, we use a multi-modality treatment approach with the intent to cure the disease. […] We are trying to determine the best standard treatment for older adults with rhabdomyosarcoma. […] Im also leading a clinical trial to see if T-cell therapy can be used to treat patients with advanced or metastatic rhabdomyosarcoma. […] Any patient diagnosed with adult rhabdomyosarcoma or suspected of having this diagnosis should seek care at a sarcoma center that specializes in seeing and treating this disease.

• #91 Rhabdomyosarcoma > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/rhabdomyosarcoma

  Doctors almost always recommend surgery to remove the tumor, plus a margin of healthy tissue. […] After surgery, doctors may order radiation therapy to kill any remaining cancer cells. […] Chemotherapy is nearly always prescribed after surgery for rhabdomyosarcomait helps to kill off any cancer cells that may have been left behind in the body. […] Other treatments may be available via clinical trials at Yale, such as the use of precision medicine to guide target and biologic therapies. […] At Yale, we treat children and adults with rhabdomyosarcoma through our Solid Tumor Program, which includes oncologists specializing in the treatment of sarcoma, along with surgeons, radiation oncologists, pathologists, and specialized radiologists, says Dr. Vasquez. […] We work together closely to make the diagnosis and determine the best course of treatment individualized to each patients disease and with the most advanced options possible. […] Clinical trials for rhabdomyosarcoma may involve the study of new drugs or examine the most effective treatments with the least amount of side effects.

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