Łagodny nowotwór nerwu obwodowego – Zapobieganie i profilaktyka

Łagodny nowotwór nerwu obwodowego
Zapobieganie i profilaktyka

Łagodne nowotwory nerwów obwodowych, takie jak schwannoma i neurofibroma, rozwijają się z komórek osłonek nerwów obwodowych i w około 10% przypadków mają podłoże genetyczne, związane z zespołami predysponującymi, np. nerwiakowłókniakowatością typu 2 (NF2) czy zespołem Carneya. Profilaktyka obejmuje przede wszystkim wczesne wykrycie poprzez badania genetyczne u pacjentów z rodzinnym występowaniem tych guzów oraz regularne kontrole neurologiczne. Zalecane jest utrzymanie zdrowego stylu życia, w tym regularna aktywność fizyczna, zbilansowana dieta oraz unikanie czynników ryzyka uszkodzenia nerwów, takich jak powtarzalne ruchy, nieergonomiczne stanowiska pracy czy urazy mechaniczne. Wczesna diagnostyka obrazowa i kliniczna atypowych nerwiakowłókniaków umożliwia identyfikację zmian predysponujących do transformacji złośliwej (MPNST), co jest kluczowe dla wdrożenia odpowiednich strategii leczenia i zapobiegania.

Profilaktyka łagodnych nowotworów nerwów obwodowych

Genetyczne uwarunkowania i badania przesiewowe
Zdrowy styl życia jako element profilaktyki
Zapobieganie uszkodzeniom nerwów
Regularne kontrole i wczesne wykrywanie

Identyfikacja i leczenie zmian prekursorowych
Leczenie chirurgiczne jako forma profilaktyki wtórnej

Technika mikrochirurgiczna
Wczesna interwencja chirurgiczna
Kolejne rozdziały

Profilaktyka łagodnych nowotworów nerwów obwodowych

Łagodny nowotwór nerwu obwodowego (schwannoma, neurofibroma) należy do grupy guzów osłonek nerwowych, które rozwijają się z komórek tworzących osłonki nerwów obwodowych. Choć całkowita profilaktyka tych nowotworów nie jest obecnie możliwa, istnieją strategie, które mogą przyczynić się do wczesnego wykrycia i zmniejszenia ryzyka powikłań.¹²

Genetyczne uwarunkowania i badania przesiewowe

Niestety, w większości przypadków łagodne nowotwory nerwów obwodowych pojawiają się spontanicznie z nieznanych przyczyn, co sprawia, że ich całkowita prewencja jest niemożliwa. Około 10% przypadków ma jednak podłoże genetyczne, związane z określonymi zespołami predysponującymi do rozwoju tych guzów.³

Lekarz może zalecić przeprowadzenie badań genetycznych w następujących przypadkach:⁴

Występowanie nowotworów osłonek nerwowych w rodzinie
Rozwój mnogich guzów osłonek nerwowych, co może wskazywać na nerwiakowłókniakowatość

⁵

Jeśli u krewnego biologicznego zdiagnozowano nerwiakowłókniakowatość typu 2 (NF2), schwannomatozę lub zespół Carneya, wskazana jest konsultacja z lekarzem, który może zalecić poradnictwo genetyczne w celu oceny ryzyka genetycznego.⁶

Zdrowy styl życia jako element profilaktyki

Jedną z głównych strategii profilaktycznych jest utrzymanie zdrowego stylu życia. Obejmuje to:⁷

Regularna aktywność fizyczna, która wspiera ogólny dobrostan i pomaga w utrzymaniu optymalnego zdrowia nerwów
Zbilansowana dieta bogata w składniki odżywcze wspierające układ nerwowy
Unikanie szkodliwych nawyków, takich jak palenie tytoniu czy nadmierne spożywanie alkoholu

⁸

Zapobieganie uszkodzeniom nerwów

Istotnym elementem profilaktyki jest również świadomość potencjalnych zagrożeń, które mogą prowadzić do uszkodzenia nerwów. Zaleca się:⁹

Unikanie powtarzalnych ruchów lub pozycji, które nadmiernie obciążają nerwy
Stosowanie zasad ergonomii na stanowiskach pracy
Używanie odpowiedniego sprzętu ochronnego podczas aktywności o podwyższonym ryzyku urazu

¹⁰

Regularne kontrole i wczesne wykrywanie

Regularne badania kontrolne u specjalistów są niezbędne dla wczesnego wykrycia i interwencji. Monitorowanie wszelkich zmian lub objawów związanych z nerwami obwodowymi pozwala lekarzom na wczesną diagnostykę łagodnych guzów i wdrożenie odpowiednich opcji terapeutycznych.¹¹¹²

Identyfikacja i leczenie zmian prekursorowych

Niedawna charakterystyka kliniczna i obrazowa atypowych nerwiakowłókniaków może umożliwić wcześniejszą diagnostykę zmian zagrożonych transformacją złośliwą (do MPNST – złośliwego nowotworu osłonek nerwów obwodowych) oraz wdrożenie strategii leczenia i zapobiegania.¹³

Większość złośliwych nowotworów osłonek nerwów obwodowych u pacjentów z nerwiakowłókniakowatością typu 1 (NF1) rozwija się na podłożu istniejących wcześniej nerwiakowłókniaków splotowatych, które są histologicznie łagodnymi guzami osłonek nerwów obwodowych.¹⁴

Biorąc pod uwagę znaczne wyzwania w leczeniu zaawansowanych złośliwych nowotworów, przyszłe działania kliniczne i badawcze powinny koncentrować się na identyfikacji zmian prekursorowych MPNST i, jeśli to możliwe, ich chirurgicznym usunięciu, w celu zapobiegania transformacji złośliwej.¹⁵

Leczenie chirurgiczne jako forma profilaktyki wtórnej

Zabieg chirurgiczny w przypadku łagodnych guzów nerwów jest wykonywany w celu diagnostyki patoanatomicznej i złagodzenia objawów, choć może wiązać się z pewnymi powikłaniami.¹⁶

Leczenie chirurgiczne z wycięciem guza jest zalecane w przypadku schwannoma (nerwiaka osłonkowego) i neurofibroma (nerwiakowłókniaka) powodujących ból lub inne objawy.¹⁷

Technika mikrochirurgiczna

Zaleca się stosowanie techniki mikrochirurgicznej, aby zminimalizować ryzyko uszkodzenia zdrowych włókien nerwowych podczas zabiegu.¹⁸

Po leczeniu chirurgicznym guzów nerwowych mogą wystąpić przejściowe objawy pooperacyjne, takie jak ból, parestezje i zaburzenia czucia, ale trwałe objawy są rzadko zgłaszane.¹⁹

Badania potwierdzają, że leczenie chirurgiczne łagodnych guzów nerwów obwodowych w kończynie górnej zwykle poprawia dolegliwości bólowe i zmniejsza niepełnosprawność. Inne objawy są stosunkowo rzadkie zarówno przed, jak i po operacji, a pacjenci są zazwyczaj zadowoleni z wyników i otrzymanej opieki.²⁰

Wczesna interwencja chirurgiczna

Wczesna interwencja chirurgiczna w przypadku objawowych guzów może zapobiec dalszym powikłaniom neurologicznym i poprawić jakość życia pacjentów. Jest to szczególnie istotne w przypadku guzów umiejscowionych w obszarach, gdzie wzrost może powodować ucisk na ważne struktury nerwowe.²¹²²

Systematyczne monitorowanie pacjentów z grupy zwiększonego ryzyka, zwłaszcza z rozpoznanymi zespołami genetycznymi, pozwala na wczesne wykrycie i leczenie łagodnych nowotworów nerwów obwodowych, co może zapobiec ich potencjalnej transformacji złośliwej.²³

Kolejne rozdziały

Zapraszamy do dalszego czytania naszego leksykonu.

Wybierz kolejny rozdział z menu poniżej, aby otworzyć nową podstronę kompedium wiedzy i uzyskać szczegółowe informację o leku, substancji lub chorobie.

Materiały źródłowe

#1 Nerve Sheath Tumors: Definition & Types
https://my.clevelandclinic.org/health/diseases/22526-nerve-sheath-tumors
There isnt a known way to prevent these types of tumors. Your healthcare provider may recommend genetic testing to better understand you and your familys risk if you: […] Have a family history of nerve sheath tumors. […] Develop multiple nerve sheath tumors, which may be a sign of neurofibromatosis.
#2 Schwannoma: What It Is, Causes, Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/17877-schwannoma
Unfortunately, schwannomas cant be prevented. In most cases, they happen randomly for unknown reasons. About 10% of cases are linked to certain genetic conditions that make it more likely that youll develop certain types of tumors. […] If you have a biological relative whos been diagnosed with neurofibromatosis 2 (NF2), Schwannomatosis or Carney complex, talk to your healthcare provider. They may recommend genetic counseling to see if you also have the genetic condition.
#3 Schwannoma: What It Is, Causes, Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/17877-schwannoma
Unfortunately, schwannomas cant be prevented. In most cases, they happen randomly for unknown reasons. About 10% of cases are linked to certain genetic conditions that make it more likely that youll develop certain types of tumors. […] If you have a biological relative whos been diagnosed with neurofibromatosis 2 (NF2), Schwannomatosis or Carney complex, talk to your healthcare provider. They may recommend genetic counseling to see if you also have the genetic condition.
#4 Nerve Sheath Tumors: Definition & Types
https://my.clevelandclinic.org/health/diseases/22526-nerve-sheath-tumors
There isnt a known way to prevent these types of tumors. Your healthcare provider may recommend genetic testing to better understand you and your familys risk if you: […] Have a family history of nerve sheath tumors. […] Develop multiple nerve sheath tumors, which may be a sign of neurofibromatosis.
#5 Nerve Sheath Tumors: Definition & Types
https://my.clevelandclinic.org/health/diseases/22526-nerve-sheath-tumors
There isnt a known way to prevent these types of tumors. Your healthcare provider may recommend genetic testing to better understand you and your familys risk if you: […] Have a family history of nerve sheath tumors. […] Develop multiple nerve sheath tumors, which may be a sign of neurofibromatosis.
#6 Schwannoma: What It Is, Causes, Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/17877-schwannoma
Unfortunately, schwannomas cant be prevented. In most cases, they happen randomly for unknown reasons. About 10% of cases are linked to certain genetic conditions that make it more likely that youll develop certain types of tumors. […] If you have a biological relative whos been diagnosed with neurofibromatosis 2 (NF2), Schwannomatosis or Carney complex, talk to your healthcare provider. They may recommend genetic counseling to see if you also have the genetic condition.
#7
https://continentalhospitals.com/diseases/benign-peripheral-nerve-tumor/
One of the primary prevention strategies is maintaining a healthy lifestyle. This includes regular exercise, a balanced diet, and avoiding harmful habits such as smoking or excessive alcohol consumption. Engaging in physical activity not only promotes overall well-being but also helps in maintaining optimal nerve health. […] Another important preventive measure is to be mindful of our surroundings and potential hazards that may lead to nerve damage. This includes avoiding repetitive motions or postures that strain nerves, using proper ergonomics at workstations, and wearing protective gear when engaging in activities that carry a higher risk of injury. […] Regular check-ups with healthcare professionals are essential for early detection and intervention. By monitoring any changes or symptoms related to peripheral nerves, healthcare providers can diagnose benign tumors at an early stage and provide appropriate treatment options.
#8
https://continentalhospitals.com/diseases/benign-peripheral-nerve-tumor/
One of the primary prevention strategies is maintaining a healthy lifestyle. This includes regular exercise, a balanced diet, and avoiding harmful habits such as smoking or excessive alcohol consumption. Engaging in physical activity not only promotes overall well-being but also helps in maintaining optimal nerve health. […] Another important preventive measure is to be mindful of our surroundings and potential hazards that may lead to nerve damage. This includes avoiding repetitive motions or postures that strain nerves, using proper ergonomics at workstations, and wearing protective gear when engaging in activities that carry a higher risk of injury. […] Regular check-ups with healthcare professionals are essential for early detection and intervention. By monitoring any changes or symptoms related to peripheral nerves, healthcare providers can diagnose benign tumors at an early stage and provide appropriate treatment options.
#9
https://continentalhospitals.com/diseases/benign-peripheral-nerve-tumor/
One of the primary prevention strategies is maintaining a healthy lifestyle. This includes regular exercise, a balanced diet, and avoiding harmful habits such as smoking or excessive alcohol consumption. Engaging in physical activity not only promotes overall well-being but also helps in maintaining optimal nerve health. […] Another important preventive measure is to be mindful of our surroundings and potential hazards that may lead to nerve damage. This includes avoiding repetitive motions or postures that strain nerves, using proper ergonomics at workstations, and wearing protective gear when engaging in activities that carry a higher risk of injury. […] Regular check-ups with healthcare professionals are essential for early detection and intervention. By monitoring any changes or symptoms related to peripheral nerves, healthcare providers can diagnose benign tumors at an early stage and provide appropriate treatment options.
#10
https://continentalhospitals.com/diseases/benign-peripheral-nerve-tumor/
One of the primary prevention strategies is maintaining a healthy lifestyle. This includes regular exercise, a balanced diet, and avoiding harmful habits such as smoking or excessive alcohol consumption. Engaging in physical activity not only promotes overall well-being but also helps in maintaining optimal nerve health. […] Another important preventive measure is to be mindful of our surroundings and potential hazards that may lead to nerve damage. This includes avoiding repetitive motions or postures that strain nerves, using proper ergonomics at workstations, and wearing protective gear when engaging in activities that carry a higher risk of injury. […] Regular check-ups with healthcare professionals are essential for early detection and intervention. By monitoring any changes or symptoms related to peripheral nerves, healthcare providers can diagnose benign tumors at an early stage and provide appropriate treatment options.
#11
https://continentalhospitals.com/diseases/benign-peripheral-nerve-tumor/
One of the primary prevention strategies is maintaining a healthy lifestyle. This includes regular exercise, a balanced diet, and avoiding harmful habits such as smoking or excessive alcohol consumption. Engaging in physical activity not only promotes overall well-being but also helps in maintaining optimal nerve health. […] Another important preventive measure is to be mindful of our surroundings and potential hazards that may lead to nerve damage. This includes avoiding repetitive motions or postures that strain nerves, using proper ergonomics at workstations, and wearing protective gear when engaging in activities that carry a higher risk of injury. […] Regular check-ups with healthcare professionals are essential for early detection and intervention. By monitoring any changes or symptoms related to peripheral nerves, healthcare providers can diagnose benign tumors at an early stage and provide appropriate treatment options.
#12
https://continentalhospitals.com/diseases/benign-peripheral-nerve-tumor/
In conclusion, prevention plays a vital role in managing benign peripheral nerve tumors. By adopting healthy lifestyle choices, being cautious of potential hazards, and seeking regular medical check-ups, we can take proactive steps towards reducing the risk of developing these tumors and promoting overall nerve health.
#13 Malignant Peripheral Nerve Sheath Tumor | Oncohema Key
https://oncohemakey.com/malignant-peripheral-nerve-sheath-tumor-2/
Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that arise from peripheral nerve sheaths. […] The recent clinical and imaging characterization of atypical neurofibromas may allow for earlier diagnosis of lesions at risk of transforming to MPNST, and for treatment and prevention strategies. […] Most MPNSTs in NF1 develop in preexisting plexiform neurofibromas, which are histologically benign peripheral nerve sheath tumors. […] The recent clinical and imaging characterization of atypical neurofibromas may allow for earlier diagnosis of lesions at risk of transforming to MPNST, and for treatment and prevention strategies. […] Given the great challenges in the treatment of high-grade MPNST, a future focus of clinical care and research should be in the identification of MPNST precursor lesions and surgical resection, if feasible, with the goal of preventing malignant transformation.
#14 Malignant Peripheral Nerve Sheath Tumor | Oncohema Key
https://oncohemakey.com/malignant-peripheral-nerve-sheath-tumor-2/
Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that arise from peripheral nerve sheaths. […] The recent clinical and imaging characterization of atypical neurofibromas may allow for earlier diagnosis of lesions at risk of transforming to MPNST, and for treatment and prevention strategies. […] Most MPNSTs in NF1 develop in preexisting plexiform neurofibromas, which are histologically benign peripheral nerve sheath tumors. […] The recent clinical and imaging characterization of atypical neurofibromas may allow for earlier diagnosis of lesions at risk of transforming to MPNST, and for treatment and prevention strategies. […] Given the great challenges in the treatment of high-grade MPNST, a future focus of clinical care and research should be in the identification of MPNST precursor lesions and surgical resection, if feasible, with the goal of preventing malignant transformation.
#15 Malignant Peripheral Nerve Sheath Tumor | Oncohema Key
https://oncohemakey.com/malignant-peripheral-nerve-sheath-tumor-2/
Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that arise from peripheral nerve sheaths. […] The recent clinical and imaging characterization of atypical neurofibromas may allow for earlier diagnosis of lesions at risk of transforming to MPNST, and for treatment and prevention strategies. […] Most MPNSTs in NF1 develop in preexisting plexiform neurofibromas, which are histologically benign peripheral nerve sheath tumors. […] The recent clinical and imaging characterization of atypical neurofibromas may allow for earlier diagnosis of lesions at risk of transforming to MPNST, and for treatment and prevention strategies. […] Given the great challenges in the treatment of high-grade MPNST, a future focus of clinical care and research should be in the identification of MPNST precursor lesions and surgical resection, if feasible, with the goal of preventing malignant transformation.
#16 Benign nerve tumours in the upper limb: a registry-based study of symptoms and surgical outcome | Scientific Reports
https://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. […] We conclude that surgery for benign peripheral nerve tumours provides good symptomatic relief with low risk for residual problems. […] 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.
#17 Benign nerve tumours in the upper limb: a registry-based study of symptoms and surgical outcome | Scientific Reports
https://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. […] We conclude that surgery for benign peripheral nerve tumours provides good symptomatic relief with low risk for residual problems. […] 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.
#18 Benign nerve tumours in the upper limb: a registry-based study of symptoms and surgical outcome | Scientific Reports
https://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. […] We conclude that surgery for benign peripheral nerve tumours provides good symptomatic relief with low risk for residual problems. […] 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.
#19 Benign nerve tumours in the upper limb: a registry-based study of symptoms and surgical outcome | Scientific Reports
https://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. […] We conclude that surgery for benign peripheral nerve tumours provides good symptomatic relief with low risk for residual problems. […] 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.
#20 Benign nerve tumours in the upper limb: a registry-based study of symptoms and surgical outcome | Scientific Reports
https://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. […] We conclude that surgery for benign peripheral nerve tumours provides good symptomatic relief with low risk for residual problems. […] 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.
#21 Benign nerve tumours in the upper limb: a registry-based study of symptoms and surgical outcome | Scientific Reports
https://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. […] We conclude that surgery for benign peripheral nerve tumours provides good symptomatic relief with low risk for residual problems. […] 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.
#22 Malignant Peripheral Nerve Sheath Tumor | Oncohema Key
https://oncohemakey.com/malignant-peripheral-nerve-sheath-tumor-2/
Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that arise from peripheral nerve sheaths. […] The recent clinical and imaging characterization of atypical neurofibromas may allow for earlier diagnosis of lesions at risk of transforming to MPNST, and for treatment and prevention strategies. […] Most MPNSTs in NF1 develop in preexisting plexiform neurofibromas, which are histologically benign peripheral nerve sheath tumors. […] The recent clinical and imaging characterization of atypical neurofibromas may allow for earlier diagnosis of lesions at risk of transforming to MPNST, and for treatment and prevention strategies. […] Given the great challenges in the treatment of high-grade MPNST, a future focus of clinical care and research should be in the identification of MPNST precursor lesions and surgical resection, if feasible, with the goal of preventing malignant transformation.
#23 Malignant Peripheral Nerve Sheath Tumor | Oncohema Key
https://oncohemakey.com/malignant-peripheral-nerve-sheath-tumor-2/
Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that arise from peripheral nerve sheaths. […] The recent clinical and imaging characterization of atypical neurofibromas may allow for earlier diagnosis of lesions at risk of transforming to MPNST, and for treatment and prevention strategies. […] Most MPNSTs in NF1 develop in preexisting plexiform neurofibromas, which are histologically benign peripheral nerve sheath tumors. […] The recent clinical and imaging characterization of atypical neurofibromas may allow for earlier diagnosis of lesions at risk of transforming to MPNST, and for treatment and prevention strategies. […] Given the great challenges in the treatment of high-grade MPNST, a future focus of clinical care and research should be in the identification of MPNST precursor lesions and surgical resection, if feasible, with the goal of preventing malignant transformation.