Materiały źródłowe

• #1 Malignant peripheral nerve sheath tumors – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/malignant-peripheral-nerve-sheath-tumors/diagnosis-treatment/drc-20362619

  Treatment for malignant peripheral nerve sheath tumors often involves: […] Surgery. The goal of surgery is to remove the tumor and some of the healthy tissue around it. When that can’t be done, surgeons remove as much of the tumor as they can. […] Sometimes, radiation before surgery might shrink a tumor. That might make it more likely that all of the tumor is removed during surgery. […] Radiation therapy. Radiation therapy uses powerful energy beams to kill cancer cells. The energy can come from X-rays, protons or other sources. […] Radiation may be used before surgery to shrink a tumor. This might make it more likely that all of the tumor is removed during surgery. After surgery, radiation therapy can be used to kill any cancer cells that might remain. […] Chemotherapy. Chemotherapy uses strong medicines to kill cancer cells. Chemotherapy might be an option when a malignant peripheral nerve sheath tumor has spread to other parts of the body. Chemotherapy might help control symptoms and slow the growth of the cancer. […] Rehabilitation. After surgery, physical therapists and occupational therapists can help you regain function and movement lost due to nerve damage or from removing an arm or leg.

• #2 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9954030/

  There are limited treatment options for MPNST, and the only effective treatment is complete surgical resection to achieve negative margins. […] According to data, gross-total resection results in lower recurrence and higher five-years survival rates. […] Although the recurrence rate remains very high and post-operative morbidity is significant, the advantages of an aggressive surgical approach outweigh the disadvantages. […] Chemotherapy is an alternative option for those with unresectable or metastatic MPNST. […] Doxorubicin is a first-line treatment for MPNST, and Kroep et al. reported that a doxorubicin-ifosfamide combination regimen provided the best response in MPNST. […] Radiation therapy is often recommended for high-grade lesions or tumors larger than 5 cm. […] However, adjuvant radiation therapy is not beneficial in MPNST survival, even though, in some studies, adjuvant radiation has been used to reduce the tumor size in order to make surgery possible.

• #2 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9954030/

  Targeted therapy is the way forward for patients with unresectable or metastatic MPNST. […] Complete surgical resection remains the most effective treatment for MPNST. A combination regimen of chemotherapy, radiation, and targeted therapy may achieve better survival, but more consensus is needed and toxicity should also be carefully assessed.

• #2 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9954030/

  Malignant peripheral nerve sheath tumor (MPNST) is a soft tissue sarcoma with limited therapeutic interventions and a poor prognosis. This review summarized the current understanding of the pathogenic mechanisms behind MPNST and the latest concepts in clinical management from diagnosis to therapeutic intervention. Additionally, the developments in molecular diagnosis and targeted therapies for MPNST are highlighted. It concluded with the challenges and prospects of MPNST management. […] Malignant peripheral nerve sheath tumor (MPNST) is an aggressive soft tissue sarcoma with limited therapeutic options and a poor prognosis. […] This review summarized the current understanding of MPNST and the latest consensus from its diagnosis to treatment, with highlights on molecular biomarkers and targeted therapies. Additionally, we discussed the current challenges and prospects for MPNST management.

• #3 Malignant Peripheral Nerve Sheath Tumor – NCI

  https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/mpnst

  Surgery: Once MPNST is diagnosed, you may have surgery to remove the tumor and surrounding tissue. But sometimes surgery is not an option. In that case, your doctor will discuss other options with you. […] Radiation therapy: Radiation therapy can be used before and after surgery. […] Chemotherapy: If the tumor cant be safely removed by surgery, chemotherapy can be used to shrink it and make it easier to remove. Sometimes chemotherapy is used with radiation. Chemotherapy can also be used when MPNST has spread to other parts of the body. […] It is important to talk with a team of specialists to decide the right treatment for you. […] It is very important to work with a team of experts as soon as possible after diagnosis to improve your chances of survival.

• #4 Malignant peripheral nerve sheath tumour (MPNST) | Soft tissue sarcoma | Cancer Research UK

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

  Malignant peripheral nerve sheath tumours can be difficult to treat. […] The main treatment is surgery. Your surgeon aims to remove as much of the cancer as possible and the surrounding tissue. […] You might have radiotherapy before surgery. Radiotherapy aims to shrink the cancer, making it easier to remove. This is called neoadjuvant treatment. […] Radiotherapy after surgery can kill any cancer cells left behind. This when your surgeon wasn’t able to remove all the cancer. It also aims to lower the risk of sarcoma coming back. This is called adjuvant treatment. […] Malignant peripheral nerve sheath tumours don’t respond very well to chemotherapy. Chemotherapy may be used to try to shrink the tumour or slow its growth, but it is usually unlikely to cure it. […] You might have treatment with a targeted drug as part of a clinical trial. Talk to your specialist to find out more.

• #4 Malignant peripheral nerve sheath tumour (MPNST) | Soft tissue sarcoma | Cancer Research UK

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

  A malignant peripheral nerve sheath tumour can come back in the same place. This is called local recurrence. […] It can be more difficult to treat if the sarcoma comes back. You might have surgery again if it is possible to remove the new tumour. You might also have radiotherapy again depending on the previous radiotherapy dose.

• #5 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management

  https://www.mdpi.com/2072-6694/15/4/1077

  Malignant peripheral nerve sheath tumor (MPNST) is a soft tissue sarcoma with limited therapeutic interventions and a poor prognosis. This review summarized the current understanding of the pathogenic mechanisms behind MPNST and the latest concepts in clinical management from diagnosis to therapeutic intervention. Additionally, the developments in molecular diagnosis and targeted therapies for MPNST are highlighted. It concluded with the challenges and prospects of MPNST management. […] There are limited treatment options for MPNST, and the only effective treatment is complete surgical resection to achieve negative margins. According to data, gross-total resection results in lower recurrence and higher five-years survival rates. Additionally, patients with negative tumor margins have a relatively higher survival rate than those with positive margins.

• #5 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management

  https://www.mdpi.com/2072-6694/15/4/1077

  Chemotherapy is an alternative option for those with unresectable or metastatic MPNST. According to the SARC006 prospective study, chemotherapy with adriamycin and ifosfamide resulted in a minimal response, whereas sporadic MPNST responded better than NF1-associated MPNST. Doxorubicin is a first-line treatment for MPNST, and Kroep et al. reported that a doxorubicin–ifosfamide combination regimen provided the best response in MPNST. […] Radiation therapy is often recommended for high-grade lesions or tumors larger than 5 cm. The long-term outcome of radiation therapy results in excellent local control. However, adjuvant radiation therapy is not beneficial in MPNST survival, even though, in some studies, adjuvant radiation has been used to reduce the tumor size in order to make surgery possible.

• #5 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management

  https://www.mdpi.com/2072-6694/15/4/1077

  Targeted therapy is the way forward for patients with unresectable or metastatic MPNST. Several clinical trials for targeted therapies have been conducted as our understanding of the molecular pathogenesis of MPNST has increased. EGFR inhibitor is one of the earliest attempts at MPNST therapy, as preclinical studies demonstrated that NF1/p53 murine MPNSTs in vitro are stimulated by EGF and inhibited by EGFR inhibitors. However, in a 22-month phase II clinical trial of 24 patients, 19 of 20 evaluable patients did not respond to erlotinib (an EGFR inhibitor), and six patients experienced grade 3 toxicities. […] Complete surgical resection remains the most effective treatment for MPNST. A combination regimen of chemotherapy, radiation, and targeted therapy may achieve better survival, but more consensus is needed and toxicity should also be carefully assessed.

• #6 The Multimodality Management of Malignant Peripheral Nerve Sheath Tumours

  https://www.mdpi.com/2072-6694/16/19/3266

  The landscape of malignant peripheral nerve sheath tumours (MPNSTs) is usually challenging both in terms of recognition and management. […] The primary therapeutic approach is most often radical surgery, with non-surgical modalities playing an important role, especially in locally advanced or metastatic cases. […] The aim of multimodality approaches is to optimize both local and systemic control while keeping to a minimum acute and late treatment morbidity. […] For localized MPNST, complete surgical excision with clear margins is a pivotal element of potentially curative management. […] Current guidelines recommend limb-preserving surgery (LPS); nevertheless, up to 5–10% will undergo an amputation due to a more conservative approach not being feasible. […] Pre- or post-operative radiotherapy has a significant role in localized disease by reducing the risk of local relapse in MPNSTs of the extremities, trunk or head and neck, especially when there are close margins or R1 on the surgical specimen.

• #6 The Multimodality Management of Malignant Peripheral Nerve Sheath Tumours

  https://www.mdpi.com/2072-6694/16/19/3266

  The long-term outcome of RT results in excellent local control and improved progression-free survival (PFS), but unclear benefit on survival. […] There is limited available data about the role of chemotherapy in the neo-/adjuvant setting. […] The use of peri-operative chemotherapy should be considered on a case-by-case basis. […] For inoperable or metastatic MPNST, the outcome is usually poor. The standard of care in this setting remains chemotherapy. […] While an increasing amount is understood around the molecular pathology of MPNST, little progress has been made in developing molecularly targeted therapies. […] Given the poor outcome with available systemic treatments, clinical trials for MPNST are encouraged. […] Despite ongoing clinical trials, complete surgical resection remains the most efficient treatment option for MPNST.

• #7

  https://www.aurorahealthcare.org/services/neuroscience/brain-skull-base-care/peripheral-nerve-tumors/malignant-peripheral-nerve-sheath-tumor

  The primary goal of malignant peripheral nerve sheath tumor treatment is tumor removal. Surgery aims to remove as much of the tumor as possible while avoiding damage to the affected nerves. When an MPNST affects an arm or a leg, amputation is sometimes needed. […] In cases where complete removal of the tumor isn’t feasible, chemotherapy or radiation therapy may be used before surgery to shrink the tumor and make it easier to remove. Post-surgery, these treatments may also target any remaining tumor or cancer that has spread to other parts of the body. […] MPNST cancers tend to return after being removed through surgery or other treatments. When they return, theyre also known to spread (metastasize) to the lungs. If your cancer recurs, your team of caregivers will work with you to decide what to do next.

• #8 Nerve Sheath Tumors: Definition & Types

  https://my.clevelandclinic.org/health/diseases/22526-nerve-sheath-tumors

  If a nerve sheath tumor isnt causing symptoms, your healthcare provider may choose to watch and wait. Routine monitoring will reveal if the tumor is growing or changing. […] For symptomatic tumors, or if you want the tumor removed for cosmetic reasons, surgery is usually the only option. […] In addition to surgery, treatment for malignant peripheral nerve sheath tumors may include cancer therapies such as radiation therapy and chemotherapy. […] Surgery involving the nerves also carries a risk of nerve damage and permanent disability. Your healthcare team will work with you after surgery to manage any lasting disability challenges. Different types of therapy (physical, occupational and speech) may help you regain function. […] Malignant peripheral nerve sheath tumors have a poor prognosis, especially if the tumor is larger than 2 inches. Fewer than half of people with this condition live five years after diagnosis.

• #9 Malignant peripheral nerve sheath tumors | Altru Health System

  https://www.altru.org/health-library/conditions/malignant-peripheral-nerve-sheath-tumors

  Chemotherapy. Chemotherapy uses strong medicines to kill cancer cells. Chemotherapy might be an option when a malignant peripheral nerve sheath tumor has spread to other parts of the body. Chemotherapy might help control symptoms and slow the growth of the cancer. […] Rehabilitation. After surgery, physical therapists and occupational therapists can help you regain function and movement lost due to nerve damage or from removing an arm or leg.

• #9 Malignant peripheral nerve sheath tumors | Altru Health System

  https://www.altru.org/health-library/conditions/malignant-peripheral-nerve-sheath-tumors

  Surgery is the usual treatment for malignant peripheral nerve sheath tumors. Sometimes, treatment might include radiation therapy and chemotherapy. […] Treatment for malignant peripheral nerve sheath tumors often involves: […] Surgery. The goal of surgery is to remove the tumor and some of the healthy tissue around it. When that can’t be done, surgeons remove as much of the tumor as they can. […] Sometimes, radiation before surgery might shrink a tumor. That might make it more likely that all of the tumor is removed during surgery. […] Radiation therapy. Radiation therapy uses powerful energy beams to kill cancer cells. […] Radiation may be used before surgery to shrink a tumor. This might make it more likely that all of the tumor is removed during surgery. After surgery, radiation therapy can be used to kill any cancer cells that might remain.

• #10 Peripheral nerve tumors – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/peripheral-nerve-tumors/diagnosis-treatment/drc-20355075

  Stereotactic radiosurgery is used to treat some peripheral nerve tumors in or around the brain. Radiation is delivered precisely to a tumor without making an incision. One type of this kind of surgery is called Gamma Knife radiosurgery. […] Cancerous tumors are treated with standard cancer therapies. These include surgery, chemotherapy and radiation therapy. Early diagnosis and treatment are the most important factors for a good outcome. Tumors may come back after treatment. […] After surgery, you may need physical rehabilitation. Your health care provider may use a brace or a splint to keep your arm or leg in a position that helps you to heal. Physical therapists and occupational therapists can help you recover function and mobility lost due to nerve damage or limb amputation.

• #11 Malignant peripheral nerve sheath tumors | Health Library | Memorial Health System

  https://www.mhsystem.org/health-library/con-20313998/

  These cancers form in the linings of nerves. Treatment includes surgery, radiation therapy and, sometimes, chemotherapy. […] Surgery is the usual treatment for malignant peripheral nerve sheath tumors. Sometimes, treatment might include radiation therapy and chemotherapy. […] Treatment for malignant peripheral nerve sheath tumors often involves: […] Surgery. The goal of surgery is to remove the tumor and some of the healthy tissue around it. When that cant be done, surgeons remove as much of the tumor as they can. […] Sometimes, radiation before surgery might shrink a tumor. That might make it more likely that all of the tumor is removed during surgery. […] Radiation therapy. Radiation therapy uses powerful energy beams to kill cancer cells. […] Radiation may be used before surgery to shrink a tumor. This might make it more likely that all of the tumor is removed during surgery. After surgery, radiation therapy can be used to kill any cancer cells that might remain.

• #12 A Rare Case of Malignant Peripheral Nerve Sheath Tumor in Neck and Role of VMAT Radiotherapy

  https://www.ijhns.com/abstractArticleContentBrowse/IJHNS/31747/JPJ/fullText

  Malignant peripheral nerve sheath tumor (MPNST) is a rare and aggressive variety of sarcoma. […] The aim of this paper is to present the clinicopathological features with the main focus on the role of VMAT radiotherapy, which decreases recurrence and increases survival. […] The patient was then planned for radiotherapy by VMAT to a dose of 60 Gy/30 fractions (Fr) @1 Fr/day and 5 Fr/week to the tumor bed. […] Due to its large size and aggressive nature, adjuvant radiation therapy (RT) has been shown to decrease local recurrence. […] VMAT radiotherapy aims at giving radiation to the targeted area without affecting nearby critical organs or unnecessary irradiation of surrounding areas which may again lead to the development of RT-induced sarcomas in already susceptible patients like those with NF-1. […] VMAT radiotherapy is an advanced technique of radiotherapy where the radiation dose is delivered continuously as the machine rotates like an arc. […] More case reports are required to establish a standard of care, particularly radiotherapy guidelines, which have a role in all stages of MPNST.

• #13 Systemic Treatment for Advanced and Metastatic Malignant Peripheral Nerve Sheath Tumors—A Sarcoma Reference Center Experience

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7601777/

  The survival of patients with metastatic or unresectable disease is poor, with a median progression-free survival of approximately four months and overall-survival of 13 months. […] In the first line, doxorubicin-based regimens were the most commonly used in 50.4% of patients, followed by high-dose ifosfamide in 34.8% and etoposide with ifosfamide in 14.8%. […] The objective response rate to chemotherapy in the first line was 25.2% (n = 29) with 1 complete response (in patients receiving doxorubicin and ifosfamide) and 28 partial responses. […] The median PFS in the first line was 3.9 months (95% CI 2.55.4). […] Patients who received additional palliative local therapy had significantly longer median PFS8.0 vs. 3.6 months (p = 0.044) in patients treated with surgery and 9.1 vs. 3.3 months (p = 0.041) in those receiving radiotherapy.

• #13 Systemic Treatment for Advanced and Metastatic Malignant Peripheral Nerve Sheath Tumors—A Sarcoma Reference Center Experience

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7601777/

  Malignant peripheral nerve sheath tumor (MPNST) is a rare type of soft tissue sarcomas. The localized disease is usually treated with surgery along with perioperative chemo- or radiotherapy. […] The study aimed to evaluate the modes and outcomes of systemic treatment of patients with diagnosed MPNST treated in a reference center. […] Doxorubicin-based regimens were the most commonly used in the first line (50.4% of patients). […] MPNST are resistant to the majority of systemic therapies, resulting in poor survival in advanced settings. Chemotherapy with doxorubicin and ifosfamide is associated with the best response and longest PFS. Future studies and the development of novel treatment options are necessary for the improvement of treatment outcomes. […] Treatment of metastatic disease is highly challenging due to a lack of high-quality evidence on the efficacy of systemic treatments in this sarcoma type. Anthracycline-based chemotherapy is most commonly used as the first-line treatment. A combination of doxorubicin with ifosfamide has been shown to be associated with the best outcomes and is generally recommended in this situation.

• #13 Systemic Treatment for Advanced and Metastatic Malignant Peripheral Nerve Sheath Tumors—A Sarcoma Reference Center Experience

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7601777/

  The most commonly used systemic therapies in the second and third line of treatment were doxorubicin-based schemes, followed by gemcitabine-based chemotherapy. […] The median PFS was 3.7 months (95% CI 2.05.5) in the second and 3.2 (95% CI 2.35.6) in the third line. […] Overall, available therapies offer very limited benefits to patients with advanced MPNST. There is a high need for new options, preferably targeted therapies, however many already completed trials have failed to show any benefit of new compounds.

• #14 Nerve Sheath Tumor Treatment in New Jersey – Centers for Neurosurgery, Spine & Orthopedics

  https://www.cnsomd.com/surgery-conditions/nerve-tumors/nerve-sheath-tumor/

  As nerve sheath tumor growth progresses, it can enlarge to a degree when it starts to compress the nerve. As a result, a patient may feel pain, numbness, itching, burning or tingling. In that case, the doctor will consider surgery to remove the tumor cells and any other affected soft tissue. Ideally, a neurosurgeon will remove the tumor without interfering with the nerve. If the circumstances make it impossible to preserve the nerve, the doctor will attempt to repair it during the surgery. […] When neurosurgeons cannot remove the tumor surgically, chemotherapy is another treatment option. Chemotherapy is used to shrink the growth, making its removal relatively more straightforward or even unnecessary. Based on the nature of the tumor, chemotherapy can be combined with radiation for effective treatment. Chemotherapy helps particularly when treating MPNST that has spread to other body parts.

• #15 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20250128/New-treatment-identified-for-peripheral-nerve-sheath-tumors.aspx

  However, the most remarkable moment was when we observed some tumors disappearing completely with the triple MEKi-BETi-CDKi combination. […] Initially, the findings supported the compassionate use of the MEKi-BETi combination in paediatric patients with a MPNST. […] For the MEKi-BETi-CDKi combination, further preclinical studies are needed to optimise administration regimens and minimise toxic effects. […] There is still a lot to do -further preclinical data, optimized treatment regimens, reduced toxicity- but the first steps for precision medicine in treating MPNSTs in the future are already in place.

• #16 A Discussion of Malignant Peripheral Nerve Sheath Tumors (MPNSTs) – Neurofibromatosis Program

  https://www.uab.edu/medicine/nfprogram/blog/a-discussion-of-malignant-peripheral-nerve-sheath-tumors-mpnsts

  Also, immunotherapy, which trains the individuals immune system to attack tumors, is an important new area of potential treatment, though it has not yet been tested extensively on MPNSTs. […] In conclusion, because MPNSTs are difficult to treat, early detection is critically important. Individuals with NF1 should report instances of chronic pain or rapidly growing tumors. Clinical trials are focused on finding better indicators of tumors that have the potential to become malignant to facilitate early detection and treatment.

• #17 Neoadjuvant Nivolumab plus Ipilimumab for the Treatment of Malignant and Pre-Malignant Peripheral Nerve Sheath Tumors – NCIFacebookFollow on XInstagramYoutubeLinkedin

  https://www.cancer.gov/about-cancer/treatment/clinical-trials/search/v?id=NCT04465643

  Trial Status: active […] This phase I trial tests the safety, side effects and effectiveness of nivolumab plus ipilimumab in treating malignant and pre-malignant peripheral nerve sheath tumors (PNST) before primary treatment (neoadjuvant). […] Primary treatment may include surgery, chemotherapy and radiation, however there are no accepted standards of care treatment for PNST, including atypical neurofibromatous neoplasm of uncertain biologic potential (ANNUBP), a pre-malignant tumor. […] Immunotherapy with monoclonal antibodies, such as nivolumab and ipilimumab, may help the body’s immune system attack the cancer, and may interfere with the ability of tumor cells to grow and spread. […] Giving neoadjuvant nivolumab and ipilimumab may be safe, tolerable and/or effective in treating patients with malignant and pre-malignant PNST.

• #18 Rare primary intrapulmonary malignant peripheral nerve sheath tumor showing significant response to sintilimab: A case report and literature review

  https://www.spandidos-publications.com/10.3892/ol.2024.14556

  Primary pulmonary malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma with a low incidence, poor prognosis and limited treatment options. […] At present, there is still no standard treatment for MPNST. The existing treatment options are mostly based on the treatment of STS. Although surgery is the preferred treatment for MPNST, it’s difficult to achieve extended or complete resection due to its high aggressiveness. The role of radiation, chemotherapy and targeted therapy for MPNST is still limited and uncertain. […] The present study reported a case of intrapulmonary MPNST in an elderly man who received sintilimab and achieved a remarkable response. […] Sintilimab single-agent immunotherapy may be a novel treatment for pulmonary MPNST. […] The long-term efficacy of sintilimab is still being observed in the patient by performing CT examinations every three months.

• #18 Rare primary intrapulmonary malignant peripheral nerve sheath tumor showing significant response to sintilimab: A case report and literature review

  https://www.spandidos-publications.com/10.3892/ol.2024.14556

  The present study reported for the first time that sintilimab single-agent immunotherapy achieved a remarkable response of intrapulmonary MPNST. […] We are confident about the outlook of immunotherapy for MPNST and expect that the outcome of the ongoing clinical trials will contribute to the design of personalized immunotherapy.

• #19 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20231201/New-drug-combination-may-improve-treatment-for-rare-nerve-sheath-tumors.aspx

  Combining a pair of experimental drugs may help treat malignant peripheral nerve sheath tumors with fewer harmful side effects, according to preliminary animal studies led by investigators at the Johns Hopkins Kimmel Cancer Center and Johns Hopkins Drug Discovery. […] The study, published Dec. 1 in the journal Molecular Cancer Therapeutics, shows that combining an experimental drug that blocks the metabolism of the amino acid glutamine with a second experimental drug that blocks recycling of purines (building blocks of the genetic material DNA and RNA that tumors need to proliferate) reduced the size of peripheral nerve sheath tumors in mice and increased their rates of survival. […] „We need better treatments for these tumors.” […] Combining JHU395 with the classical purine antimetabolite 6-mercaptopurine (6-MP) produces a better anti-tumor response because 6-MP itself can cause liver and gastrointestinal toxicity based on the way the body metabolizes it to the active form.

• #20 Drug Combination Shows Promise in the Treatment in Malignant Peripheral Nerve Sheath Tumors – Onco’Zine

  https://www.oncozine.com/drug-combination-shows-promise-in-the-treatment-in-malignant-peripheral-nerve-sheath-tumors/

  A combination of two cancer drugs could be effective against malignant peripheral nerve sheath tumors (MPNSTs). This is a conclusion based on the outcome of a recent study led by researchers at the Johns Hopkins Kimmel Cancer Center published in Science Advances. […] To date, there are limited treatment options available, and new therapeutic strategies are desperately needed to meet the unmet medical needs of patients diagnosed with MPNST. […] Both TNO155*, a highly potent, selective, orally efficacious, first-in-class, investigational SHP2 inhibitor being developed by Novartis currently in Phase 2 studies, and ribociclib (Kisqali®; Novartis), interfere with cell growth and replication but have different mechanisms of action. However, the initial observations support advancing this combination strategy in clinical trials with patients diagnosed with MPNST and other tumors driven by a loss of NF1. […] While rare, these tumors are aggressive and notoriously hard to treat. […] “So we’re always working for better treatments,” she added. […] “Our data support SHP2 as a target for tumors driven by the loss of NF1,” Pratillas concluded.

• #21 Combination therapy for malignant peripheral nerve sheath tumors | Washington University Office of Technology Management

  https://tech.wustl.edu/tech-summary/combination-therapy-for-malignant-peripheral-nerve-sheath-tumors/

  Researchers in Angela Hirbes lab at Washington University have developed a method of treating malignant peripheral nerve sheath tumors (MPNSTs) with a combination of inhibitors. The most effective therapy is to combine an inhibitor of TYK2 with a MEK inhibitor. […] The researchers have tested the combination of TYK2 and MEK inhibitors on MPNST cells in vitro and on an NF1 model in vivo. […] Increases response to MEK inhibitor.

• #22

  https://www.advocatehealth.com/health-services/brain-spine-institute/peripheral-nerve-tumors/malignant-peripheral-nerve-sheath-tumor

  Removing the tumor is the goal of malignant peripheral nerve sheath tumor treatment. Surgery will be used to remove as much of the nerve as possible. These surgeries are complex because they need to be done while minimizing damage to the nerves. When the malignant peripheral nerve sheath tumor is on an arm or leg, amputation is sometimes needed. […] If imaging indicates the tumor cant be completely removed, you may receive chemotherapy before surgery to help shrink the tumor and make it easier to remove. Radiation therapy may also be used to shrink your tumor before surgery. […] Either chemotherapy or radiation may also be used after surgery to remove any part of the tumor that wasnt able to be removed or to treat cancer that has spread to other areas of your body. […] Treatment for cancer isnt limited to the medical side of things. We provide wellness support to help with things like: […] We provide care to enhance your quality of life during your treatment and afterward.

• #23 Outcomes of Treatment for Malignant Peripheral Nerve Sheath Tumors: Different Clinical Features Associated with Neurofibromatosis Type 1

  https://www.e-crt.org/journal/view.php?doi=10.4143/crt.2016.271

  Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of sarcoma that occur spontaneously or in association with neurofibromatosis type 1 (NF-1). This study aimed to clinically differentiate these types of MPNSTs. […] Surgery was generally performed with curative intent. Palliative surgery was performed to relieve the symptoms caused by the tumor. A biopsy alone was performed in two cases involving unresectable lesions. Radiotherapy was generally administered to patients with positive tumor margins after surgery or to reduce symptoms, such as pain or other neurologic signs. Chemotherapy was generally administered for metastatic MPNST or recurrent disease. First-line adjuvant treatments for localized MPNST were evaluated as treatment patterns. […] NF-MPNST has different clinical features from sMPNST and requires more careful management. Further study will be needed to develop specific management plans for NF-MPNST.

• #24 Treatment for Peripheral Nerve Sheath Tumors | Neurological Surgery

  https://neurosurgery.weillcornell.org/condition/peripheral-nerve-sheath-tumors/treatment-peripheral-nerve-sheath-tumors

  The best treatment for a peripheral nerve sheath tumor will depend on the imaging studies and biopsy results as well as the patients personal and family history. […] If the mass is entwined with an adjacent nerve or structure, sometimes the medical team will recommend other treatments to avoid damaging healthy tissue. […] Surgical choices depend on the size, location, rate of growth of the tumor, and whether it is benign or malignant. The advanced treatment options performed at Weill Cornell Medicine Medicine Neurological Surgery include microsurgery and stereotactic radiosurgery, with the goal of removing as much of the tumor as possible and preserving or restoring nerve function without damaging healthy tissue. […] In the rare cases when a peripheral nerve tumor is found to be malignant, Weill Cornell Medicines neurosurgeons and radiologists will team up with oncologists to determine the best course of treatment for these aggressive tumors, which may include a combination of surgery, chemotherapy, and radiation therapy.

• #25 Malignant peripheral nerve sheath tumor—from genetics to multidisciplinary treatment | Czarnecka | Oncology in Clinical Practice

  https://journals.viamedica.pl/oncology_in_clinical_practice/article/view/98716

  Malignant peripheral nerve sheath tumor (MPNST) is an aggressive soft tissue sarcoma (STS); it originates from nervous tissue and typically develops in proximity to nerve trunks in the limbs and trunk. […] The cornerstone of MPNST management involves radical surgical measures, specifically tumor excision within healthy tissue boundaries (wide local excision), which is complemented by adjuvant radiotherapy. In case of metastatic disease, palliative chemotherapy employing doxorubicin or a combination of doxorubicin and ifosfamide is utilized. Approximately 25-30% of patients experience clinical improvement after chemotherapy. […] Looking ahead, advancements in research on molecular biology may lead to the development of inhibitors demonstrating greater efficacy than traditional chemotherapy for MPNST patients. At present, ongoing clinical trials of the therapeutic management of MPNST encompass pembrolizumab, the combination of nivolumab with ipilimumab, pexydartinib (an inhibitor targeting KIT, CSF1R, and FLT3) in conjunction with sirolimus, sapanisertib (a TORC1/2 inhibitor), or LOXO-195 (an inhibitor of neurotrophic tyrosine kinase receptors NTRK type 1, 2, and 3).

• #26 New Frontiers in Therapy of Peripheral Nerve Sheath Tumors in Patients With Neurofibromatosis Type 1: Latest Evidence and Clinical Implications | Anticancer Research

  https://ar.iiarjournals.org/content/40/4/1817

  Almost all individuals with neurofibromatosis type 1 (NF1) develop peripheral nerve sheath tumors (PNSTs), mainly benign neurofibromas, however about 10% of PNSTs will undergo transformation to malignant peripheral nerve sheath tumors (MPNSTs). Surgical treatment of PNSTs has traditionally been regarded as a standard approach. […] This review presents the latest evidence and clinical implications for new therapies of PNSTs in patients with NF1 emphasizing the potential benefit from the use of Ras/MAPK pathway inhibitors, immunotherapy, chemotherapy or radiation therapy. […] The poor response to currently available therapies underlines the need for more effective, targeted treatment methods for NF1-associated PNSTs. […] In this review, we analyzed the latest evidence and clinical implications of new therapies of PNSTs in patients with NF1 emphasizing the importance of patient risk stratification to identify those who are likely to benefit from the use of Ras/MAPK pathway inhibitors, immunotherapy, chemotherapy or radiotherapy.

• #26 New Frontiers in Therapy of Peripheral Nerve Sheath Tumors in Patients With Neurofibromatosis Type 1: Latest Evidence and Clinical Implications | Anticancer Research

  https://ar.iiarjournals.org/content/40/4/1817

  The treatment objective for BPNSTs within main peripheral nerves should be lesion control and not complete ablation, since most normal fibers travel within the capsule of the tumor. […] The management of MPNST is much more aggressive than that of BPNST, so a tissue diagnosis should be received prior to percutaneous treatment. […] MEK inhibitors. Mitogen-activated protein kinase (MEK) is a key protein in the mitogen activated protein (MAP) kinase signal transduction pathway for many growth factor receptors that supply growth signals to tumor cells. […] The phase I trial of selumetinib in 24 pediatric NF1-patients (3-17 y) with inoperable PNFs showed unprecedented activity with objective responses (PNF volume decrease 20%) in 71% of enrolled children. […] The standard of care in locally advanced MPNST is to receive local disease control, primarily using operating techniques.

• #27 Malignant Peripheral Nerve Sheath Tumor (MPNST)

  https://my.clevelandclinic.org/health/diseases/malignant-peripheral-nerve-sheath-tumor-mpnst

  Malignant peripheral nerve sheath tumors (MPNSTs) affect nerves that manage your muscles and sense of touch. The most common treatment is surgery to remove the tumors, but they often come back. […] Removing tumors with surgery can cure the condition, but the tumors often come back (recur). […] Healthcare providers typically do surgery to remove a malignant peripheral nerve sheath tumor. Providers may treat MPNST by combining surgery and chemotherapy or radiation therapy. They may do radiation therapy before or after surgery. […] Researchers are studying immunotherapy or targeted therapy as potential new or additional treatments. If you have a malignant peripheral nerve sheath tumor, you may want to talk to your provider about participating in a clinical trial that’s testing new treatments. […] Surgery may cause the following complications: Reaction to general anesthesia. Excessive bleeding (hemorrhage). Pain. Surgical scars. Surgical wound infection. […] Treatments like chemotherapy or radiation may cause side effects, including: Diarrhea. Fatigue. Nausea and vomiting.

• #28 Pediatric Malignant Peripheral Nerve Sheath Tumors – Conditions and Treatments | Children’s National Hospital

  https://www.childrensnational.org/get-care/health-library/malignant-peripheral-nerve-sheath-tumors

  The treatment for malignant peripheral nerve sheath tumors continues to evolve. Patients have the best outcomes if tumors can be fully removed. This does not, however, ensure long-term disease control. Other therapies used to try to control malignant peripheral nerve sheath tumors, especially those, which are only partially resected, include multi-agent chemotherapy and radiation therapy (in selected cases). […] Children’s National Hospital in cooperation with the Children’s Oncology Group and the National Cancer Institute, are performing a series of innovative studies in attempts to create better therapies. Children’s National is collaborating with the Department of Defense Neurofibromatosis Clinical Trials Consortium.

• #29 Malignant peripheral nerve sheath tumor | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/malignant-peripheral-nerve-sheath-tumour?lang=us

  MPNST are aggressive tumors that carry a poor prognosis, with 20-25% of patients developing metastases. Overall 5-year survival for sporadic tumors is 65%. Poor prognostic factors include NF1, large size, location on the trunk, and high-grade histological features. […] MPNSTs usually arise from a large nerve, and thus usually occur close to a plexus (e.g. brachial plexus/sacral plexus), with the sciatic nerve the most commonly affected peripheral nerve.

• #30 Survival outcomes of malignant peripheral nerve sheath tumors (MPNSTs) with and without neurofibromatosis type I (NF1): a meta-analysis | World Journal of Surgical Oncology | Full Text

  https://wjso.biomedcentral.com/articles/10.1186/s12957-023-03296-z

  NF1 is associated with a substantially higher risk of all-cause and disease-specific mortality. This finding suggests that closer surveillance is required for NF1 patients at risk of developing MPNSTs. […] It is imperative, as MPNSTs are largely resistant to chemotherapy and radiotherapy, and a timely wide excision with clear margins remains the only possible curative option. […] Complete surgical extirpation with clear margins remains the only potential curative therapy for MPNST. […] Given that NF1 has been established as a significant predictor of mortality, we propose considering NF1 status when staging MPNSTs and advocate closer monitoring of these patients. […] The findings of our study suggest that NF1 patients require closer clinical follow-up to diagnose and treat these aggressive tumors early. […] As such, clinicians should consider NF1 status in staging the disease and closer monitoring of NF1 patients at risk of developing MPNSTs to enhance their survival rates through timely intervention.

• #31 Efficacy of MEK inhibition in a recurrent malignant peripheral nerve sheath tumor | npj Precision Oncology

  https://www.nature.com/articles/s41698-021-00145-8

  Following the second resection, the patient underwent 6 months of radiological surveillance but experienced a second metastatic relapse in the left 4th anterior intercostal region, adjacent to the previous chest wall resection. […] Given the multiple MAPK pathway aberrations, she was commenced on trametinib 2mg daily, achieving a complete remission of the metastatic recurrence in 3 months. […] Therapeutic decision-making was challenging in this case, but factors favoring the choice of trametinib were the biological rationale, its ability to target downstream molecular abnormalities and alleviate therapy resistance, accessibility in Australia outside a clinical trial setting, and experience in monitoring and managing drug toxicity. […] Improving our understanding of the molecular basis of MPNST through genomic, transcriptomic, drug screening responses, and tumor microenvironment approaches is critical to the discovery of new effective therapy for this challenging disease. Given the high proportion of MPNST tumors with aberrations in the MAPK pathway, treatment with MEK inhibitors may represent an effective therapy for many patients with the appropriate genotype and merits further prospective evaluation through clinical trials.

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