Łagodny nowotwór nerwu obwodowego
Leczenie
Łagodne nowotwory nerwu obwodowego (BPNST) wymagają zindywidualizowanego podejścia terapeutycznego, uwzględniającego lokalizację, wielkość, tempo wzrostu guza oraz objawy neurologiczne. W przypadku małych, bezobjawowych guzów zalecana jest obserwacja z kontrolami obrazowymi (MRI, CT, USG) co 6-12 miesięcy, z możliwością wydłużenia interwału przy stabilności zmian. Chirurgiczne usunięcie guza pozostaje podstawową metodą leczenia, szczególnie przy objawach neurologicznych, dużych rozmiarach, szybkim wzroście lub podejrzeniu złośliwości. Techniki mikrochirurgiczne z monitorowaniem neurofizjologicznym umożliwiają precyzyjną resekcję z zachowaniem funkcji nerwu. Radioterapia stereotaktyczna (np. Gamma Knife) jest opcją w trudno dostępnych lokalizacjach lub u pacjentów niekwalifikujących się do operacji, jednak przeciwwskazana u chorych z NF ze względu na ryzyko wtórnych nowotworów. Kompleksowe leczenie wymaga interdyscyplinarnej współpracy neurochirurgów, neurologów, radiologów, onkologów i rehabilitantów.
- Leczenie łagodnego nowotworu nerwu obwodowego
- Obserwacja i monitorowanie
- Leczenie chirurgiczne
- Radioterapia stereotaktyczna
- Leczenie interdyscyplinarne
- Rehabilitacja i opieka pooperacyjna
- Specyficzne aspekty leczenia różnych typów guzów
- Leczenie nowotworu złośliwego osłonek nerwów obwodowych
- Nowe kierunki w leczeniu łagodnych nowotworów nerwu obwodowego
- Znaczenie ośrodków specjalistycznych w leczeniu
- Podsumowanie wyników leczenia
- Zalecenia dotyczące postępowania
Leczenie łagodnego nowotworu nerwu obwodowego
Leczenie łagodnego nowotworu nerwu obwodowego (BPNST – Benign Peripheral Nerve Sheath Tumor) obejmuje różne strategie terapeutyczne w zależności od lokalizacji, wielkości, tempa wzrostu guza oraz występowania objawów neurologicznych. Podejście terapeutyczne musi być zindywidualizowane dla każdego pacjenta, z uwzględnieniem specyficznych czynników klinicznych i preferencji chorego.12
Obserwacja i monitorowanie
W przypadku małych, bezobjawowych i wolno rosnących guzów, obserwacja (tzw. watchful waiting) jest często stosowanym podejściem terapeutycznym:34
- Regularne badania kontrolne i badania obrazowe (MRI, CT, USG) co 6-12 miesięcy w celu monitorowania wzrostu guza
- Jeśli kolejne badania kontrolne wykazują stabilność guza, można wydłużyć okres między kontrolami do kilku lat
- Obserwacja jest szczególnie zalecana w przypadkach, gdy guz znajduje się w miejscu trudno dostępnym dla chirurgii
- Niskie prawdopodobieństwo transformacji złośliwej oraz brak objawów klinicznych są dodatkowymi czynnikami przemawiającymi za obserwacją
Leczenie chirurgiczne
Chirurgiczne usunięcie guza jest podstawową metodą leczenia łagodnych nowotworów nerwu obwodowego, szczególnie w następujących przypadkach:13
- Obecność objawów neurologicznych takich jak ból, osłabienie, drętwienie lub mrowienie
- Duże rozmiary guza
- Szybki wzrost guza
- Podejrzenie złośliwego charakteru nowotworu
- Kompresja okolicznych struktur anatomicznych
Celem zabiegu chirurgicznego jest całkowite usunięcie guza z zachowaniem funkcji nerwu i uniknięciem uszkodzenia okolicznych tkanek. Techniki operacyjne obejmują:39
- Mikrochirurgię – umożliwiającą precyzyjne usunięcie guza z minimalnym uszkodzeniem struktury nerwu
- Monitorowanie neurofizjologiczne – stosowane śródoperacyjnie w celu zachowania funkcji nerwu
- Techniki minimalnie inwazyjne – w wybranych przypadkach, zmniejszające traumatyzację tkanek
Warto podkreślić, że w przypadku różnych typów guzów stosuje się odmienne techniki chirurgiczne:1213
- W przypadku schwannoma – guz zazwyczaj można oddzielić od nerwu bez niszczenia jego funkcji, ponieważ rozwija się on otoczce nerwu
- W przypadku nerwiakowłókniaków – operacja jest bardziej skomplikowana, ponieważ guz rozwija się wewnątrz nerwu, co może wymagać otwarcia nerwu i usunięcia guza z jego wnętrza, co wiąże się z potencjalnym uszkodzeniem funkcji nerwu
Radioterapia stereotaktyczna
Radioterapia stereotaktyczna (radiochirurgia) może być stosowana w wybranych przypadkach łagodnych nowotworów nerwu obwodowego:164
- Gdy guz znajduje się w lokalizacji trudnodostępnej chirurgicznie (np. w obrębie czaszki lub przy podstawie czaszki)
- W przypadku guzów przylegających do ważnych struktur neurologicznych, gdzie resekcja mogłaby spowodować znaczące deficyty neurologiczne
- U pacjentów niekwalifikujących się do zabiegu operacyjnego ze względu na współistniejące choroby
- Najczęściej stosowaną techniką jest tzw. Gamma Knife, umożliwiająca precyzyjne dostarczenie promieniowania do guza bez wykonywania nacięcia
Należy jednak zauważyć, że radioterapia może być przeciwwskazana u pacjentów z zespołem nerwiakowłókniakowatości (NF) ze względu na teoretyczne ryzyko wtórnych nowotworów złośliwych w zespole z predyspozycją do nowotworów.1920
Leczenie interdyscyplinarne
Optymalne leczenie łagodnych nowotworów nerwu obwodowego wymaga podejścia interdyscyplinarnego z udziałem specjalistów różnych dziedzin:1018
- Neurochirurgów – odpowiedzialnych za przeprowadzenie zabiegu operacyjnego
- Neurologów – oceniających deficyty neurologiczne przed i po leczeniu
- Radiologów – wykonujących i interpretujących badania obrazowe
- Onkologów – w przypadku podejrzenia złośliwego charakteru guza
- Specjalistów rehabilitacji – prowadzących rehabilitację pourazową
Wczesna diagnoza i skierowanie do ośrodka specjalistycznego są kluczowe dla osiągnięcia optymalnych wyników leczenia. Ze względu na rzadkość występowania tych guzów i ich złożoność, rekomenduje się leczenie w ośrodkach posiadających doświadczenie w terapii nowotworów nerwów obwodowych.1023
Rehabilitacja i opieka pooperacyjna
Po leczeniu chirurgicznym łagodnego nowotworu nerwu obwodowego, pacjenci często wymagają kompleksowej rehabilitacji w celu odzyskania funkcji neurologicznych i poprawy jakości życia:1617
- Fizjoterapia – ukierunkowana na poprawę siły mięśniowej, zakresu ruchu i ogólnej sprawności fizycznej
- Terapia zajęciowa – mająca na celu przywrócenie zdolności wykonywania codziennych czynności
- Stosowanie ortez i szyn – w celu stabilizacji kończyny i wspomagania procesu gojenia
- Terapia przeciwbólowa – w przypadku bólu pooperacyjnego lub neuropatycznego
Regularne wizyty kontrolne są niezbędne do monitorowania procesów gojenia, oceny funkcji neurologicznych oraz wczesnego wykrycia ewentualnego nawrotu guza. Harmonogram wizyt kontrolnych zazwyczaj obejmuje:2127
- Ocenę kliniczną po 4-6 tygodniach w celu oceny gojenia rany
- Badania obrazowe i ocenę neurologiczną po 6 miesiącach w celu wykluczenia wznowy guza
- Długoterminowe monitorowanie w przypadku niecałkowitej resekcji guza lub wyższego ryzyka nawrotu
Specyficzne aspekty leczenia różnych typów guzów
Leczenie schwannoma
Schwannoma (nerwiak osłonkowy) jest najczęstszym typem łagodnego nowotworu nerwu obwodowego, który rozwija się z komórek Schwanna. Leczenie tego typu guza obejmuje:1229
- Mikrochirurgiczną resekcję guza, która zwykle pozwala na całkowite usunięcie bez uszkodzenia nerwu, ponieważ guz rozwija się na powierzchni nerwu
- Radiochirurgię stereotaktyczną (Gamma Knife) w przypadku trudno dostępnych lokalizacji, szczególnie w obrębie nerwów czaszkowych
- Doskonałe rokowanie po całkowitym usunięciu, z niskim ryzykiem nawrotu
Leczenie nerwiakowłókniaków
Nerwiakowłókniaki stanowią większe wyzwanie terapeutyczne ze względu na ich wzrost wewnątrz struktur nerwowych. Strategia leczenia obejmuje:1214
- Dokładną ocenę przedoperacyjną w celu określenia relacji guza do pęczków nerwowych
- Techniki mikrochirurgiczne z monitorowaniem neurofizjologicznym
- W przypadku nerwiakowłókniaków splotowatych (plexiform neurofibromas) – trudna resekcja ze względu na wzrost guza w obrębie nerwu i między warstwami izolacji nerwowej
- W przypadku niecałkowitej resekcji – ścisłe monitorowanie ze względu na ryzyko nawrotu
- W wybranych przypadkach nerwiakowłókniaków splotowatych związanych z NF1 – rozważenie terapii systemowej inhibitorem MEK (selumetinib) u dzieci z nieresekcyjnymi lub objawowymi guzami
Leczenie nowotworu złośliwego osłonek nerwów obwodowych
Chociaż głównym tematem tego artykułu są łagodne nowotwory nerwu obwodowego, warto wspomnieć o postępowaniu w przypadku złośliwego nowotworu osłonek nerwów obwodowych (MPNST), który wymaga odmiennego, bardziej agresywnego podejścia terapeutycznego:3435
- Leczenie chirurgiczne – radykalna resekcja guza z szerokim marginesem zdrowych tkanek, w skrajnych przypadkach może być konieczna amputacja kończyny
- Radioterapia – stosowana przed operacją w celu zmniejszenia guza lub po operacji w celu eliminacji pozostałych komórek nowotworowych
- Chemioterapia – najczęściej stosowane schematy oparte na doksorubicynie i ifosfamidzie, stosowane w leczeniu nieoperacyjnych lub przerzutowych MPNST
- Leczenie skojarzone – połączenie różnych metod terapeutycznych dla osiągnięcia optymalnego efektu leczniczego
W przypadku MPNST prowadzone są liczne badania kliniczne nad nowymi metodami leczenia, w tym terapiami celowanymi i immunoterapią, które mogą poprawić wyniki leczenia tej agresywnej choroby.383940
Nowe kierunki w leczeniu łagodnych nowotworów nerwu obwodowego
Badania naukowe i rozwój technologiczny prowadzą do pojawienia się nowych metod leczenia łagodnych nowotworów nerwu obwodowego:4142
- Zaawansowane techniki obrazowania śródoperacyjnego – pozwalające na dokładniejszą identyfikację granic guza i struktur nerwowych
- Metody ablacyjne – takie jak krioablacja, termoablacja czy ablacja mikrofalowa jako alternatywa dla tradycyjnej chirurgii w wybranych przypadkach
- Terapie celowane molekularnie – ukierunkowane na specyficzne szlaki sygnałowe zaangażowane w rozwój guzów nerwów obwodowych
- Badania kliniczne – oceniające skuteczność nowych metod leczenia, szczególnie w przypadku guzów związanych z zespołami genetycznymi
Znaczenie ośrodków specjalistycznych w leczeniu
Leczenie łagodnych nowotworów nerwu obwodowego powinno być prowadzone w wyspecjalizowanych ośrodkach posiadających doświadczenie w diagnozowaniu i leczeniu tych rzadkich zmian. Dane pokazują, że nieprawidłowa diagnoza wstępna może prowadzić do niewłaściwego leczenia i poważnych powikłań neurologicznych:4523
- Badania wykazują, że w 44,7% przypadków łagodnych nowotworów nerwu obwodowego stawiana jest początkowa błędna diagnoza
- Nieodpowiednie leczenie chirurgiczne wynikające z błędnej diagnozy może prowadzić do poważnych deficytów neurologicznych
- Leczenie operacyjne bez użycia technik mikrochirurgicznych i monitorowania neurofizjologicznego zwiększa ryzyko powikłań
- Zaleca się, aby leczenie było prowadzone wyłącznie w wyspecjalizowanych ośrodkach przez doświadczonych chirurgów
Podsumowanie wyników leczenia
Wyniki leczenia łagodnych nowotworów nerwu obwodowego są generalnie dobre, szczególnie przy zastosowaniu odpowiednich technik chirurgicznych i kompleksowym podejściu terapeutycznym:2746
- Leczenie chirurgiczne prowadzi do znaczącej poprawy jakości życia pacjentów, zmniejszenia niepełnosprawności i dolegliwości bólowych
- Badania wykazują redukcję ogólnej niepełnosprawności w kończynie (wyniki kwestionariusza QuickDASH: 18/100 przed operacją vs 5/100 12 miesięcy po operacji)
- Obserwuje się poprawę w zakresie codziennych aktywności oraz zmniejszenie dolegliwości bólowych
- Ryzyko trwałych powikłań po leczeniu chirurgicznym jest niskie, przy zastosowaniu odpowiednich technik operacyjnych
- Większość pacjentów wyraża zadowolenie z wyniku leczenia i opieki
W przypadku niecałkowitej resekcji lub specyficznych typów guzów, konieczne może być długoterminowe monitorowanie i dodatkowe interwencje terapeutyczne.4849
Zalecenia dotyczące postępowania
Na podstawie aktualnych danych naukowych i doświadczeń klinicznych, można sformułować następujące zalecenia dotyczące postępowania w przypadku łagodnych nowotworów nerwu obwodowego:1920
- Wczesna i dokładna diagnoza – oparta na badaniu klinicznym, badaniach obrazowych i w wybranych przypadkach biopsji
- Indywidualizacja leczenia – wybór metody leczenia dostosowany do specyfiki guza, jego lokalizacji i objawów klinicznych
- Mikrochirurgiczna resekcja – jako metoda z wyboru w przypadku objawowych guzów, wykonywana przez doświadczonych chirurgów z zastosowaniem monitorowania neurofizjologicznego
- Zachowanie funkcji nerwu – priorytetem podczas zabiegu operacyjnego powinno być zachowanie funkcji nerwu przy maksymalnej resekcji guza
- Interdyscyplinarne podejście – ścisła współpraca specjalistów różnych dziedzin w celu optymalizacji wyniku leczenia
- Regularne monitorowanie – systematyczne kontrole w przypadku obserwacji lub niecałkowitej resekcji guza
- Skierowanie do ośrodka specjalistycznego – w przypadku złożonych, nietypowych lub nawrotowych guzów
Przestrzeganie powyższych zaleceń pozwala na optymalizację wyników leczenia i minimalizację ryzyka powikłań, przyczyniając się do poprawy jakości życia pacjentów z łagodnymi nowotworami nerwu obwodowego.2849
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Materiały źródłowe
- #1 Benign peripheral nerve tumor – Diagnosis and treatment – Mayo Clinichttps://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.
- #2https://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.
- #3 Peripheral nerve tumors – Diagnosis and treatment – Mayo Clinichttps://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.
- #4 Treatment for Peripheral Nerve Sheath Tumors | Neurological Surgeryhttps://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: […] Surgical choices depend on the size, location, rate of growth of the tumor, and whether it is benign or malignant. […] Microsurgery is often recommended to remove all or part of a peripheral nerve tumor. […] 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.
- #5 Peripheral nerve tumors // Middlesex Healthhttps://middlesexhealth.org/learning-center/diseases-and-conditions/peripheral-nerve-tumors
Peripheral nerve tumors can occur anywhere in the body. Most of them are benign, meaning they’re not cancerous. But they can lead to pain, nerve damage and loss of function in the affected area. […] 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.
- #6https://www.advocatehealth.com/health-services/brain-spine-institute/peripheral-nerve-tumors
Treatment for peripheral nerve tumors depends on the location, size, type and growth rate of the tumor. […] Your doctor may recommend a watchful waiting approach for a small, slow-growing tumor that isn’t likely to cause significant symptoms. You’ll have regularly scheduled checkups with imaging scans of the tumor to be sure it’s still growing slowly and not getting close to organs or tissues that would cause problems with movement, speech or other important functions. […] 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.
- #7 Treatment for Peripheral Nerve Tumorshttps://www.rwjbh.org/treatment-care/neuroscience/neurosurgery/conditions-treated/peripheral-nerve-tumors/
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 isnt 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.
- #8 Does My Benign Nerve Sheath Tumor Need to be Removed?https://www.neurosurgeonsofnewjersey.com/blog/benign-nerve-sheath-tumor-removal/
Benign nerve sheath tumors come in two forms, schwannomas and neurofibromas. They are very similar and treatment considerations for both are virtually identical. If you’ve been diagnosed with a benign nerve sheath tumor, you and your doctor will need to decide how to treat it. […] Typical treatment options for benign sheath tumors include surgical resection, which is a common choice, but it isn’t always necessary if the tumor is small or in a non-sensitive location. […] If you and your doctor determine that your benign nerve sheath tumor requires active treatment, surgery is the treatment of choice. […] Surgical removal is a highly effective treatment option for benign nerve sheath tumors, but it isn’t for everyone. […] The location of your benign nerve sheath tumor plays an important role in determining whether removal is necessary.
- #9 Nerve Tumors | Brain Institute | OHSUhttps://www.ohsu.edu/brain-institute/nerve-tumors
The OHSU Nerve Center offers the expertise to precisely diagnose and treat peripheral nerve tumors. Specialty care and early diagnosis can be especially important for nerve tumors, which can range from simple to complex. […] The Nerve Center offers: […] A surgical team led by Dr. Kim Burchiel, an internationally known neurosurgeon with a detailed understanding of nerve tumors. […] The latest minimally invasive surgical options. […] Even some noncancerous tumors need treatment, though, because they can press on nerves and cause pain, nerve damage and/or loss of function. […] Rarely, nerve tumors are malignant and need aggressive treatment. […] Treatment can vary widely, depending on: […] Options include: […] Surgery: Our neurosurgeons and plastic/reconstructive surgeons offer a full range of options for tumor removal, depending on type. We also offer expertise in minimally invasive endoscopic surgery, which uses small incisions, a lighted scope and tiny tools.
- #10 Peripheral Nerve Sheath Tumor: A Diagnostic and Therapeutic Challengehttps://pmc.ncbi.nlm.nih.gov/articles/PMC11031624/
Treatment of peripheral nerve sheath tumors is predominantly surgical. […] Proliferative lesions should be treated surgically in specialist centers by experienced doctors with appropriate skills and equipment for microsurgical procedures to ensure full recovery. […] Early diagnosis and prompt referral for specialist care are paramount in swift and successful treatment of these rare tumors. […] Malignant peripheral nerve tumors need to be treated with local radiotherapy after surgical excision, and these patients should be on regular follow-ups.
- #11 Effectivebenign Peripheral Nerve Sheath Tumor Treatment: Medical And Surgical Approacheshttps://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.
- #12 Nerve Sheath Tumors: Definition & Typeshttps://my.clevelandclinic.org/health/diseases/22526-nerve-sheath-tumors
Nerve sheath tumors develop in tissues that surround and support nerve cells. Theyre usually noncancerous, treatable with surgery and dont come back after treatment. […] 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. […] Some neurofibromas, especially plexiform neurofibromas, are difficult to remove because they grow into the nerve and between the layers of insulation. If surgery results in incomplete removal, your healthcare provider will monitor you closely, as the tumor may come back. […] Schwannomas in your head, such as vestibular schwannomas, may involve nerves that control critical functions. In these areas, healthcare providers use stereotactic radiosurgery (Gamma Knife) to prevent nerve damage.
- #13 Nerve Sheath Tumor Treatment in New Jersey â Centers for Neurosurgery, Spine & Orthopedicshttps://www.cnsomd.com/surgery-conditions/nerve-tumors/nerve-sheath-tumor/
The treatment options for nerve sheath tumors depend on the stage of the tumor, location, and involvement of surrounding structures, such as peripheral nerves. Fortunately, benign tumors grow at a significantly slower rate, enabling doctors to administer non-invasive treatment methods. Otherwise, they might recommend surgery. […] Doctors initially consider non-invasive treatment approaches for treating tumors, when indicated. The majority of benign nerve sheath tumors, that are smaller in size and do not interfere with the functioning of the vital structures, would be treated with observation, that includes repeat physical examinations and serial imaging. […] 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.
- #14 Nerve Sheath Tumor Treatment in New Jersey â Centers for Neurosurgery, Spine & Orthopedicshttps://www.cnsomd.com/surgery-conditions/nerve-tumors/nerve-sheath-tumor/
Usually, surgeries involving schwannoma tumors make it easy for the surgeon to leave the nerve intact. Neurofibroma surgeries are more challenging because the growth takes place within the nerve cellâs center. That means a neurosurgeon will have first to open the nerve and remove the tumor from within. The incision damages the nerve, but the nerve has the ability to repair itself to restore the function. […] The type of tumor and its location in the body, and the extent of the surgery all affect the recovery time after the procedure. The neurosurgeon will discuss the surgical procedure and recovery process before the operation in great detail. If the tumor is determined to be MPNST, the surgeon will coordinate with other physicians as needed to provide a comprehensive treatment plan. […] 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 caret_down iconhttps://www.spectrumhealth.org/services/neurosciences/brain-and-spine-tumors/nerve-sheath-tumors
Since the tumor is inside the nerve, your surgeon removes the tumor from within the nerve. Our goal is to preserve the nerve. Your doctor may recommend a nerve repair surgery, if the nerve is damaged. […] If you have a Schwanomma sheath tumor, your surgeon will remove the tumor off the surface of the nerve. Our goal is to preserve the nerve. If damaged during surgery, your doctor will recommend a nerve repair surgery.
- #16 Peripheral nerve tumors – Diagnosis and treatment – Mayo Clinichttps://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.
- #17 Peripheral nerve tumors // Middlesex Healthhttps://middlesexhealth.org/learning-center/diseases-and-conditions/peripheral-nerve-tumors
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. […] 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. […] 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.
- #18 Effectivebenign Peripheral Nerve Sheath Tumor Treatment: Medical And Surgical Approacheshttps://drchandrilchugh.com/peripheral-nerve-tumors/benign-peripheral-nerve-sheath-tumor-treatment/
Stereotactic radiosurgery is a method used for some benign peripheral nerve sheath tumors. These tumors are hard to reach through surgery or risk nerve damage. It uses focused radiation beams to treat the tumor directly, avoiding open surgery. This is especially helpful for tumors near the brain or spinal cord. […] This treatment has its risks, like nerve weakness or numbness in the treated area. However, for some patients, it’s a good option if regular surgery is risky or not possible. Stereotactic radiosurgery is a great alternative to open surgery for benign peripheral nerve sheath tumors, including Schwannomas, in certain cases. […] Benign peripheral nerve sheath tumors are best treated by a team of experts from different fields. This group includes neurologists, neurosurgeons, radiologists, and rehab specialists. Having a team with varied skills is key because these tumors are rare, and treatment can be complex. Thus, working together helps create the best treatment plans for patients.
- #19 Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiativehttps://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. […] 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.
- #20 Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiativehttps://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.
- #21 Effectivebenign Peripheral Nerve Sheath Tumor Treatment: Medical And Surgical Approacheshttps://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.
- #22 Peripheral Nerve Sheath Tumors | Conditions | Penn State Healthhttps://www.pennstatehealth.org/services-treatments/peripheral-nerve-sheath-tumors
Experts at Penn State Health offer comprehensive diagnostic imaging and multidisciplinary treatment options for patients with peripheral nerve sheath tumors. […] Our expert team at Penn State Health will assess your peripheral nerve sheath tumor and use diagnostic testing to determine if it is malignant or benign. Treatment options include: […] Periodic monitoring with examination and imaging […] Emerging chemotherapy and immunotherapy treatment options […] Surgical removal to minimize the risk of damage to the normal nerve fibers and structures surrounding the tumor […] Gamma Knife (highly focused radiation) […] Percutaneous treatments to freeze or heat the tumor, causing it to stop growing and in some cases, to shrink the tumor.
- #23https://link.springer.com/article/10.1007/s10143-023-02107-z
We aimed to evaluate the rate of primary misdiagnosis and mistreatment in patients suffering from deep-seated bPNSTs. […] Suboptimal treatment was assumed in symptomatic patients, if symptoms remained the same or progressed for more than 6 months under conservative treatment and without surgical treatment. […] Inappropriate surgical treatment was present, if the patient was surgically treated not due to the bPNST, but because of an accompanying disc prolapse or a nerve entrapment syndrome, for example, without relieve of the symptoms. […] Mistreatment was present, if the surgical treatment of the bPNST did not follow the current treatment standard for deep-seated nerval tumors (microsurgical tumor resection with neurophysiological monitoring). […] Furthermore, an unnecessary surgical treatment unrelated to the bPNST was performed in 26.3%. […] Surgical removal or partial removal of the bPNST was performed using an inappropriate surgical technique (non-microsurgical and without intraoperative electrophysiological testing) in 26.3%. […] Treatment should be performed only at a specialized center.
- #24 Peripheral nerve tumors | Altru Health Systemhttps://www.altru.org/health-library/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.
- #25 Peripheral Nerve Tumor Surgery in India | Dr Sumit Sinhahttps://drsumitsinha.com/nerve-tumor-treatment.php
Standard cancer therapies, such as surgery, chemotherapy, and radiation therapy, are used to treat malignant nerve tumors. […] You may require rehabilitation after the surgery. The doctors may use braces to keep your arm or leg steady, which helps you to heal. Physical therapists like physiotherapists can help you restore your movements or functions lost due to nerve damage.
- #26 Nerve Tumors | Brain Institute | OHSUhttps://www.ohsu.edu/brain-institute/nerve-tumors
Radiation therapy: Radiation therapy can be used to reduce some nerve tumors. […] For malignant peripheral nerve sheath tumors, immediate treatment is recommended. Your care team will seek to surgically remove as much of the tumor as possible. Other treatments for these tumors may include radiation therapy and/or chemotherapy. […] The expert physical therapists, occupational therapists and hand therapists at OHSUs rehabilitation center can work with you to recover from surgery and restore function.
- #27 Benign nerve tumours in the upper limb: a registry-based study of symptoms and surgical outcome | Scientific Reportshttps://www.nature.com/articles/s41598-023-38184-9
Surgery for benign nerve tumours is performed for pathoanatomical diagnosis and symptomatic relief, but might cause residual problems. […] Surgery reduced overall disability in the affected limb (QuickDASH 18/100 [IQR 536] preoperatively and 5/100 [IQR 022] 12 months postoperatively), improved ability to perform daily activities (HQ-8; 11/100 [IQR 050] preoperatively and 0/100 [IQR 020] 12 months postoperatively) and decreased three evaluated pain modalities: pain at rest (HQ-8; 20/100 [IQR 040] preoperatively and 0/100 [IQR 010] 12 months postoperatively), pain on motion without load (HQ-8; 20/100 [IQR 040] preoperatively and 0/100 [IQR 010] 12 months postoperatively), and pain on load (HQ-8; 24/100 [IQR 169] preoperatively and 1/100 [IQR 030] 12 months postoperatively). […] We conclude that surgery for benign peripheral nerve tumours provides good symptomatic relief with low risk for residual problems.
- #28 Surgical outcome of isolated benign peripheral nerve sheath tumors without neurofibromatosis | Egyptian Journal of Neurosurgery | Full Texthttps://ejns.springeropen.com/articles/10.1186/s41984-024-00297-2
Surgical resection remains the treatment of choice of BPNSTs because they may cause neural compromise, rapid growth, and pressure they apply to nearby structures. […] We usually recommended excisional biopsy to remove lesions that appeared benign on imaging if they were symptomatic or grew over time. […] The surgical technique for removing schwannomas and neurofibromas, the most common benign PNSTs, depends on how they relate to the nerve fascicles. […] The patients are evaluated after 4-6 weeks for wound healing and after 6 months for tumor recurrence. […] Benign peripheral nerve sheath tumors are safely resected without increased sensory and motor deficits after surgery and with improved clinical outcome with no recurrence on follow up.
- #29 Peripheral Nerve Tumour | London Bridge Sports Medicinehttps://lbsm.co.uk/peripheral-nerve-tumour-presenting-as-shin-pain/
Peripheral nerve tumors (PNTs) are a heterogeneous group of mostly benign tumors that are rare in the general population. They usually develop in the peripheral nerve axonal nerve sheath and surrounding connective tissue. Although usually noncancerous, they still may require treatment because they can cause nerve compression, damage and/or loss of function. […] Peripheral Nerve tumors can grow slowly or quickly, depending on the type. Some need no treatment or only monitoring. Rarely, nerve tumors are malignant and need aggressive treatment. […] Patient put on anti-epileptic Pregabalin medication to reduce sensitivity of neuropathic pain as preliminary treatment. Patient referred to Peripheral Nerve Tumour unit for excision of lesion. Patient had only very mild ongoing symptoms post resection of tumour. […] Desensitisation programme for the affected nerve lesion. Ongoing strength conditioning training. […] Schwannomas are the commonest peripheral nerve tumour and are usually benign in nature.
- #30 Peripheral nerve tumors of the hand: Clinical features, diagnosis, and treatmenthttps://www.wjgnet.com/2307-8960/full/v8/i21/5086.htm
Treatment options for neurofibromas depend on subtypes, signs, and symptoms. […] Surgical resection is necessary for patients who experience intolerable pain or progressive neurologic function deficits or if the diagnosis is uncertain or when malignancy is suspected. […] In the presence of pain or neurologic injury, surgical excision should be the main treatment with extremely low risk of recurrence. […] Surgical excision with careful preservation of nerve fascicles can relieve symptoms in most patients, and microsurgical technique aids in the process of precise tumor dissection.
- #31 Benign peripheral nerve tumor | Beacon Health Systemhttps://www.beaconhealthsystem.org/library/diseases-and-conditions/benign-peripheral-nerve-tumor?content_id=CON-20341913
Surgeons carefully remove schwannomas while taking care to preserve nerve fascicles that aren’t affected by the tumors. […] 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. […] 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.
- #32 Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiativehttps://pmc.ncbi.nlm.nih.gov/articles/PMC10093509/
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. […] 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. In this regard, gross total resection may be curative for benign tumors. […] 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.
- #33 Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiativehttps://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 â¥2 years with inoperable/symptomatic plexiform neurofibromas, while anthracycline-based treatment is the first choice for unresectable/locally advanced/metastatic MPNST. […] 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. […] In this regard, gross total resection may be curative for benign tumors. […] 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.
- #34https://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.
- #35https://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.
- #36 Malignant peripheral nerve sheath tumour (MPNST) | Soft tissue sarcoma | Cancer Research UKhttps://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.
- #37 Successful Management of Intrathoracic Phrenic Malignant Peripheral Nerve Sheath Tumor by Multimodal Treatment | Rais | Journal of Medical Caseshttps://www.journalmc.org/index.php/JMC/article/view/3464/2777
Malignant peripheral nerve sheath tumors (MPNSTs) are rare and can be associated with type 1 neurofibromatosis. […] Complete surgical resection remains the only radical treatment. However, the outcomes after this treatment alone cannot be considered satisfactory. Preoperative chemotherapy could be an interesting therapeutic option for unresectable tumors. […] Multimodal treatment including preoperative chemotherapy, wide local resection with safe margins and adjuvant radiation, constitutes a successful management of this unresectable MPNST. Complete surgical resection is the best indicator of good prognosis. […] The mainstay of therapy of MPNST is complete surgical resection with negative margins. […] The use of pre- or postoperative adjuvant therapy is not yet well codified. Adjuvant radiotherapy to the tumor bed and possibly other sites of bulky may be beneficial in preventing local tumor relapse.
- #38 Malignant Peripheral Nerve Sheath Tumor (MPNST)https://my.clevelandclinic.org/health/diseases/malignant-peripheral-nerve-sheath-tumor-mpnst
Healthcare providers typically do surgery to remove a malignant peripheral nerve sheath tumor. In some cases, surgery may not be an option for people who have: […] 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: […] Treatments like chemotherapy or radiation may cause side effects, including:
- #39 Malignant peripheral nerve sheath tumorâfrom genetics to multidisciplinary treatment | Czarnecka | Oncology in Clinical Practicehttps://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).
- #40 Azthena logo with the word Azthenahttps://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.
- #41 Effectivebenign Peripheral Nerve Sheath Tumor Treatment: Medical And Surgical Approacheshttps://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.
- #42 Effectivebenign Peripheral Nerve Sheath Tumor Treatment: Medical And Surgical Approacheshttps://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. […] New surgical methods and drug therapies are under study. Clinical trials are working to find effective and safe treatments for these tumors.
- #43 New Frontiers in Therapy of Peripheral Nerve Sheath Tumors in Patients With Neurofibromatosis Type 1: Latest Evidence and Clinical Implications | Anticancer Researchhttps://ar.iiarjournals.org/content/40/4/1817
In addition to expected management with routine imaging and surgical resection, possible alternative treatment options for non-cutaneous PNSTs include cryoablation, radiosurgery, microwave ablation and radiofrequency ablation. […] 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. […] In 2019, U.S. FDA approved selumetinib for the treatment of pediatric patients (3 y) with NF1 symptomatic and/or progressive, inoperable PNFs.
- #44 Immunotherapeutic treatments for spinal and peripheral nerve tumors: a primer in: Neurosurgical Focus Volume 52 Issue 2 (2022) Journalshttps://thejns.org/focus/view/journals/neurosurg-focus/52/2/article-pE8.xml
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. […] 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.
- #45https://link.springer.com/article/10.1007/s10143-023-02107-z
A benign peripheral nerve sheath tumor (bPNST) is a rare lesion associated with peripheral nerval structures. […] Management concepts of bPNST may vary. […] Therefore, we analyzed patients treated for bPNST in our specialized institution with a primary focus on prior misdiagnosis and possible mistreatment. […] An incorrect primary diagnosis was detected in 44.7% (n=38), leading to suboptimal or insufficient treatment in these cases. […] Inappropriate surgery based on prior misdiagnosis, which led to severe neurological deficits in all these cases, was reported in 26.3% (n=10/38). […] For the first time, our data shows the quantity and impact of incorrect initial diagnosis in bPNST causing a delay in causative treatment or resulting in unnecessary or potentially harmful treatment.
- #46 Benign nerve tumours in the upper limb: a registry-based study of symptoms and surgical outcome | Scientific Reportshttps://www.nature.com/articles/s41598-023-38184-9
Surgical treatment with excision is recommended for Schwannomas and neurofibromas causing pain or other symptoms. […] Microsurgical technique is recommended to minimise the risk of damaging healthy nerve fibres. […] Following surgical treatment of nerve tumours, temporary postoperative symptoms, such as pain, paraesthesia, and sensory dysfunction, may be present, but permanent symptoms are reported as unusual. […] We conclude that surgical treatment of benign peripheral nerve tumours in the upper limb usually improve pain modalities and disability. Other symptoms were rather rare both before and after surgery, and patients were generally satisfied with outcome and received care.
- #47https://journals.lww.com/annalsplasticsurgery/fulltext/2009/08000/benign_peripheral_nerve_tumors__treatment.12.aspx
Peripheral nerve tumors are mostly benign; however, their excision can result in profound deficits. Nerve reconstruction strategies offer techniques to minimize morbidity. […] Benign peripheral nerve tumors require highly specialized surgical care. Tumor excision with immediate nerve reconstruction, for fascicles inseparable from the tumor, optimizes outcomes. Nerve reconstruction with available conduits or allografts should be attempted to restore anatomic integrity to any killed fascicles, thereby minimizing possible deficits.
- #48 Nerve Sheath Tumors: Definition & Typeshttps://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. […] 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. […] Nerve sheath tumors are usually noncancerous. They grow slowly, and your healthcare provider may opt for observation if you’re not having any symptoms. When a tumor requires removal, surgery is usually highly effective. Rarely, surgical complications such as incomplete removal of the tumor or damage to the nerve can occur. Very rarely, a tumor can turn into cancer. Your healthcare provider will work with you to develop a plan for treatment and follow-up care to successfully manage your condition.
- #49 Management of peripheral nerve sheath tumors: 17 years of experience at Toronto Western Hospital in: Journal of Neurosurgery Volume 128 Issue 4 (2018) Journalshttps://thejns.org/view/journals/j-neurosurg/128/4/article-p1226.xml
A surgical series of 201 benign and malignant peripheral nerve sheath tumors (PNSTs) was assessed to characterize the anatomical and clinical presentation of tumors and identify predictors of neurological outcome, recurrence, and extent of resection. […] Outcomes following resection of benign PNSTs depend on tumor histopathology, tumor location, and genetic predisposition syndrome. Gross-total resection should be attempted for benign lesions where possible. […] The management of malignant PNSTs remains challenging, requiring a multimodal approach.