Materiały źródłowe

• #1 Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiative

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10093509/

  The EUropean Network for RAre CANcers (EURACAN) Task Force on Ultrarare Brain Tumors (domain 10, subdomain 10) has reviewed the evidence of diagnostic and therapeutic interventions and drawn recommendations on peripheral and cranial nerve sheath tumors. […] The Task Force has reviewed the evidence of diagnostic and therapeutic interventions, which is particularly low due to the rarity, and drawn recommendations accordingly. Tumor diagnosis is primarily based on hematoxylin and eosin-stained sections and immunohistochemistry. […] Gross-total resection is the first choice, and can be curative in benign lesions; however, the extent of resection must be balanced with preservation of nerve functioning. […] Systemic therapy should be considered in incomplete resected plexiform neurofibromas/MPNSTs. MEK inhibitor selumetinib can be used in NF1 children 2 years with inoperable/symptomatic plexiform neurofibromas, while anthracycline-based treatment is the first choice for unresectable/locally advanced/metastatic MPNST.

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

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

  At Mayo Clinic, your surgeon will talk with you about the procedure beforehand and answer any questions you may have. […] Treatment of a peripheral nerve tumor depends on the type of tumor, what nerves and other tissues it affects, and symptoms. Treatment options may include: […] Watching and waiting to see if the tumor grows may be an option if it’s in a place that makes removal difficult. Or it may be an option if the tumor is small, slow growing, and causes few or no symptoms. You’ll have regular checkups and may have MRI scans, CT scans or ultrasounds done every 6 to 12 months to see if the tumor is growing. If repeat scans show that the tumor is stable, then it may be monitored every several years. […] Some peripheral nerve tumors are removed with surgery. The goal of surgery is to take out the entire tumor without damaging nearby healthy tissue and nerves. When that isn’t possible, surgeons remove as much of the tumor as they can.

• #1 Benign peripheral nerve tumor – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/peripheral-nerve-tumors-benign/diagnosis-treatment/drc-20504138

  Treatment of peripheral nerve tumors involves either surgical removal or watching the tumor for changes. If there’s a low chance that the tumor may become cancerous and if it isn’t causing symptoms, you might not need surgery. […] Your healthcare professional also might recommend observation if your tumor is in a place that makes it hard to remove. Observation includes regular checkups and imaging tests to see if the tumor is growing. […] Surgery may be needed if there is a concern that the tumor is cancerous. The tumor also may be surgically removed if it is large or causing pain or other symptoms, such as weakness, numbness or tingling.

• #1

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

  Treatment for peripheral nerve tumors depends on the location, size, type and growth rate of the tumor. […] For some benign tumors that are small, slow-growing and not likely to cause significant symptoms, we’ll recommend ongoing observation. We’ll schedule you for regular checkups and imaging scans to make sure it’s still growing slowly. […] Surgery on peripheral nerve tumors carries risks unique to the type of tumors. For example, surgery on a vestibular schwannoma needs to be done in an area that’s hard to access and may be close to the brain stem. […] Any surgery on a peripheral nerve tumor requires your surgeon to be careful to avoid any damage to the nerve or surrounding tissues. It may be difficult to remove the entire tumor because of its size or location. […] Surgery may be recommended for some benign tumors that are associated with important functions or may affect nearby organs or tissues. In these cases, we’ll recommend surgery to remove as much of the tumor as possible.

• #1 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 peripheral nerve sheath tumor is not causing any symptoms, the medical team may suggest observation alone. […] A specialist may recommend that the tumor should be removed surgically in certain cases: Patient is in pain, The patient shows neurologic deficits, Structures near the tumor have impaired function, The tumor is growing, A biopsy reveals the peripheral nerve tumor is malignant. […] Surgical choices depend on the size, location, rate of growth of the tumor, and whether it is benign or malignant. […] Microsurgery: Surgery is the preferred treatment for most benign peripheral nerve sheath tumors, since it offers excellent results.

• #1 Effectivebenign Peripheral Nerve Sheath Tumor Treatment: Medical And Surgical Approaches

  https://drchandrilchugh.com/peripheral-nerve-tumors/benign-peripheral-nerve-sheath-tumor-treatment/

  If the tumor doesn’t grow and you don’t get more symptoms, just watching it may be enough. But, if it starts growing fast or you feel sicker, surgery could be needed. Keep an eye on it and act fast if things change. […] Patients with benign peripheral nerve sheath tumors may need surgery if they have severe symptoms or if their tumors are growing quickly. The main goal of this surgery is to fully remove the tumor. It aims to keep the healthy nerve tissue around it safe and lower the risk of nerve damage. […] The focus is on removing benign peripheral nerve sheath tumors like schwannomas. Surgeons use advanced tools to do this. They work very carefully to not harm the healthy nerves near the tumor. This type of surgery is very precise and detailed. […] High-powered microscopes and nerve stimulators are new tools that make surgery on these tumors easier. These tools help surgeons reach difficult areas more easily. With microsurgery, the goal is to fully remove the tumor without hurting the nearby healthy nerves.

• #1 Peripheral Nerve Sheath Tumors: From Preclinical Studies to Advanced Therapies | SpringerLink

  https://link.springer.com/book/10.1007/978-3-031-76382-3

  This book offers an update about peripheral nerve sheath tumors (PNST), both in their benign and malignant forms (MPNST). […] Another relevant part is reserved to the description of the most recent surgical techniques (fluorescent dye for intraoperative surgical guidance, neurophysiological monitoring, esoscopic view). […] Finally, new oral treatment for PNST related to neurocutaneous diseases (as selumetinib in plexiform neurofibromas) is described. […] MEK Inhibitors and Other Medical Therapies for Benign Peripheral Nerve Sheath Tumors […] Systemic Treatment in Malignant Peripheral Nerve Sheath Tumors: Current Practice and Future Possibilities […] Proton and Carbon Ion Therapy for MPNST.

• #1 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.

• #1 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.

• #1 Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiative

  https://www.mdpi.com/2072-6694/15/7/1930

  The EUropean Network for RAre CANcers (EURACAN) Task Force on Ultrarare Brain Tumors has reviewed the evidence of diagnostic and therapeutic interventions and drawn recommendations on peripheral and cranial nerve sheath tumors. […] The Task Force has reviewed the evidence of diagnostic and therapeutic interventions, which is particularly low due to the rarity, and drawn recommendations accordingly. Tumor diagnosis is primarily based on hematoxylin and eosin-stained sections and immunohistochemistry. […] Gross-total resection is the first choice, and can be curative in benign lesions; however, the extent of resection must be balanced with preservation of nerve functioning. […] Radiotherapy can be omitted in benign tumors after complete resection and in NF-related tumors, due to the theoretic risk of secondary malignancies in a tumor-suppressor syndrome.

• #1

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

  Malignant peripheral nerve sheath tumor treatment usually includes cancer surgery to remove as much of the tumor as possible. In extreme cases, surgery for an MPNST may require partial amputation of an affected limb. […] Radiation therapy and chemotherapy may be used before surgery to shrink the tumor, after surgery to remove the rest of the tumor, or both before and after surgery.

• #1 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.

• #1 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.

• #1 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.

• #1 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.

• #1 Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiative

  https://www.mdpi.com/2072-6694/15/7/1930

  Systemic therapy should be considered in incomplete resected plexiform neurofibromas/MPNSTs. […] MEK inhibitor selumetinib can be used in NF1 children ≥2 years with inoperable/symptomatic plexiform neurofibromas, while anthracycline-based treatment is the first choice for unresectable/locally advanced/metastatic MPNST. […] Clinical trials on other MEK1-2 inhibitors alone or in combination with mTOR inhibitors are under investigation in plexiform neurofibromas and MPNST, respectively.

• #1 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.

• #1 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 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. […] In mouse models of the condition, they found that Pro-905 in combination with JHU395 reduced tumor growth more effectively than 6-MP and with less toxicity. […] They also want to test whether this combination might be effective in patients with malignant peripheral nerve sheath tumors or if Pro-905 might be used as maintenance therapy to prevent tumor regrowth, as 6-MP is. […] Using JHU395 to cut cancer cells off from their supply of glutamine strengthens immune cells, invigorating their response to cancer, Slusher says. […] „Building on observations from one study and keeping the science moving forward can open up a lot of potential routes for future therapies for diseases that need better treatment options,” Lemberg says.

• #1 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.

• #1 Effectivebenign Peripheral Nerve Sheath Tumor Treatment: Medical And Surgical Approaches

  https://drchandrilchugh.com/peripheral-nerve-tumors/benign-peripheral-nerve-sheath-tumor-treatment/

  After surgery, patients may need rehab to get their function and movement back. The outcomes depend on the tumor’s size, where it is, and how much of it was removed. The skill of the surgical team also plays a big part in a patient’s recovery. […] How we treat benign peripheral nerve sheath tumors depends on many things. This includes size, where they are, how fast they grow, your symptoms, and general health. If a tumor is small and doesn’t cause any problems, doctors might just keep an eye on it. But, if it’s big, gives you trouble, or grows quickly, it might need to be removed with surgery. […] Doctors will try to take out the whole tumor while not hurting the good nerve around it. They also want to lower the chance of nerve damage. For some hard-to-reach tumors, doctors may use a treatment called stereotactic radiosurgery. This treatment sends strong radiation beams right at the tumor. It’s a planned approach where a team of doctors, like neurologists and neurosurgeons, will decide the best treatment for you.

• #1

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

  At Aurora Health Care, your treatment for neurological cancer will be provided by a multidisciplinary team that addresses both your medical treatment and your emotional well-being throughout treatment and recovery. We offer: Physical therapy to maintain strength and range of motion, Nutritional guidance, Spiritual and emotional counseling, Support for sexual health, Palliative care and hospice care, Integrative cancer care for stress, pain, fatigue and nerve damage. […] Your journey with Aurora Health Care is not just about treating disease but also about providing care to enhance your quality of life.

• #1 Effectivebenign Peripheral Nerve Sheath Tumor Treatment: Medical And Surgical Approaches

  https://drchandrilchugh.com/peripheral-nerve-tumors/benign-peripheral-nerve-sheath-tumor-treatment/

  Teams with experience in treating peripheral nerve tumors offer better patient care. They know how to diagnose, treat, and help patients recover from these tumors. Their experience leads to better outcomes for those affected by benign peripheral nerve sheath tumors. […] For the best results, all healthcare providers must work together. Communication and collaboration are key. This approach helps in every step of the patient’s care journey, making sure they receive the best treatment and support. It aims to improve both their health and quality of life. […] After surgery for a benign peripheral nerve sheath tumor, keeping up with postoperative care is key. Your doctors will watch you carefully for any signs of the tumor coming back. They will use regular exams and imaging tests. If the tumor does come back, more treatment like another surgery or radiation might be needed.

• #1 Effectivebenign Peripheral Nerve Sheath Tumor Treatment: Medical And Surgical Approaches

  https://drchandrilchugh.com/peripheral-nerve-tumors/benign-peripheral-nerve-sheath-tumor-treatment/

  Going to follow-up appointments and having regular scans, like MRI or CT scans, is important. This way, your healthcare team can catch any signs of the tumor early. Doing this helps ensure you get help quickly, which is good for your recovery. […] Rehabilitation and physical therapy are vital for your recovery. You might work with physical and occupational therapists to get back your strength and ability to move. This team effort in your care aims to get the best results and decrease any lasting problems. […] The treatment of benign peripheral nerve sheath tumors is getting better all the time. This is thanks to new ways of doing surgery and understanding these tumors better. Surgical techniques now include better imaging and checking the tumor as it’s removed. This helps make surgery more accurate and safer.

• #1

  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.

• #1 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.

• #1 Childhood Peripheral Nerve Sheath Tumor (Neurofibrosarcoma) | Dana-Farber Cancer Institute

  https://www.dana-farber.org/cancer-care/types/childhood-peripheral-nerve-sheath-tumor

  At Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, your child will receive care from leading cancer center doctors and world-renowned pediatric experts. Specialists in our Bone and Soft Tissue Tumors Program have extensive experience treating children with peripheral nerve sheath tumors. […] We typically perform surgery to remove the tumor and nearby tissue to treat peripheral nerve sheath tumors. In most cases, our doctors use limb-salvage surgery to preserve as much of the limb and limb function as possible. […] We may also treat patients with chemotherapy and radiation therapy before or after surgery, depending on the child’s individual needs. Your medical team will discuss every step of treatment with you and your child. […] After treatment, children treated for peripheral nerve sheath tumors continue to receive care through our pediatric cancer survivorship programs. Continued monitoring and care are essential as these tumors can recur, even after aggressive treatment.

• #1 Peripheral Neuropathy | Macmillan Cancer Support

  https://www.macmillan.org.uk/cancer-information-and-support/impacts-of-cancer/peripheral-neuropathy

  Most people find that their symptoms gradually improve with time as the nerves slowly recover. This may take several months or more. For some people, nerve damage will be permanent. […] Some types of drugs can change nerve impulses and help relieve nerve pain. Gabapentin (Neurontin) and pregabalin (Lyrica) are drugs that work in this way. […] Many hospitals have pain teams with specialist doctors and nurses who are experts in pain control. […] You can ask to be referred to an occupational therapist if you are finding it hard to do daily tasks because of peripheral neuropathy, such as washing or dressing.

• #1 Peripheral Nerve Tumors: Treatment, Managment and Support

  https://www.medicoverhospitals.in/articles/peripheral-nerve-tumors

  Support for nerve tumour patients extends beyond medical treatments. Psychological and emotional support is crucial for coping with the diagnosis and treatment process. […] A tailored care plan can significantly improve a patient’s quality of life. […] Effective pain management is a critical component of care for peripheral nerve tumour patients. […] Rehabilitation services are essential for helping patients regain function and mobility. […] Peripheral nerve tumours present a range of challenges, but with the correct information and support, patients can navigate their treatment journey more effectively. Early diagnosis, comprehensive treatment plans, and a robust support system are vital for managing these tumours. […] Management options include surgery, radiation therapy, and medication. […] Yes, new treatments include advanced surgical techniques and targeted therapies. […] Surgery can remove tumors and alleviate symptoms. […] Radiation therapy targets cancer cells and can shrink tumors. […] Support services include pain management, physical therapy, and counseling.

• #1 Effectivebenign Peripheral Nerve Sheath Tumor Treatment: Medical And Surgical Approaches

  https://drchandrilchugh.com/peripheral-nerve-tumors/benign-peripheral-nerve-sheath-tumor-treatment/

  Many studies are looking at how well and safely these treatments work on peripheral nerve sheath tumors. As these studies progress, new treatment options are becoming available. This gives patients and their doctors the chance to use the latest and greatest care. […] Staying up-to-date about the progress in treating these tumors is key. With the support of doctors and the latest information, patients can face their situation with hope.

• #1 Immunotherapeutic treatments for spinal and peripheral nerve tumors: a primer in: Neurosurgical Focus Volume 52 Issue 2 (2022) Journals

  https://thejns.org/focus/view/journals/neurosurg-focus/52/2/article-pE8.xml

  Spinal and peripheral nerve tumors are a heterogeneous group of neoplasms that can be associated with significant morbidity and mortality despite the current standard of care. Immunotherapy is an emerging therapeutic option to improve the prognoses of these tumors. […] Four primary modalities of immunotherapy (CAR T cell, monoclonal antibody, viral, and cytokine) have been reported to target spine and peripheral nerve tumors. […] Within peripheral nerve tumors, the use of immunotherapy to treat neurofibromas in the setting of syndromes has been suggested in theory, and possible immunotherapeutic targets have been identified in malignant peripheral nerve tumors. […] Immunotherapy to treat spinal and peripheral nerve tumors has become an emerging area of research and interest. A large amount of preclinical data supporting the translation of this therapy into practice, aimed at ameliorating the poor prognoses of specific tumors, have been reported. Future clinical studies for translation will focus on the optimal therapy type and administration route to best target these tumors, which often preclude total surgical resection given their proximity to the neural and vascular elements of the spine.

• #1 Immunotherapeutic treatments for spinal and peripheral nerve tumors: a primer in: Neurosurgical Focus Volume 52 Issue 2 (2022) Journals

  https://thejns.org/focus/view/journals/neurosurg-focus/52/2/article-pE8.xml

  The rise in detection and treatment of peripheral nerve tumors signals an emerging interest in developing possible immunotherapies for these tumors. Although shadowed in development when compared with their spinal counterparts, peripheral nerve tumors, such as neurofibromas and malignant peripheral nerve tumors, may be vulnerable to specific immunotherapies. […] Immunotherapy for management of malignant peripheral nerve sheath tumors (MPNSTs) is largely limited to in vitro and in vivo investigations, although some small-scale clinical trials have been reported. […] Several immunomodulatory techniques centered on macrophages are also currently under investigation for treatment in MPNSTs and other neurofibroma-related peripheral nerve tumors.

• #1 PERIPHERAL NERVE TUMORS – Dr Prem Pillay

  https://singaporebrain.org/en/nerves/peripheral-nerve-tumors/

  Research into molecular pathways involved in PNSTs, especially MPNSTs, has propelled the exploration of targeted agents. […] Emerging evidence suggests that immunomodulatory approaches or other molecularly guided treatments may benefit a subset of aggressive MPNSTs, though these remain experimental. […] Accurate diagnosis via imaging and biopsy, alongside precision surgical techniques, can often lead to remission in benign lesions and offer the best chance for disease control in malignant ones. […] Meanwhile, current innovations in targeted therapy, molecular diagnostics, and radiation approaches hold promise for improving outcomes in these rare yet complex tumors.

• #1 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).

• #2 Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiative

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10093509/

  Surgery is the therapy of first choice in central and peripheral nerve sheath tumors to obtain a histological diagnosis and reduce tumor burden with the primary aim to preserve surrounding soft tissues and nerve functioning. […] Given the heterogeneity and rarity of these tumors, there is a paucity of well-powered clinical trials, thus it is not possible to generate evidence-based treatment recommendations for non-surgical modalities. However, some clinical trials have been reported on targeted therapies in plexiform neurofibromas of NF1 patients or in heterogenous cohorts of soft-tissue tumors, including MPNSTs, with initial data of efficacy that need to be further investigated.

• #2 Benign peripheral nerve tumor – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/peripheral-nerve-tumors-benign/diagnosis-treatment/drc-20504138

  Treatment of peripheral nerve tumors involves either surgical removal or watching the tumor for changes. If there’s a low chance that the tumor may become cancerous and if it isn’t causing symptoms, you might not need surgery. […] Your healthcare professional also might recommend observation if your tumor is in a place that makes it hard to remove. Observation includes regular checkups and imaging tests to see if the tumor is growing. […] Surgery may be needed if there is a concern that the tumor is cancerous. The tumor also may be surgically removed if it is large or causing pain or other symptoms, such as weakness, numbness or tingling.

• #2 Treatment for Peripheral Nerve Tumors

  https://www.rwjbh.org/treatment-care/neuroscience/neurosurgery/conditions-treated/peripheral-nerve-tumors/

  Peripheral nerve tumors are uncommon and should be treated by neurology physicians to ensure the best possible outcome. […] Peripheral nerve tumors become problematic when they interfere with normal activities, cause pain, weakness, numbness, and tingling. Usually, these tumors can be removed without destroying any nerves or harming the functions these nerves perform. For most patients, waiting and watching to see if the tumor grows is an option, particularly if its location makes it difficult to remove, or if the tumor is small. Regular checkups using imaging scans are useful for monitoring the status of a peripheral nerve tumor. Typically, these checkups occur every few months. […] If you are a candidate for surgery, the goal is to keep the entire tumor intact when removing it so as not to damage any nearby, healthy tissues. If this isn’t possible, your neurosurgeon will remove as much of the tumor as possible, and may recommend radiation therapy instead of surgery, which can kill tumor cells. Successful surgery can relieve symptoms, but it is possible for tumors to grow back. […] If your peripheral nerve sheath tumor is cancerous, it may be treated with a combination of surgery, chemo, and radiation therapy, but tumors may recur after treatment and/or spread to other areas of the body.

• #2 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 peripheral nerve sheath tumor is not causing any symptoms, the medical team may suggest observation alone. […] A specialist may recommend that the tumor should be removed surgically in certain cases: Patient is in pain, The patient shows neurologic deficits, Structures near the tumor have impaired function, The tumor is growing, A biopsy reveals the peripheral nerve tumor is malignant. […] Surgical choices depend on the size, location, rate of growth of the tumor, and whether it is benign or malignant. […] Microsurgery: Surgery is the preferred treatment for most benign peripheral nerve sheath tumors, since it offers excellent results.

• #2

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

  Treatment for peripheral nerve tumors depends on the location, size, type and growth rate of the tumor. […] For some benign tumors that are small, slow-growing and not likely to cause significant symptoms, we’ll recommend ongoing observation. We’ll schedule you for regular checkups and imaging scans to make sure it’s still growing slowly. […] Surgery on peripheral nerve tumors carries risks unique to the type of tumors. For example, surgery on a vestibular schwannoma needs to be done in an area that’s hard to access and may be close to the brain stem. […] Any surgery on a peripheral nerve tumor requires your surgeon to be careful to avoid any damage to the nerve or surrounding tissues. It may be difficult to remove the entire tumor because of its size or location. […] Surgery may be recommended for some benign tumors that are associated with important functions or may affect nearby organs or tissues. In these cases, we’ll recommend surgery to remove as much of the tumor as possible.

• #2 Peripheral nerve tumors | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/peripheral-nerve-tumors

  Treatment usually involves surgery to remove the tumor. When the tumor can’t be removed without damaging nearby healthy tissue and nerves, other treatments may be considered. […] Treatment of a peripheral nerve tumor depends on the type of tumor, what nerves and other tissues it affects, and symptoms. Treatment options may include: […] Watching and waiting to see if the tumor grows may be an option if it’s in a place that makes removal difficult. Or it may be an option if the tumor is small, slow growing, and causes few or no symptoms. […] Some peripheral nerve tumors are removed with surgery. The goal of surgery is to take out the entire tumor without damaging nearby healthy tissue and nerves. When that isn’t possible, surgeons remove as much of the tumor as they can. […] 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.

• #2 Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiative

  https://www.mdpi.com/2072-6694/15/7/1930

  The EUropean Network for RAre CANcers (EURACAN) Task Force on Ultrarare Brain Tumors has reviewed the evidence of diagnostic and therapeutic interventions and drawn recommendations on peripheral and cranial nerve sheath tumors. […] The Task Force has reviewed the evidence of diagnostic and therapeutic interventions, which is particularly low due to the rarity, and drawn recommendations accordingly. Tumor diagnosis is primarily based on hematoxylin and eosin-stained sections and immunohistochemistry. […] Gross-total resection is the first choice, and can be curative in benign lesions; however, the extent of resection must be balanced with preservation of nerve functioning. […] Radiotherapy can be omitted in benign tumors after complete resection and in NF-related tumors, due to the theoretic risk of secondary malignancies in a tumor-suppressor syndrome.

• #2 Effectivebenign Peripheral Nerve Sheath Tumor Treatment: Medical And Surgical Approaches

  https://drchandrilchugh.com/peripheral-nerve-tumors/benign-peripheral-nerve-sheath-tumor-treatment/

  If the tumor doesn’t grow and you don’t get more symptoms, just watching it may be enough. But, if it starts growing fast or you feel sicker, surgery could be needed. Keep an eye on it and act fast if things change. […] Patients with benign peripheral nerve sheath tumors may need surgery if they have severe symptoms or if their tumors are growing quickly. The main goal of this surgery is to fully remove the tumor. It aims to keep the healthy nerve tissue around it safe and lower the risk of nerve damage. […] The focus is on removing benign peripheral nerve sheath tumors like schwannomas. Surgeons use advanced tools to do this. They work very carefully to not harm the healthy nerves near the tumor. This type of surgery is very precise and detailed. […] High-powered microscopes and nerve stimulators are new tools that make surgery on these tumors easier. These tools help surgeons reach difficult areas more easily. With microsurgery, the goal is to fully remove the tumor without hurting the nearby healthy nerves.

• #2 Peripheral Nerve Sheath Tumors: From Preclinical Studies to Advanced Therapies | SpringerLink

  https://link.springer.com/book/10.1007/978-3-031-76382-3

  This book offers an update about peripheral nerve sheath tumors (PNST), both in their benign and malignant forms (MPNST). […] Another relevant part is reserved to the description of the most recent surgical techniques (fluorescent dye for intraoperative surgical guidance, neurophysiological monitoring, esoscopic view). […] Finally, new oral treatment for PNST related to neurocutaneous diseases (as selumetinib in plexiform neurofibromas) is described. […] MEK Inhibitors and Other Medical Therapies for Benign Peripheral Nerve Sheath Tumors […] Systemic Treatment in Malignant Peripheral Nerve Sheath Tumors: Current Practice and Future Possibilities […] Proton and Carbon Ion Therapy for MPNST.

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

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

  Stereotactic radiosurgery: Neurological surgeons can direct highly targeted radiation beams at a tumor from multiple angles using a Gamma Knife, or other stereotactic radiosurgery equipment. […] 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.

• #2 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.

• #2 Peripheral nerve tumors | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/peripheral-nerve-tumors

  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.

• #2 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.

• #2

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

  Surgery may be recommended for benign tumors if they’re in a place that might cause significant symptoms and it seems possible to remove all or most of the tumor. […] Malignant peripheral nerve sheath tumor treatment usually includes cancer surgery to remove as much of the tumor as possible. In extreme cases, surgery for an MPNST may require partial amputation of an affected limb. […] Radiation therapy and chemotherapy may be applied before surgery to shrink the tumor, after surgery to remove the rest of the tumor, or both before and after surgery.

• #2 Malignant peripheral nerve sheath tumors (MPNST) – Clinicopathological study and treatment outcome of twenty-four cases | World Journal of Surgical Oncology | Full Text

  https://wjso.biomedcentral.com/articles/10.1186/1477-7819-4-55

  Malignant peripheral nerve sheath tumor (MPNST) is biologically an aggressive tumor for which the treatment of choice is the surgery. […] Surgery is the main stay of treatment of this tumor though they are biologically aggressive in nature. […] Curative surgery was performed in all 24 cases in the form of wide local excision in 17 cases, amputation or disarticulation in 5 cases and two patients had pelvic exenteration for large pelvic MPNST. […] In view of the size (5 cm), location, and grade, postoperative adjuvant radiotherapy, ranging from 54 to 62 Gy (median dose 58 Gy), was given to 16 patients. […] Postoperative radiotherapy has shown a definite role in both disease free and overall survival in this study. […] The overall treatment approach should be like that of any other high grade sarcomas. […] Although adjuvant radiotherapy has not been cited as a significant prognostic factor in the current study, the results do reveal a trend towards an impact on both disease free and overall survival.

• #2

  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.

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

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

  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 Chemotherapy for the treatment of malignant peripheral nerve sheath tumors in neurofibromatosis 1: a 10-year institutional review | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-8-127

  Neurofibromatosis 1 (NF1) is associated with significant morbidity and mortality because of complications, especially malignant peripheral nerve sheath tumors (MPNSTs), which mainly develop during adulthood. […] We evaluated our experience with management of NF1 with MPNSTs by standard chemotherapy with anthracycline and/or ifosfamide in terms of time to treatment failure and overall survival. […] Median time to treatment failure and overall survival were 7.8 and 17 months, respectively. […] MPNSTs are highly aggressive in NF1. Conventional chemotherapy does not seem to reduce mortality, and its role must be questioned. […] Adjuvant chemotherapy is not standard treatment in adult-type soft-tissue sarcomas and can be proposed for high-risk tumors. Extensive disease is treated with anthracycline-based chemotherapy. Ifosfamide may be discussed for patients with good performance status.

• #2 Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiative

  https://www.mdpi.com/2072-6694/15/7/1930

  Systemic therapy should be considered in incomplete resected plexiform neurofibromas/MPNSTs. […] MEK inhibitor selumetinib can be used in NF1 children ≥2 years with inoperable/symptomatic plexiform neurofibromas, while anthracycline-based treatment is the first choice for unresectable/locally advanced/metastatic MPNST. […] Clinical trials on other MEK1-2 inhibitors alone or in combination with mTOR inhibitors are under investigation in plexiform neurofibromas and MPNST, respectively.

• #2 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. […] 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. […] Currently research on gene therapy for the treatment of NF1 related MPNSTs is ongoing. […] In conclusion, no effective PNSTs treatment was found except complete surgical resection, which can be performed in selected patients with NF1.

• #2 Peripheral nerve tumors | Health Library | Memorial Health System

  https://www.mhsystem.org/health-library/con-20213617/

  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.

• #2 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.

• #2 Nerve Sheath Tumors: Definition & Types

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

  In addition to surgery, treatment for malignant peripheral nerve sheath tumors may include cancer therapies such as radiation therapy and chemotherapy. […] General complications of surgery include: Bleeding, Local infection, Pain, Scarring. […] 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. […] Most nerve sheath tumors are noncancerous. Theyre treatable with surgery and rarely come back. If your tumor cant be completely removed surgically, youll require ongoing monitoring. […] The risk of nerve sheath tumors becoming cancerous is very low. The greatest risk is for people with NF1 who develop plexiform neurofibroma. 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.

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