Nowotwory nerwów obwodowych
Rokowania, prognozy i postęp choroby

Złośliwe nowotwory osłonek nerwów obwodowych (MPNST) stanowią 5-10% mięsaków tkanek miękkich i charakteryzują się agresywnym przebiegiem oraz słabą odpowiedzią na chemioterapię i radioterapię. Wskaźniki przeżycia całkowitego (OS) wahają się od 30% do 69,5% w ciągu 5 lat, a mediana czasu przeżycia wynosi około 6 lat. Kluczowymi czynnikami negatywnie wpływającymi na rokowanie są: wielkość guza >5 cm, obecność przerzutów przy diagnozie, brak operacji guza pierwotnego, dodatnie marginesy chirurgiczne, lokalizacja w obrębie tułowia, głowy i szyi, głębokość guza pod powięzią, wiek pacjenta >60 lat oraz wysoki stopień złośliwości histologicznej (FNCLCC grade 3). Neurofibromatoza typu 1 (NF1) jest istotnym czynnikiem ryzyka, zwiększającym ryzyko MPNST niemal 5000-krotnie i wiążącym się z gorszym 5-letnim przeżyciem całkowitym na poziomie 32% oraz niższą odpowiedzią na chemioterapię (17,6% vs 55,3% u pacjentów bez NF1). Kompleksowa resekcja chirurgiczna z ujemnymi marginesami pozostaje podstawą leczenia, znacząco poprawiając wskaźniki OS i przeżycia bez wznowy miejscowej (LRFS).

  1. Wprowadzenie do nowotworów nerwów obwodowych
  2. Ogólne wskaźniki przeżycia
  3. Czynniki prognostyczne wpływające na rokowanie
    1. Czynniki kliniczne
    2. Czynniki histopatologiczne i molekularne
    3. Związek z neurofibromatozą typu 1
  4. Różnice w rokowaniu w porównaniu z łagodnymi guzami nerwów obwodowych
  5. Wpływ metod leczenia na rokowanie
    1. Znaczenie leczenia chirurgicznego
    2. Wpływ terapii uzupełniających
  6. Wskaźniki nawrotów i przerzutów
  7. Perspektywy i przyszłe kierunki
  8. Podsumowanie czynników prognostycznych
    1. Kolejne rozdziały

Wprowadzenie do nowotworów nerwów obwodowych

Nowotwory nerwów obwodowych, szczególnie złośliwe nowotwory osłonek nerwów obwodowych (MPNST – Malignant Peripheral Nerve Sheath Tumors), stanowią rzadką, ale wysoce agresywną grupę mięsaków tkanek miękkich, stanowiącą około 5-10% wszystkich mięsaków tkanek miękkich.1 Pomimo postępów w leczeniu mięsaków dziecięcych, MPNST słabo reagują na chemioterapię i radioterapię, co wpływa na ich niekorzystne rokowanie.2 W praktyce klinicznej poza resekcją chirurgiczną nie istnieją w pełni skuteczne opcje leczenia tego agresywnego nowotworu pochodzenia neuroektodermalnego.3

Ogólne wskaźniki przeżycia

Nowotwory nerwów obwodowych charakteryzują się zróżnicowanym rokowaniem, zależnym od wielu czynników. Wskaźniki przeżycia dla MPNST według dostępnych badań przedstawiają się następująco:

  • 5-letni wskaźnik przeżycia całkowitego (OS) waha się między 30% a 69,5%4567
  • 3-letni wskaźnik przeżycia całkowitego wynosi około 50,0%8
  • 5-letni wskaźnik przeżycia bez zdarzeń (EFS) osiąga około 37%9
  • 3- i 5-letni wskaźnik przeżycia bez guza (TFS) wynosi odpowiednio 40,0% i 34,0%10
  • Mediana czasu przeżycia MPNST wynosi około sześciu lat11

Wśród pacjentów z chorobą zlokalizowaną w momencie diagnozy, którzy przeszli operację guza pierwotnego, 5-letnie wskaźniki przeżycia całkowitego (OS), przeżycia bez wznowy miejscowej (LRFS) i przeżycia bez przerzutów (MFS) wynoszą odpowiednio 81,1%, 78,2% i 70,3%.12 Ogólnie, pomimo złożonej terapii wielomodalnej, MPNST zachowuje się jak agresywny mięsak ze skłonnością do nawrotów miejscowych lub przerzutów do odległych narządów.13

Czynniki prognostyczne wpływające na rokowanie

Czynniki kliniczne

Na podstawie analizy jednowymiarowej i wielowymiarowej zidentyfikowano następujące kliniczne czynniki prognostyczne wpływające na rokowanie w MPNST:

  • Wielkość guza – guzy o średnicy powyżej 5 cm znacząco zmniejszają przeżycie specyficzne dla choroby (DSS) i przeżycie całkowite (OS)1415
  • Obecność przerzutów w momencie diagnozy – istotnie związana z gorszym OS16
  • Brak operacji guza pierwotnego – pacjenci niepoddani operacji mają gorsze rokowanie17
  • Stan marginesów chirurgicznych – dodatni margines chirurgiczny wiąże się z niekorzystnym OS i LRFS18
  • Stopień zaawansowania wg AJCC – wyższy stopień zaawansowania jest niezależnym czynnikiem związanym z krótszym OS19
  • Lokalizacja guza – guzy zlokalizowane w obrębie tułowia oraz głowy i szyi wiążą się z gorszym rokowaniem20
  • Głębokość guza – guzy położone pod powięzią mają gorsze rokowanie2122
  • Wiek pacjenta – wiek powyżej 60 lat jest uważany za niezależny predyktor gorszego rokowania23

Czynniki histopatologiczne i molekularne

Istotne znaczenie prognostyczne mają również parametry histopatologiczne i biomarkery molekularne:

  • Stopień złośliwości histologicznej – wysoki stopień (grade) jest związany z niekorzystnym MFS i gorszym rokowaniem2425
  • Stopień FNCLCC – wysoki stopień MPNST (zwłaszcza grade 3) silnie koreluje z gorszymi wynikami przeżycia2627
  • Ekspresja S-100 – status S-100 jest niezależnym czynnikiem wpływającym na OS, przy czym S-100 negatywny status wiąże się z niekorzystnym rokowaniem2829
  • Ekspresja Ki67 – wysoki indeks proliferacyjny Ki67 wpływa na OS30
  • Utrata H3K27me3 – występuje częściej w MPNST związanych z neurofibromatozą typu 1 w porównaniu ze sporadycznymi przypadkami i jest szczególnie częsta w guzach o wyższym stopniu złośliwości oraz związanych z radioterapią31
  • Nadekspresja surwiwiny – zwiększone poziomy transkryptów surwiwiny korelują z wyższym stopniem FNCLCC i niższym prawdopodobieństwem przeżycia3233

Związek z neurofibromatozą typu 1

Neurofibromatoza typu 1 (NF1) jest istotnym czynnikiem prognostycznym w MPNST:

  • Prawie połowa pacjentów z MPNST ma NF1, zespół predysponujący do rozwoju nowotworów, który zwiększa ryzyko MPNST prawie 5000 razy w porównaniu z populacją ogólną34
  • Pacjenci z MPNST i NF1 mają gorsze rokowanie – 5-letnie przeżycie całkowite na poziomie 32%35
  • Wskaźnik odpowiedzi na chemioterapię jest niższy u pacjentów z NF1 (17,6% vs 55,3% u pacjentów bez NF1)36
  • Mutacja NF1 wiąże się z gorszym przeżyciem w porównaniu ze sporadycznym MPNST37

Różnice w rokowaniu w porównaniu z łagodnymi guzami nerwów obwodowych

Istotne różnice rokowania obserwuje się między MPNST a łagodnymi guzami nerwów obwodowych, takimi jak komórkowy schwannoma:

  • 5-letnie wskaźniki przeżycia bez progresji wynoszą 100% dla komórkowego schwannoma i jedynie 18% dla MPNST38
  • 5-letnie wskaźniki przeżycia specyficznego dla choroby wynoszą 100% dla komórkowego schwannoma i 32% dla MPNST3940
  • W przeciwieństwie do pacjentów z MPNST, u których przerzuty występują u 37,3% chorych, u pacjentów z komórkowym schwannoma nie stwierdzono przerzutów ani zgonów związanych z chorobą4142

Te drastyczne różnice w rokowaniu podkreślają znaczenie właściwej diagnostyki różnicowej między MPNST a komórkowym schwannoma, biorąc pod uwagę diametralnie różne implikacje terapeutyczne i prognostyczne.4344

Wpływ metod leczenia na rokowanie

Znaczenie leczenia chirurgicznego

Leczenie chirurgiczne pozostaje najważniejszym elementem terapii wpływającym na rokowanie w MPNST:

  • Kompletna resekcja chirurgiczna z ujemnymi marginesami jest konieczna dla pomyślnego leczenia MPNST45
  • W analizie wieloczynnikowej operacja guza pierwotnego była istotnie związana z korzystnym OS46
  • 5-letni wskaźnik OS pacjentów, którzy przeszli całkowitą resekcję, wynosi 45,0%47
  • Ujemny margines chirurgiczny jest znacząco związany z lepszym OS i LRFS48
  • Resekcja z ujemnymi marginesami jest czynnikiem ochronnym dla lepszego rokowania49

Wpływ terapii uzupełniających

Terapie uzupełniające mogą wpływać na rokowanie w MPNST, zwłaszcza w określonych sytuacjach klinicznych:

  • Chemioterapia i radioterapia są istotne dla poprawy przeżycia, szczególnie u pacjentów z dodatnimi marginesami50
  • Chirurgia i/lub chemioterapia systemowa dla zmian przerzutowych może zwiększyć przeżycie pacjentów z przerzutami odległymi, którzy przeszli resekcję chirurgiczną guza pierwotnego51
  • Ogólne doświadczenie kliniczne wskazuje jednak, że pacjenci z MPNST słabo reagują na leczenie uzupełniające52
  • Wskaźnik odpowiedzi na chemioterapię jest niższy u pacjentów z NF1 (17,6%) w porównaniu z pacjentami bez NF1 (55,3%)53

Wskaźniki nawrotów i przerzutów

MPNST charakteryzuje się wysokim ryzykiem wznowy miejscowej i przerzutów odległych, co wpływa znacząco na rokowanie:

  • Łączny wskaźnik wznowy miejscowej (LR) wynosi 38% (zakres 13-86%)54
  • 5-letni wskaźnik przeżycia bez wznowy miejscowej (LRFS) u pacjentów z chorobą zlokalizowaną wynosi 78,2%55
  • 5-letni wskaźnik przeżycia bez przerzutów (MFS) wynosi 70,3% u pacjentów z chorobą zlokalizowaną56
  • Wysoki stopień złośliwości jest niekorzystnym wskaźnikiem prognostycznym dla MFS57

MPNST ma tendencję do nawracania miejscowo lub tworzenia przerzutów do odległych miejsc, co podkreśla potrzebę agresywnej i skutecznej terapii w celu kontrolowania wznowy miejscowej.5859

Perspektywy i przyszłe kierunki

Obecne badania skupiają się na identyfikacji nowych biomarkerów i celów terapeutycznych mogących poprawić rokowanie w MPNST:

  • Identyfikacja predyktorów prognostycznych jest niezbędna dla dokładnej diagnozy i wyboru leczenia60
  • Nadekspresja surwiwiny w przypadkach dziecięcych podkreśla jej potencjalną rolę jako celu interwencji terapeutycznych61
  • Wartość H3K27me3 jako markera predykcyjnego dla wrażliwości na terapie oparte na modyfikacjach epigenetycznych pozostaje do określenia62
  • Zaproponowano badanie kliniczne z terapią ukierunkowaną w MPNST opartą na ekspresji biomarkerów, które są również celami dla znanych leków cytostatycznych, takich jak etopozyd i 5-FU, rzadko stosowanych w leczeniu MPNST63

Zaleca się wielodyscyplinarne podejście terapeutyczne dla pacjentów z MPNST o agresywnych cechach, aby zmaksymalizować dobre rokowanie.64 Istnieje duża potrzeba nowych opcji leczenia dla tej grupy pacjentów, biorąc pod uwagę ograniczoną skuteczność obecnie dostępnych terapii.65

Podsumowanie czynników prognostycznych

Podsumowując, kluczowe czynniki wpływające na rokowanie w nowotworach nerwów obwodowych obejmują:

Kategoria Czynniki negatywne Czynniki pozytywne
Kliniczne – Wielkość guza >5 cm
– Przerzuty w momencie diagnozy
– Brak operacji guza pierwotnego
– Lokalizacja w obrębie tułowia, głowy i szyi
– Guz położony pod powięzią
– Wiek >60 lat 		– Choroba zlokalizowana w momencie diagnozy
– Możliwość całkowitej resekcji chirurgicznej
– Ujemne marginesy chirurgiczne
Histopatologiczne i molekularne – Wysoki stopień złośliwości histologicznej
– Wysoki stopień FNCLCC (grade 3)
– Negatywny status S-100
– Wysoki indeks Ki67
– Utrata H3K27me3
– Nadekspresja surwiwiny 		– Niski stopień złośliwości histologicznej (grade 1-2)
– Pozytywny status S-100
– Niski indeks Ki67
Związane z chorobą – Neurofibromatoza typu 1 (NF1)
– Wyższy stopień zaawansowania wg AJCC
– MPNST związany z radioterapią 		– Sporadyczny MPNST (bez NF1)
– Niższy stopień zaawansowania wg AJCC
Terapeutyczne – Dodatnie marginesy chirurgiczne
– Brak możliwości leczenia chirurgicznego
– Słaba odpowiedź na chemioterapię (szczególnie u pacjentów z NF1) 		– Całkowita resekcja chirurgiczna
– Ujemne marginesy chirurgiczne
– Wielodyscyplinarne podejście terapeutyczne
– Terapie uzupełniające w wybranych przypadkach

Pomimo postępu w rozumieniu biologii i leczeniu nowotworów nerwów obwodowych, MPNST pozostaje trudnym do diagnozowania i leczenia nowotworem o ogólnie złym rokowaniu.66 Kompleksowe zrozumienie czynników prognostycznych może pomóc w stratyfikacji ryzyka pacjentów i optymalizacji indywidualnych strategii leczenia.

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  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Survivin Expression and Prognostic Significance in Pediatric Malignant Peripheral Nerve Sheath Tumors (MPNST) | PLOS One
    https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0080456
    Malignant peripheral nerve sheath tumors (MPNST) are very aggressive malignancies comprising approximately 510% of all soft tissue sarcomas. […] Survivin mRNA was overexpressed in pediatric MPNST and associated to a copy number gain of BIRC5; furthermore, increased levels of transcripts correlated with a higher FNCLCC tumor grade (grade 1 and 2 vs. 3, p=0.0067), and with a lower survival probability (Log-rank test, p=0.0038). Overall, these data support the concept that survivin can be regarded as a useful prognostic marker for pediatric MPNST and a promising target for therapeutic interventions. […] Despite progress in the treatment of pediatric sarcomas, MPNST are poorly responsive to chemotherapy and radiotherapy, and their 5-year survival rate ranges from 82% for patients in Intergroup Rhabdomyosarcoma Study (IRS) stage I, to 26% for those affected by metastatic disease (IRS stage IV).
  • #2 Survivin Expression and Prognostic Significance in Pediatric Malignant Peripheral Nerve Sheath Tumors (MPNST) | PLOS One
    https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0080456
    Malignant peripheral nerve sheath tumors (MPNST) are very aggressive malignancies comprising approximately 510% of all soft tissue sarcomas. […] Survivin mRNA was overexpressed in pediatric MPNST and associated to a copy number gain of BIRC5; furthermore, increased levels of transcripts correlated with a higher FNCLCC tumor grade (grade 1 and 2 vs. 3, p=0.0067), and with a lower survival probability (Log-rank test, p=0.0038). Overall, these data support the concept that survivin can be regarded as a useful prognostic marker for pediatric MPNST and a promising target for therapeutic interventions. […] Despite progress in the treatment of pediatric sarcomas, MPNST are poorly responsive to chemotherapy and radiotherapy, and their 5-year survival rate ranges from 82% for patients in Intergroup Rhabdomyosarcoma Study (IRS) stage I, to 26% for those affected by metastatic disease (IRS stage IV).
  • #3 Adjuvant Treatment For Patients With Malignant Peripheral Nerve S
    https://www.longdom.org/open-access/adjuvant-treatment-for-patients-with-malignant-peripheral-nerve-sheathtumours-22489.html
    In current clinical practice, there are no curative treatment options for the aggressive neuroectodermal cancer malignant peripheral nerve sheath tumor (MPNST) beyond surgical resection. Nearly one half of the patients have neurofibromatosis type 1 (NF1), a cancer predisposition syndrome that increases the risk of MPNST almost 5000 times as compared to the general population, and MPNST patients with NF1 have been reported to have worse prognosis, although the differences in outcome seem to have diminished in recent years. For both NF1 and non-NF1 patients, the 5-year overall survival after MPNST diagnosis is between 30 and 50%. […] The general clinical experience with MPNST is that these patients respond poorly to adjuvant treatment, and there is a great need for new treatment options for this patient group. We have recently described an IHC profile in MPNST identifying prognostic factors that are also targets to the well-known cytostatic drugs, etoposide and 5-FU rarely used in the treatment of MPNST. To further explore these findings, we suggest a clinical trial guiding therapy in MPNST based on the expression of these biomarkers.
  • #4 Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8635905/
    Few studies have described the characteristics and prognostic factors of patients with malignant peripheral nerve sheath tumour (MPNST). […] The 5-year overall survival (OS) rate of all patients was 69.5%. […] Univariate analysis revealed that large-sized tumour, metastasis at diagnosis, and no surgery of the primary tumour were significantly associated with patients with worse OS. […] Multivariate analysis identified surgery of the primary tumour as an independent prognostic factor for improved OS. […] Among patients with localised disease at diagnosis who underwent surgery of the primary tumour at our institutions, the 5-year OS, local recurrence-free survival (LRFS), and metastasis-free survival (MFS) rates were 81.1%, 78.2%, and 70.3%, respectively. […] Univariate analysis revealed that positive surgical margin was significantly correlated with unfavourable OS and LRFS, and high grade was a poor prognostic indicator for MFS.
  • #5 Clinicopathological features and prognosis of malignant peripheral nerve sheath tumor: a retrospective study of 159 cases from 1999 to 2016 | Oncotarget
    https://www.oncotarget.com/article/18975/text/
    A total of 159 patients with MPNST were enrolled in the study. The 3- and 5-year overall survival rates were 50.0% and 43.0%, respectively. Multivariate analysis showed that AJCC stage and S-100 were independent factors affecting overall survival (p 0.05 for both). […] MPSNT is a highly aggressive tumor with poor prognosis and this study may be useful for prognostic assessment and management decisions. […] The 3- and 5-year overall survival rates of the whole group were 50.0% and 43.0%, respectively (Table 1). […] The 5-year OS rate of 140 patients who underwent a complete resection was 45.0%. […] Univariate analysis showed that the location and the depth of tumor, AJCC stage, margin status, S-100 and Ki67 staining independently affected OS (p 0.05, Tables 2 and 4). However, by multivariate analysis, only AJCC stage and S-100 were factors associated with prolonged OS (p 0.05, Table 5).
  • #6 Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis | World Journal of Surgical Oncology | Full Text
    https://wjso.biomedcentral.com/articles/10.1186/s12957-020-02036-x
    No available meta-analysis was printed to systematically introduce the MPNST clinic outcome and risk factors based on largely pooled data. […] The pooled 5-year OS rate, 5-year EFS rate, and LR rate were 49%, 37%, and 38%, respectively. […] Survival and local recurrence of MPNST are poor. Worse prognosis is mainly associated with NF 1, large size, deep to fascia, high grade, metastases, and location (trunk and head and neck). Complete resection with adequate surgical margins is the mainstay protective factor of MPNST patients, following necessary adjuvant therapies. […] The 5-year OS rate was 49% (range, 1663%), which consisted with the most published studies. […] The pooled LR rate was 37% (range, 1386%). This result also revealed that aggressively effective therapy was necessary to be applied in order to control local recurrence.
  • #7 Adjuvant Treatment For Patients With Malignant Peripheral Nerve S
    https://www.longdom.org/open-access/adjuvant-treatment-for-patients-with-malignant-peripheral-nerve-sheathtumours-22489.html
    In current clinical practice, there are no curative treatment options for the aggressive neuroectodermal cancer malignant peripheral nerve sheath tumor (MPNST) beyond surgical resection. Nearly one half of the patients have neurofibromatosis type 1 (NF1), a cancer predisposition syndrome that increases the risk of MPNST almost 5000 times as compared to the general population, and MPNST patients with NF1 have been reported to have worse prognosis, although the differences in outcome seem to have diminished in recent years. For both NF1 and non-NF1 patients, the 5-year overall survival after MPNST diagnosis is between 30 and 50%. […] The general clinical experience with MPNST is that these patients respond poorly to adjuvant treatment, and there is a great need for new treatment options for this patient group. We have recently described an IHC profile in MPNST identifying prognostic factors that are also targets to the well-known cytostatic drugs, etoposide and 5-FU rarely used in the treatment of MPNST. To further explore these findings, we suggest a clinical trial guiding therapy in MPNST based on the expression of these biomarkers.
  • #8 Clinicopathological features and prognosis of malignant peripheral nerve sheath tumor: a retrospective study of 159 cases from 1999 to 2016 | Oncotarget
    https://www.oncotarget.com/article/18975/text/
    A total of 159 patients with MPNST were enrolled in the study. The 3- and 5-year overall survival rates were 50.0% and 43.0%, respectively. Multivariate analysis showed that AJCC stage and S-100 were independent factors affecting overall survival (p 0.05 for both). […] MPSNT is a highly aggressive tumor with poor prognosis and this study may be useful for prognostic assessment and management decisions. […] The 3- and 5-year overall survival rates of the whole group were 50.0% and 43.0%, respectively (Table 1). […] The 5-year OS rate of 140 patients who underwent a complete resection was 45.0%. […] Univariate analysis showed that the location and the depth of tumor, AJCC stage, margin status, S-100 and Ki67 staining independently affected OS (p 0.05, Tables 2 and 4). However, by multivariate analysis, only AJCC stage and S-100 were factors associated with prolonged OS (p 0.05, Table 5).
  • #9 Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis | World Journal of Surgical Oncology | Full Text
    https://wjso.biomedcentral.com/articles/10.1186/s12957-020-02036-x
    No available meta-analysis was printed to systematically introduce the MPNST clinic outcome and risk factors based on largely pooled data. […] The pooled 5-year OS rate, 5-year EFS rate, and LR rate were 49%, 37%, and 38%, respectively. […] Survival and local recurrence of MPNST are poor. Worse prognosis is mainly associated with NF 1, large size, deep to fascia, high grade, metastases, and location (trunk and head and neck). Complete resection with adequate surgical margins is the mainstay protective factor of MPNST patients, following necessary adjuvant therapies. […] The 5-year OS rate was 49% (range, 1663%), which consisted with the most published studies. […] The pooled LR rate was 37% (range, 1386%). This result also revealed that aggressively effective therapy was necessary to be applied in order to control local recurrence.
  • #10 Clinicopathological features and prognosis of malignant peripheral nerve sheath tumor: a retrospective study of 159 cases from 1999 to 2016 | Oncotarget
    https://www.oncotarget.com/article/18975/text/
    The 3-and 5-year TFS rates for the 140 patients were 40.0% and 34.0%, respectively. […] Data showed that the location, the depth, the size, the AJCC stage of the tumor, and S-100 were associated with the tumor-free survival. Whereas, late AJCC stage and S-100 negative were independent unfavorable factors affecting OS. […] In summary, despite combined multimodality therapy, MPNST behaves as an aggressive sarcoma with a propensity to recur locally or to metastasize to distant sites.
  • #11 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management
    https://www.mdpi.com/2072-6694/15/4/1077
    Malignant peripheral nerve sheath tumor (MPNST) is a soft tissue sarcoma with limited therapeutic interventions and a poor prognosis. […] MPNST is still difficult to diagnose and treat, and the overall prognosis is poor. Although MPNST is a rare disease, the mortality rate is high. The median survival time is largely based on subtypes of MPNST and molecular variations. […] MPNST has a poor prognosis on average. Previous studies have shown that the five-year overall survival rate is 50–60%, and the median survival of MPNST is six years. […] The identification of prognostic predictors is necessary for accurate diagnosis and treatment selection. […] Recent studies have revealed a strong correlation between high FNCLCC grade MPNST and survival outcomes. […] NF1 mutation is associated with worse survival than sporadic MPNST.
  • #12 Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8635905/
    Few studies have described the characteristics and prognostic factors of patients with malignant peripheral nerve sheath tumour (MPNST). […] The 5-year overall survival (OS) rate of all patients was 69.5%. […] Univariate analysis revealed that large-sized tumour, metastasis at diagnosis, and no surgery of the primary tumour were significantly associated with patients with worse OS. […] Multivariate analysis identified surgery of the primary tumour as an independent prognostic factor for improved OS. […] Among patients with localised disease at diagnosis who underwent surgery of the primary tumour at our institutions, the 5-year OS, local recurrence-free survival (LRFS), and metastasis-free survival (MFS) rates were 81.1%, 78.2%, and 70.3%, respectively. […] Univariate analysis revealed that positive surgical margin was significantly correlated with unfavourable OS and LRFS, and high grade was a poor prognostic indicator for MFS.
  • #13 Clinicopathological features and prognosis of malignant peripheral nerve sheath tumor: a retrospective study of 159 cases from 1999 to 2016 | Oncotarget
    https://www.oncotarget.com/article/18975/text/
    The 3-and 5-year TFS rates for the 140 patients were 40.0% and 34.0%, respectively. […] Data showed that the location, the depth, the size, the AJCC stage of the tumor, and S-100 were associated with the tumor-free survival. Whereas, late AJCC stage and S-100 negative were independent unfavorable factors affecting OS. […] In summary, despite combined multimodality therapy, MPNST behaves as an aggressive sarcoma with a propensity to recur locally or to metastasize to distant sites.
  • #14 Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8635905/
    Few studies have described the characteristics and prognostic factors of patients with malignant peripheral nerve sheath tumour (MPNST). […] The 5-year overall survival (OS) rate of all patients was 69.5%. […] Univariate analysis revealed that large-sized tumour, metastasis at diagnosis, and no surgery of the primary tumour were significantly associated with patients with worse OS. […] Multivariate analysis identified surgery of the primary tumour as an independent prognostic factor for improved OS. […] Among patients with localised disease at diagnosis who underwent surgery of the primary tumour at our institutions, the 5-year OS, local recurrence-free survival (LRFS), and metastasis-free survival (MFS) rates were 81.1%, 78.2%, and 70.3%, respectively. […] Univariate analysis revealed that positive surgical margin was significantly correlated with unfavourable OS and LRFS, and high grade was a poor prognostic indicator for MFS.
  • #15 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management
    https://www.mdpi.com/2072-6694/15/4/1077
    Age over 60 years was also considered an independent predictor, although it was rarely reported. […] Some studies show that tumors larger than 5 cm significantly reduced disease-specific survival (DSS) and overall survival (OS). […] The influence of different therapeutic methods is under investigation.
  • #16 Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8635905/
    Few studies have described the characteristics and prognostic factors of patients with malignant peripheral nerve sheath tumour (MPNST). […] The 5-year overall survival (OS) rate of all patients was 69.5%. […] Univariate analysis revealed that large-sized tumour, metastasis at diagnosis, and no surgery of the primary tumour were significantly associated with patients with worse OS. […] Multivariate analysis identified surgery of the primary tumour as an independent prognostic factor for improved OS. […] Among patients with localised disease at diagnosis who underwent surgery of the primary tumour at our institutions, the 5-year OS, local recurrence-free survival (LRFS), and metastasis-free survival (MFS) rates were 81.1%, 78.2%, and 70.3%, respectively. […] Univariate analysis revealed that positive surgical margin was significantly correlated with unfavourable OS and LRFS, and high grade was a poor prognostic indicator for MFS.
  • #17 Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8635905/
    Few studies have described the characteristics and prognostic factors of patients with malignant peripheral nerve sheath tumour (MPNST). […] The 5-year overall survival (OS) rate of all patients was 69.5%. […] Univariate analysis revealed that large-sized tumour, metastasis at diagnosis, and no surgery of the primary tumour were significantly associated with patients with worse OS. […] Multivariate analysis identified surgery of the primary tumour as an independent prognostic factor for improved OS. […] Among patients with localised disease at diagnosis who underwent surgery of the primary tumour at our institutions, the 5-year OS, local recurrence-free survival (LRFS), and metastasis-free survival (MFS) rates were 81.1%, 78.2%, and 70.3%, respectively. […] Univariate analysis revealed that positive surgical margin was significantly correlated with unfavourable OS and LRFS, and high grade was a poor prognostic indicator for MFS.
  • #18 Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8635905/
    Few studies have described the characteristics and prognostic factors of patients with malignant peripheral nerve sheath tumour (MPNST). […] The 5-year overall survival (OS) rate of all patients was 69.5%. […] Univariate analysis revealed that large-sized tumour, metastasis at diagnosis, and no surgery of the primary tumour were significantly associated with patients with worse OS. […] Multivariate analysis identified surgery of the primary tumour as an independent prognostic factor for improved OS. […] Among patients with localised disease at diagnosis who underwent surgery of the primary tumour at our institutions, the 5-year OS, local recurrence-free survival (LRFS), and metastasis-free survival (MFS) rates were 81.1%, 78.2%, and 70.3%, respectively. […] Univariate analysis revealed that positive surgical margin was significantly correlated with unfavourable OS and LRFS, and high grade was a poor prognostic indicator for MFS.
  • #19 Clinicopathological features and prognosis of malignant peripheral nerve sheath tumor: a retrospective study of 159 cases from 1999 to 2016 | Oncotarget
    https://www.oncotarget.com/article/18975/text/
    A total of 159 patients with MPNST were enrolled in the study. The 3- and 5-year overall survival rates were 50.0% and 43.0%, respectively. Multivariate analysis showed that AJCC stage and S-100 were independent factors affecting overall survival (p 0.05 for both). […] MPSNT is a highly aggressive tumor with poor prognosis and this study may be useful for prognostic assessment and management decisions. […] The 3- and 5-year overall survival rates of the whole group were 50.0% and 43.0%, respectively (Table 1). […] The 5-year OS rate of 140 patients who underwent a complete resection was 45.0%. […] Univariate analysis showed that the location and the depth of tumor, AJCC stage, margin status, S-100 and Ki67 staining independently affected OS (p 0.05, Tables 2 and 4). However, by multivariate analysis, only AJCC stage and S-100 were factors associated with prolonged OS (p 0.05, Table 5).
  • #20 Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis | World Journal of Surgical Oncology | Full Text
    https://wjso.biomedcentral.com/articles/10.1186/s12957-020-02036-x
    No available meta-analysis was printed to systematically introduce the MPNST clinic outcome and risk factors based on largely pooled data. […] The pooled 5-year OS rate, 5-year EFS rate, and LR rate were 49%, 37%, and 38%, respectively. […] Survival and local recurrence of MPNST are poor. Worse prognosis is mainly associated with NF 1, large size, deep to fascia, high grade, metastases, and location (trunk and head and neck). Complete resection with adequate surgical margins is the mainstay protective factor of MPNST patients, following necessary adjuvant therapies. […] The 5-year OS rate was 49% (range, 1663%), which consisted with the most published studies. […] The pooled LR rate was 37% (range, 1386%). This result also revealed that aggressively effective therapy was necessary to be applied in order to control local recurrence.
  • #21 Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis | World Journal of Surgical Oncology | Full Text
    https://wjso.biomedcentral.com/articles/10.1186/s12957-020-02036-x
    No available meta-analysis was printed to systematically introduce the MPNST clinic outcome and risk factors based on largely pooled data. […] The pooled 5-year OS rate, 5-year EFS rate, and LR rate were 49%, 37%, and 38%, respectively. […] Survival and local recurrence of MPNST are poor. Worse prognosis is mainly associated with NF 1, large size, deep to fascia, high grade, metastases, and location (trunk and head and neck). Complete resection with adequate surgical margins is the mainstay protective factor of MPNST patients, following necessary adjuvant therapies. […] The 5-year OS rate was 49% (range, 1663%), which consisted with the most published studies. […] The pooled LR rate was 37% (range, 1386%). This result also revealed that aggressively effective therapy was necessary to be applied in order to control local recurrence.
  • #22 Clinicopathological features and prognosis of malignant peripheral nerve sheath tumor: a retrospective study of 159 cases from 1999 to 2016 | Oncotarget
    https://www.oncotarget.com/article/18975/text/
    A total of 159 patients with MPNST were enrolled in the study. The 3- and 5-year overall survival rates were 50.0% and 43.0%, respectively. Multivariate analysis showed that AJCC stage and S-100 were independent factors affecting overall survival (p 0.05 for both). […] MPSNT is a highly aggressive tumor with poor prognosis and this study may be useful for prognostic assessment and management decisions. […] The 3- and 5-year overall survival rates of the whole group were 50.0% and 43.0%, respectively (Table 1). […] The 5-year OS rate of 140 patients who underwent a complete resection was 45.0%. […] Univariate analysis showed that the location and the depth of tumor, AJCC stage, margin status, S-100 and Ki67 staining independently affected OS (p 0.05, Tables 2 and 4). However, by multivariate analysis, only AJCC stage and S-100 were factors associated with prolonged OS (p 0.05, Table 5).
  • #23 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management
    https://www.mdpi.com/2072-6694/15/4/1077
    Age over 60 years was also considered an independent predictor, although it was rarely reported. […] Some studies show that tumors larger than 5 cm significantly reduced disease-specific survival (DSS) and overall survival (OS). […] The influence of different therapeutic methods is under investigation.
  • #24 Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8635905/
    Few studies have described the characteristics and prognostic factors of patients with malignant peripheral nerve sheath tumour (MPNST). […] The 5-year overall survival (OS) rate of all patients was 69.5%. […] Univariate analysis revealed that large-sized tumour, metastasis at diagnosis, and no surgery of the primary tumour were significantly associated with patients with worse OS. […] Multivariate analysis identified surgery of the primary tumour as an independent prognostic factor for improved OS. […] Among patients with localised disease at diagnosis who underwent surgery of the primary tumour at our institutions, the 5-year OS, local recurrence-free survival (LRFS), and metastasis-free survival (MFS) rates were 81.1%, 78.2%, and 70.3%, respectively. […] Univariate analysis revealed that positive surgical margin was significantly correlated with unfavourable OS and LRFS, and high grade was a poor prognostic indicator for MFS.
  • #25 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management
    https://www.mdpi.com/2072-6694/15/4/1077
    Malignant peripheral nerve sheath tumor (MPNST) is a soft tissue sarcoma with limited therapeutic interventions and a poor prognosis. […] MPNST is still difficult to diagnose and treat, and the overall prognosis is poor. Although MPNST is a rare disease, the mortality rate is high. The median survival time is largely based on subtypes of MPNST and molecular variations. […] MPNST has a poor prognosis on average. Previous studies have shown that the five-year overall survival rate is 50–60%, and the median survival of MPNST is six years. […] The identification of prognostic predictors is necessary for accurate diagnosis and treatment selection. […] Recent studies have revealed a strong correlation between high FNCLCC grade MPNST and survival outcomes. […] NF1 mutation is associated with worse survival than sporadic MPNST.
  • #26 Survivin Expression and Prognostic Significance in Pediatric Malignant Peripheral Nerve Sheath Tumors (MPNST) | PLOS One
    https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0080456
    For MPNST occurring in patients with NF1, the rate of response to chemotherapy is lower (17.6% vs. 55.3% in patients without NF1), and the outcome is worse with a 5-year overall survival of 32%. […] The survival analysis revealed an association between low levels of survivin mRNA and long-term survival (Figure 2A; Log-rank test, p=0.0038). FNCLCC Grade 1 and 2 MPNST had a more prolonged survival when compared to FNCLCC grade 3 (Figure 2B; Log-rank test, p=0.0322). […] High levels of survivin mRNA were significantly associated with FNCLCC tumor grade 3 and a high mitotic rate. […] An important feature for survivin molecular functions is its localization within the cell: in this regard, both nuclear and cytoplasmic staining have been correlated either to favorable or unfavorable outcome depending on the tumor histotype. […] Overall, even though further studies are needed to precisely unveil the role and significance of survivin in MPNST, its large expression in pediatric cases underscores a potential role as target of therapeutic interventions.
  • #27 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management
    https://www.mdpi.com/2072-6694/15/4/1077
    Malignant peripheral nerve sheath tumor (MPNST) is a soft tissue sarcoma with limited therapeutic interventions and a poor prognosis. […] MPNST is still difficult to diagnose and treat, and the overall prognosis is poor. Although MPNST is a rare disease, the mortality rate is high. The median survival time is largely based on subtypes of MPNST and molecular variations. […] MPNST has a poor prognosis on average. Previous studies have shown that the five-year overall survival rate is 50–60%, and the median survival of MPNST is six years. […] The identification of prognostic predictors is necessary for accurate diagnosis and treatment selection. […] Recent studies have revealed a strong correlation between high FNCLCC grade MPNST and survival outcomes. […] NF1 mutation is associated with worse survival than sporadic MPNST.
  • #28 Clinicopathological features and prognosis of malignant peripheral nerve sheath tumor: a retrospective study of 159 cases from 1999 to 2016 | Oncotarget
    https://www.oncotarget.com/article/18975/text/
    A total of 159 patients with MPNST were enrolled in the study. The 3- and 5-year overall survival rates were 50.0% and 43.0%, respectively. Multivariate analysis showed that AJCC stage and S-100 were independent factors affecting overall survival (p 0.05 for both). […] MPSNT is a highly aggressive tumor with poor prognosis and this study may be useful for prognostic assessment and management decisions. […] The 3- and 5-year overall survival rates of the whole group were 50.0% and 43.0%, respectively (Table 1). […] The 5-year OS rate of 140 patients who underwent a complete resection was 45.0%. […] Univariate analysis showed that the location and the depth of tumor, AJCC stage, margin status, S-100 and Ki67 staining independently affected OS (p 0.05, Tables 2 and 4). However, by multivariate analysis, only AJCC stage and S-100 were factors associated with prolonged OS (p 0.05, Table 5).
  • #29 Clinicopathological features and prognosis of malignant peripheral nerve sheath tumor: a retrospective study of 159 cases from 1999 to 2016 | Oncotarget
    https://www.oncotarget.com/article/18975/text/
    The 3-and 5-year TFS rates for the 140 patients were 40.0% and 34.0%, respectively. […] Data showed that the location, the depth, the size, the AJCC stage of the tumor, and S-100 were associated with the tumor-free survival. Whereas, late AJCC stage and S-100 negative were independent unfavorable factors affecting OS. […] In summary, despite combined multimodality therapy, MPNST behaves as an aggressive sarcoma with a propensity to recur locally or to metastasize to distant sites.
  • #30 Clinicopathological features and prognosis of malignant peripheral nerve sheath tumor: a retrospective study of 159 cases from 1999 to 2016 | Oncotarget
    https://www.oncotarget.com/article/18975/text/
    A total of 159 patients with MPNST were enrolled in the study. The 3- and 5-year overall survival rates were 50.0% and 43.0%, respectively. Multivariate analysis showed that AJCC stage and S-100 were independent factors affecting overall survival (p 0.05 for both). […] MPSNT is a highly aggressive tumor with poor prognosis and this study may be useful for prognostic assessment and management decisions. […] The 3- and 5-year overall survival rates of the whole group were 50.0% and 43.0%, respectively (Table 1). […] The 5-year OS rate of 140 patients who underwent a complete resection was 45.0%. […] Univariate analysis showed that the location and the depth of tumor, AJCC stage, margin status, S-100 and Ki67 staining independently affected OS (p 0.05, Tables 2 and 4). However, by multivariate analysis, only AJCC stage and S-100 were factors associated with prolonged OS (p 0.05, Table 5).
  • #31 Loss of H3K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics | Modern Pathology
    https://www.nature.com/articles/modpathol2015134
    The majority of malignant peripheral nerve sheath tumors pursue an aggressive clinical course with a 5-year survival rate of 35-50%. […] H3K27me3 loss is more common in neurofibromatosis type 1-associated compared with sporadic malignant peripheral nerve sheath tumors and is particularly frequent in higher grade and radiation-associated tumors. […] Detection of H3K27me3 loss in previously unclassified radiation-associated sarcomas suggests a subset of these tumors may represent malignant peripheral nerve sheath tumors. […] The value of H3K27me3 as a predictive marker for sensitivity to epigenetic-based therapies remains to be determined.
  • #32 Survivin Expression and Prognostic Significance in Pediatric Malignant Peripheral Nerve Sheath Tumors (MPNST) | PLOS One
    https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0080456
    Malignant peripheral nerve sheath tumors (MPNST) are very aggressive malignancies comprising approximately 510% of all soft tissue sarcomas. […] Survivin mRNA was overexpressed in pediatric MPNST and associated to a copy number gain of BIRC5; furthermore, increased levels of transcripts correlated with a higher FNCLCC tumor grade (grade 1 and 2 vs. 3, p=0.0067), and with a lower survival probability (Log-rank test, p=0.0038). Overall, these data support the concept that survivin can be regarded as a useful prognostic marker for pediatric MPNST and a promising target for therapeutic interventions. […] Despite progress in the treatment of pediatric sarcomas, MPNST are poorly responsive to chemotherapy and radiotherapy, and their 5-year survival rate ranges from 82% for patients in Intergroup Rhabdomyosarcoma Study (IRS) stage I, to 26% for those affected by metastatic disease (IRS stage IV).
  • #33 Survivin Expression and Prognostic Significance in Pediatric Malignant Peripheral Nerve Sheath Tumors (MPNST) | PLOS One
    https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0080456
    For MPNST occurring in patients with NF1, the rate of response to chemotherapy is lower (17.6% vs. 55.3% in patients without NF1), and the outcome is worse with a 5-year overall survival of 32%. […] The survival analysis revealed an association between low levels of survivin mRNA and long-term survival (Figure 2A; Log-rank test, p=0.0038). FNCLCC Grade 1 and 2 MPNST had a more prolonged survival when compared to FNCLCC grade 3 (Figure 2B; Log-rank test, p=0.0322). […] High levels of survivin mRNA were significantly associated with FNCLCC tumor grade 3 and a high mitotic rate. […] An important feature for survivin molecular functions is its localization within the cell: in this regard, both nuclear and cytoplasmic staining have been correlated either to favorable or unfavorable outcome depending on the tumor histotype. […] Overall, even though further studies are needed to precisely unveil the role and significance of survivin in MPNST, its large expression in pediatric cases underscores a potential role as target of therapeutic interventions.
  • #34 Adjuvant Treatment For Patients With Malignant Peripheral Nerve S
    https://www.longdom.org/open-access/adjuvant-treatment-for-patients-with-malignant-peripheral-nerve-sheathtumours-22489.html
    In current clinical practice, there are no curative treatment options for the aggressive neuroectodermal cancer malignant peripheral nerve sheath tumor (MPNST) beyond surgical resection. Nearly one half of the patients have neurofibromatosis type 1 (NF1), a cancer predisposition syndrome that increases the risk of MPNST almost 5000 times as compared to the general population, and MPNST patients with NF1 have been reported to have worse prognosis, although the differences in outcome seem to have diminished in recent years. For both NF1 and non-NF1 patients, the 5-year overall survival after MPNST diagnosis is between 30 and 50%. […] The general clinical experience with MPNST is that these patients respond poorly to adjuvant treatment, and there is a great need for new treatment options for this patient group. We have recently described an IHC profile in MPNST identifying prognostic factors that are also targets to the well-known cytostatic drugs, etoposide and 5-FU rarely used in the treatment of MPNST. To further explore these findings, we suggest a clinical trial guiding therapy in MPNST based on the expression of these biomarkers.
  • #35 Survivin Expression and Prognostic Significance in Pediatric Malignant Peripheral Nerve Sheath Tumors (MPNST) | PLOS One
    https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0080456
    For MPNST occurring in patients with NF1, the rate of response to chemotherapy is lower (17.6% vs. 55.3% in patients without NF1), and the outcome is worse with a 5-year overall survival of 32%. […] The survival analysis revealed an association between low levels of survivin mRNA and long-term survival (Figure 2A; Log-rank test, p=0.0038). FNCLCC Grade 1 and 2 MPNST had a more prolonged survival when compared to FNCLCC grade 3 (Figure 2B; Log-rank test, p=0.0322). […] High levels of survivin mRNA were significantly associated with FNCLCC tumor grade 3 and a high mitotic rate. […] An important feature for survivin molecular functions is its localization within the cell: in this regard, both nuclear and cytoplasmic staining have been correlated either to favorable or unfavorable outcome depending on the tumor histotype. […] Overall, even though further studies are needed to precisely unveil the role and significance of survivin in MPNST, its large expression in pediatric cases underscores a potential role as target of therapeutic interventions.
  • #36 Survivin Expression and Prognostic Significance in Pediatric Malignant Peripheral Nerve Sheath Tumors (MPNST) | PLOS One
    https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0080456
    For MPNST occurring in patients with NF1, the rate of response to chemotherapy is lower (17.6% vs. 55.3% in patients without NF1), and the outcome is worse with a 5-year overall survival of 32%. […] The survival analysis revealed an association between low levels of survivin mRNA and long-term survival (Figure 2A; Log-rank test, p=0.0038). FNCLCC Grade 1 and 2 MPNST had a more prolonged survival when compared to FNCLCC grade 3 (Figure 2B; Log-rank test, p=0.0322). […] High levels of survivin mRNA were significantly associated with FNCLCC tumor grade 3 and a high mitotic rate. […] An important feature for survivin molecular functions is its localization within the cell: in this regard, both nuclear and cytoplasmic staining have been correlated either to favorable or unfavorable outcome depending on the tumor histotype. […] Overall, even though further studies are needed to precisely unveil the role and significance of survivin in MPNST, its large expression in pediatric cases underscores a potential role as target of therapeutic interventions.
  • #37 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management
    https://www.mdpi.com/2072-6694/15/4/1077
    Malignant peripheral nerve sheath tumor (MPNST) is a soft tissue sarcoma with limited therapeutic interventions and a poor prognosis. […] MPNST is still difficult to diagnose and treat, and the overall prognosis is poor. Although MPNST is a rare disease, the mortality rate is high. The median survival time is largely based on subtypes of MPNST and molecular variations. […] MPNST has a poor prognosis on average. Previous studies have shown that the five-year overall survival rate is 50–60%, and the median survival of MPNST is six years. […] The identification of prognostic predictors is necessary for accurate diagnosis and treatment selection. […] Recent studies have revealed a strong correlation between high FNCLCC grade MPNST and survival outcomes. […] NF1 mutation is associated with worse survival than sporadic MPNST.
  • #38 Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas | Modern Pathology
    https://www.nature.com/articles/modpathol2014109
    Cellular schwannoma is an uncommon, but well-recognized, benign peripheral nerve sheath tumor, which can be misdiagnosed as malignant peripheral nerve sheath tumor. […] First, in contrast to patients with malignant peripheral nerve sheath tumor, no metastases or disease-specific deaths were found in patients with cellular schwannoma. More specifically, 5-year progression-free survival rates were 100 and 18%, and 5-year disease-specific survival rates were 100 and 32% for cellular schwannoma and malignant peripheral nerve sheath tumor, respectively. […] For individuals harboring a malignant peripheral nerve sheath tumor, despite adjuvant chemotherapy and radiation, the overall prognosis is poor, with 5-year disease-specific survival ranging between 35 and 50%. […] Given dramatically different prognostic and therapeutic implications, distinguishing cellular schwannoma from malignant peripheral nerve sheath tumor is critical.
  • #39 Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas | Modern Pathology
    https://www.nature.com/articles/modpathol2014109
    Cellular schwannoma is an uncommon, but well-recognized, benign peripheral nerve sheath tumor, which can be misdiagnosed as malignant peripheral nerve sheath tumor. […] First, in contrast to patients with malignant peripheral nerve sheath tumor, no metastases or disease-specific deaths were found in patients with cellular schwannoma. More specifically, 5-year progression-free survival rates were 100 and 18%, and 5-year disease-specific survival rates were 100 and 32% for cellular schwannoma and malignant peripheral nerve sheath tumor, respectively. […] For individuals harboring a malignant peripheral nerve sheath tumor, despite adjuvant chemotherapy and radiation, the overall prognosis is poor, with 5-year disease-specific survival ranging between 35 and 50%. […] Given dramatically different prognostic and therapeutic implications, distinguishing cellular schwannoma from malignant peripheral nerve sheath tumor is critical.
  • #40 Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas | Modern Pathology
    https://www.nature.com/articles/modpathol2014109
    Overall, cellular schwannoma patients had significantly better progression-free and disease-specific survival times than malignant peripheral nerve sheath tumor patients (P<0.001, for both). Five-year progression-free survival rates were 17.7% for malignant peripheral nerve sheath tumor and 100% for cellular schwannoma, and 5-year disease-specific survival rates were 31.9% for malignant peripheral nerve sheath tumor and 100% for cellular schwannoma. [...] The significance of malignant peripheral nerve sheath tumor misdiagnosis is underscored by the relative benign nature of cellular schwannoma, which have essentially no metastatic potential and tumor-associated mortality. [...] In striking contrast, 37.3% of patients with malignant peripheral nerve sheath tumors had metastases and the 5-year disease-specific survival rate was 31.9%.
  • #41 Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas | Modern Pathology
    https://www.nature.com/articles/modpathol2014109
    Cellular schwannoma is an uncommon, but well-recognized, benign peripheral nerve sheath tumor, which can be misdiagnosed as malignant peripheral nerve sheath tumor. […] First, in contrast to patients with malignant peripheral nerve sheath tumor, no metastases or disease-specific deaths were found in patients with cellular schwannoma. More specifically, 5-year progression-free survival rates were 100 and 18%, and 5-year disease-specific survival rates were 100 and 32% for cellular schwannoma and malignant peripheral nerve sheath tumor, respectively. […] For individuals harboring a malignant peripheral nerve sheath tumor, despite adjuvant chemotherapy and radiation, the overall prognosis is poor, with 5-year disease-specific survival ranging between 35 and 50%. […] Given dramatically different prognostic and therapeutic implications, distinguishing cellular schwannoma from malignant peripheral nerve sheath tumor is critical.
  • #42 Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas | Modern Pathology
    https://www.nature.com/articles/modpathol2014109
    Overall, cellular schwannoma patients had significantly better progression-free and disease-specific survival times than malignant peripheral nerve sheath tumor patients (P<0.001, for both). Five-year progression-free survival rates were 17.7% for malignant peripheral nerve sheath tumor and 100% for cellular schwannoma, and 5-year disease-specific survival rates were 31.9% for malignant peripheral nerve sheath tumor and 100% for cellular schwannoma. [...] The significance of malignant peripheral nerve sheath tumor misdiagnosis is underscored by the relative benign nature of cellular schwannoma, which have essentially no metastatic potential and tumor-associated mortality. [...] In striking contrast, 37.3% of patients with malignant peripheral nerve sheath tumors had metastases and the 5-year disease-specific survival rate was 31.9%.
  • #43 Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas | Modern Pathology
    https://www.nature.com/articles/modpathol2014109
    Cellular schwannoma is an uncommon, but well-recognized, benign peripheral nerve sheath tumor, which can be misdiagnosed as malignant peripheral nerve sheath tumor. […] First, in contrast to patients with malignant peripheral nerve sheath tumor, no metastases or disease-specific deaths were found in patients with cellular schwannoma. More specifically, 5-year progression-free survival rates were 100 and 18%, and 5-year disease-specific survival rates were 100 and 32% for cellular schwannoma and malignant peripheral nerve sheath tumor, respectively. […] For individuals harboring a malignant peripheral nerve sheath tumor, despite adjuvant chemotherapy and radiation, the overall prognosis is poor, with 5-year disease-specific survival ranging between 35 and 50%. […] Given dramatically different prognostic and therapeutic implications, distinguishing cellular schwannoma from malignant peripheral nerve sheath tumor is critical.
  • #44 Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas | Modern Pathology
    https://www.nature.com/articles/modpathol2014109
    Overall, cellular schwannoma patients had significantly better progression-free and disease-specific survival times than malignant peripheral nerve sheath tumor patients (P<0.001, for both). Five-year progression-free survival rates were 17.7% for malignant peripheral nerve sheath tumor and 100% for cellular schwannoma, and 5-year disease-specific survival rates were 31.9% for malignant peripheral nerve sheath tumor and 100% for cellular schwannoma. [...] The significance of malignant peripheral nerve sheath tumor misdiagnosis is underscored by the relative benign nature of cellular schwannoma, which have essentially no metastatic potential and tumor-associated mortality. [...] In striking contrast, 37.3% of patients with malignant peripheral nerve sheath tumors had metastases and the 5-year disease-specific survival rate was 31.9%.
  • #45 Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8635905/
    Complete surgical resection with negative surgical margins is necessary for a successful MPNST treatment. […] The 5-year OS rate of all patients with MPNST was 69.5%. […] In the multivariate analysis, surgery of the primary tumour was significantly associated with favourable OS. […] The 5-year OS, LRFS, and MFS rates of patients with localised disease at diagnosis who underwent surgery of their primary tumour at our institutions were 81.1%, 78.2%, and 70.3%, respectively. […] Negative surgical margin was significantly associated with better OS and LRFS, and patients with high-grade tumours exhibited more unfavourable MFS. […] Surgery and/or systemic chemotherapy for metastatic lesions could increase the survival of patients with distant metastases who underwent surgical resection of the primary tumour. […] Complete surgical excision of the primary tumour with negative margins remains the only proven curative treatment. […] We recommend a multidisciplinary treatment for patients with MPNST with aggressive features to maximise a good prognosis.
  • #46 Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8635905/
    Complete surgical resection with negative surgical margins is necessary for a successful MPNST treatment. […] The 5-year OS rate of all patients with MPNST was 69.5%. […] In the multivariate analysis, surgery of the primary tumour was significantly associated with favourable OS. […] The 5-year OS, LRFS, and MFS rates of patients with localised disease at diagnosis who underwent surgery of their primary tumour at our institutions were 81.1%, 78.2%, and 70.3%, respectively. […] Negative surgical margin was significantly associated with better OS and LRFS, and patients with high-grade tumours exhibited more unfavourable MFS. […] Surgery and/or systemic chemotherapy for metastatic lesions could increase the survival of patients with distant metastases who underwent surgical resection of the primary tumour. […] Complete surgical excision of the primary tumour with negative margins remains the only proven curative treatment. […] We recommend a multidisciplinary treatment for patients with MPNST with aggressive features to maximise a good prognosis.
  • #47 Clinicopathological features and prognosis of malignant peripheral nerve sheath tumor: a retrospective study of 159 cases from 1999 to 2016 | Oncotarget
    https://www.oncotarget.com/article/18975/text/
    A total of 159 patients with MPNST were enrolled in the study. The 3- and 5-year overall survival rates were 50.0% and 43.0%, respectively. Multivariate analysis showed that AJCC stage and S-100 were independent factors affecting overall survival (p 0.05 for both). […] MPSNT is a highly aggressive tumor with poor prognosis and this study may be useful for prognostic assessment and management decisions. […] The 3- and 5-year overall survival rates of the whole group were 50.0% and 43.0%, respectively (Table 1). […] The 5-year OS rate of 140 patients who underwent a complete resection was 45.0%. […] Univariate analysis showed that the location and the depth of tumor, AJCC stage, margin status, S-100 and Ki67 staining independently affected OS (p 0.05, Tables 2 and 4). However, by multivariate analysis, only AJCC stage and S-100 were factors associated with prolonged OS (p 0.05, Table 5).
  • #48 Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8635905/
    Complete surgical resection with negative surgical margins is necessary for a successful MPNST treatment. […] The 5-year OS rate of all patients with MPNST was 69.5%. […] In the multivariate analysis, surgery of the primary tumour was significantly associated with favourable OS. […] The 5-year OS, LRFS, and MFS rates of patients with localised disease at diagnosis who underwent surgery of their primary tumour at our institutions were 81.1%, 78.2%, and 70.3%, respectively. […] Negative surgical margin was significantly associated with better OS and LRFS, and patients with high-grade tumours exhibited more unfavourable MFS. […] Surgery and/or systemic chemotherapy for metastatic lesions could increase the survival of patients with distant metastases who underwent surgical resection of the primary tumour. […] Complete surgical excision of the primary tumour with negative margins remains the only proven curative treatment. […] We recommend a multidisciplinary treatment for patients with MPNST with aggressive features to maximise a good prognosis.
  • #49 Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis | World Journal of Surgical Oncology | Full Text
    https://wjso.biomedcentral.com/articles/10.1186/s12957-020-02036-x
    Our study revealed that resection with negative margin was a protective factor for better prognosis, which is in accordance with previous literatures. Besides, the pooled results also indicated chemotherapy and radiotherapy were important to improve survival. Therefore, adjuvant therapies are recommended for patients with MPNST, especially for patients with positive margins. […] In conclusion, our results indicate that the survival and local recurrence of MPNST are poor.
  • #50 Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis | World Journal of Surgical Oncology | Full Text
    https://wjso.biomedcentral.com/articles/10.1186/s12957-020-02036-x
    Our study revealed that resection with negative margin was a protective factor for better prognosis, which is in accordance with previous literatures. Besides, the pooled results also indicated chemotherapy and radiotherapy were important to improve survival. Therefore, adjuvant therapies are recommended for patients with MPNST, especially for patients with positive margins. […] In conclusion, our results indicate that the survival and local recurrence of MPNST are poor.
  • #51 Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8635905/
    Complete surgical resection with negative surgical margins is necessary for a successful MPNST treatment. […] The 5-year OS rate of all patients with MPNST was 69.5%. […] In the multivariate analysis, surgery of the primary tumour was significantly associated with favourable OS. […] The 5-year OS, LRFS, and MFS rates of patients with localised disease at diagnosis who underwent surgery of their primary tumour at our institutions were 81.1%, 78.2%, and 70.3%, respectively. […] Negative surgical margin was significantly associated with better OS and LRFS, and patients with high-grade tumours exhibited more unfavourable MFS. […] Surgery and/or systemic chemotherapy for metastatic lesions could increase the survival of patients with distant metastases who underwent surgical resection of the primary tumour. […] Complete surgical excision of the primary tumour with negative margins remains the only proven curative treatment. […] We recommend a multidisciplinary treatment for patients with MPNST with aggressive features to maximise a good prognosis.
  • #52 Adjuvant Treatment For Patients With Malignant Peripheral Nerve S
    https://www.longdom.org/open-access/adjuvant-treatment-for-patients-with-malignant-peripheral-nerve-sheathtumours-22489.html
    In current clinical practice, there are no curative treatment options for the aggressive neuroectodermal cancer malignant peripheral nerve sheath tumor (MPNST) beyond surgical resection. Nearly one half of the patients have neurofibromatosis type 1 (NF1), a cancer predisposition syndrome that increases the risk of MPNST almost 5000 times as compared to the general population, and MPNST patients with NF1 have been reported to have worse prognosis, although the differences in outcome seem to have diminished in recent years. For both NF1 and non-NF1 patients, the 5-year overall survival after MPNST diagnosis is between 30 and 50%. […] The general clinical experience with MPNST is that these patients respond poorly to adjuvant treatment, and there is a great need for new treatment options for this patient group. We have recently described an IHC profile in MPNST identifying prognostic factors that are also targets to the well-known cytostatic drugs, etoposide and 5-FU rarely used in the treatment of MPNST. To further explore these findings, we suggest a clinical trial guiding therapy in MPNST based on the expression of these biomarkers.
  • #53 Survivin Expression and Prognostic Significance in Pediatric Malignant Peripheral Nerve Sheath Tumors (MPNST) | PLOS One
    https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0080456
    For MPNST occurring in patients with NF1, the rate of response to chemotherapy is lower (17.6% vs. 55.3% in patients without NF1), and the outcome is worse with a 5-year overall survival of 32%. […] The survival analysis revealed an association between low levels of survivin mRNA and long-term survival (Figure 2A; Log-rank test, p=0.0038). FNCLCC Grade 1 and 2 MPNST had a more prolonged survival when compared to FNCLCC grade 3 (Figure 2B; Log-rank test, p=0.0322). […] High levels of survivin mRNA were significantly associated with FNCLCC tumor grade 3 and a high mitotic rate. […] An important feature for survivin molecular functions is its localization within the cell: in this regard, both nuclear and cytoplasmic staining have been correlated either to favorable or unfavorable outcome depending on the tumor histotype. […] Overall, even though further studies are needed to precisely unveil the role and significance of survivin in MPNST, its large expression in pediatric cases underscores a potential role as target of therapeutic interventions.
  • #54 Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis | World Journal of Surgical Oncology | Full Text
    https://wjso.biomedcentral.com/articles/10.1186/s12957-020-02036-x
    No available meta-analysis was printed to systematically introduce the MPNST clinic outcome and risk factors based on largely pooled data. […] The pooled 5-year OS rate, 5-year EFS rate, and LR rate were 49%, 37%, and 38%, respectively. […] Survival and local recurrence of MPNST are poor. Worse prognosis is mainly associated with NF 1, large size, deep to fascia, high grade, metastases, and location (trunk and head and neck). Complete resection with adequate surgical margins is the mainstay protective factor of MPNST patients, following necessary adjuvant therapies. […] The 5-year OS rate was 49% (range, 1663%), which consisted with the most published studies. […] The pooled LR rate was 37% (range, 1386%). This result also revealed that aggressively effective therapy was necessary to be applied in order to control local recurrence.
  • #55 Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8635905/
    Few studies have described the characteristics and prognostic factors of patients with malignant peripheral nerve sheath tumour (MPNST). […] The 5-year overall survival (OS) rate of all patients was 69.5%. […] Univariate analysis revealed that large-sized tumour, metastasis at diagnosis, and no surgery of the primary tumour were significantly associated with patients with worse OS. […] Multivariate analysis identified surgery of the primary tumour as an independent prognostic factor for improved OS. […] Among patients with localised disease at diagnosis who underwent surgery of the primary tumour at our institutions, the 5-year OS, local recurrence-free survival (LRFS), and metastasis-free survival (MFS) rates were 81.1%, 78.2%, and 70.3%, respectively. […] Univariate analysis revealed that positive surgical margin was significantly correlated with unfavourable OS and LRFS, and high grade was a poor prognostic indicator for MFS.
  • #56 Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8635905/
    Few studies have described the characteristics and prognostic factors of patients with malignant peripheral nerve sheath tumour (MPNST). […] The 5-year overall survival (OS) rate of all patients was 69.5%. […] Univariate analysis revealed that large-sized tumour, metastasis at diagnosis, and no surgery of the primary tumour were significantly associated with patients with worse OS. […] Multivariate analysis identified surgery of the primary tumour as an independent prognostic factor for improved OS. […] Among patients with localised disease at diagnosis who underwent surgery of the primary tumour at our institutions, the 5-year OS, local recurrence-free survival (LRFS), and metastasis-free survival (MFS) rates were 81.1%, 78.2%, and 70.3%, respectively. […] Univariate analysis revealed that positive surgical margin was significantly correlated with unfavourable OS and LRFS, and high grade was a poor prognostic indicator for MFS.
  • #57 Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8635905/
    Few studies have described the characteristics and prognostic factors of patients with malignant peripheral nerve sheath tumour (MPNST). […] The 5-year overall survival (OS) rate of all patients was 69.5%. […] Univariate analysis revealed that large-sized tumour, metastasis at diagnosis, and no surgery of the primary tumour were significantly associated with patients with worse OS. […] Multivariate analysis identified surgery of the primary tumour as an independent prognostic factor for improved OS. […] Among patients with localised disease at diagnosis who underwent surgery of the primary tumour at our institutions, the 5-year OS, local recurrence-free survival (LRFS), and metastasis-free survival (MFS) rates were 81.1%, 78.2%, and 70.3%, respectively. […] Univariate analysis revealed that positive surgical margin was significantly correlated with unfavourable OS and LRFS, and high grade was a poor prognostic indicator for MFS.
  • #58 Clinicopathological features and prognosis of malignant peripheral nerve sheath tumor: a retrospective study of 159 cases from 1999 to 2016 | Oncotarget
    https://www.oncotarget.com/article/18975/text/
    The 3-and 5-year TFS rates for the 140 patients were 40.0% and 34.0%, respectively. […] Data showed that the location, the depth, the size, the AJCC stage of the tumor, and S-100 were associated with the tumor-free survival. Whereas, late AJCC stage and S-100 negative were independent unfavorable factors affecting OS. […] In summary, despite combined multimodality therapy, MPNST behaves as an aggressive sarcoma with a propensity to recur locally or to metastasize to distant sites.
  • #59 Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis | World Journal of Surgical Oncology | Full Text
    https://wjso.biomedcentral.com/articles/10.1186/s12957-020-02036-x
    No available meta-analysis was printed to systematically introduce the MPNST clinic outcome and risk factors based on largely pooled data. […] The pooled 5-year OS rate, 5-year EFS rate, and LR rate were 49%, 37%, and 38%, respectively. […] Survival and local recurrence of MPNST are poor. Worse prognosis is mainly associated with NF 1, large size, deep to fascia, high grade, metastases, and location (trunk and head and neck). Complete resection with adequate surgical margins is the mainstay protective factor of MPNST patients, following necessary adjuvant therapies. […] The 5-year OS rate was 49% (range, 1663%), which consisted with the most published studies. […] The pooled LR rate was 37% (range, 1386%). This result also revealed that aggressively effective therapy was necessary to be applied in order to control local recurrence.
  • #60 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management
    https://www.mdpi.com/2072-6694/15/4/1077
    Malignant peripheral nerve sheath tumor (MPNST) is a soft tissue sarcoma with limited therapeutic interventions and a poor prognosis. […] MPNST is still difficult to diagnose and treat, and the overall prognosis is poor. Although MPNST is a rare disease, the mortality rate is high. The median survival time is largely based on subtypes of MPNST and molecular variations. […] MPNST has a poor prognosis on average. Previous studies have shown that the five-year overall survival rate is 50–60%, and the median survival of MPNST is six years. […] The identification of prognostic predictors is necessary for accurate diagnosis and treatment selection. […] Recent studies have revealed a strong correlation between high FNCLCC grade MPNST and survival outcomes. […] NF1 mutation is associated with worse survival than sporadic MPNST.
  • #61 Survivin Expression and Prognostic Significance in Pediatric Malignant Peripheral Nerve Sheath Tumors (MPNST) | PLOS One
    https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0080456
    For MPNST occurring in patients with NF1, the rate of response to chemotherapy is lower (17.6% vs. 55.3% in patients without NF1), and the outcome is worse with a 5-year overall survival of 32%. […] The survival analysis revealed an association between low levels of survivin mRNA and long-term survival (Figure 2A; Log-rank test, p=0.0038). FNCLCC Grade 1 and 2 MPNST had a more prolonged survival when compared to FNCLCC grade 3 (Figure 2B; Log-rank test, p=0.0322). […] High levels of survivin mRNA were significantly associated with FNCLCC tumor grade 3 and a high mitotic rate. […] An important feature for survivin molecular functions is its localization within the cell: in this regard, both nuclear and cytoplasmic staining have been correlated either to favorable or unfavorable outcome depending on the tumor histotype. […] Overall, even though further studies are needed to precisely unveil the role and significance of survivin in MPNST, its large expression in pediatric cases underscores a potential role as target of therapeutic interventions.
  • #62 Loss of H3K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics | Modern Pathology
    https://www.nature.com/articles/modpathol2015134
    The majority of malignant peripheral nerve sheath tumors pursue an aggressive clinical course with a 5-year survival rate of 35-50%. […] H3K27me3 loss is more common in neurofibromatosis type 1-associated compared with sporadic malignant peripheral nerve sheath tumors and is particularly frequent in higher grade and radiation-associated tumors. […] Detection of H3K27me3 loss in previously unclassified radiation-associated sarcomas suggests a subset of these tumors may represent malignant peripheral nerve sheath tumors. […] The value of H3K27me3 as a predictive marker for sensitivity to epigenetic-based therapies remains to be determined.
  • #63 Adjuvant Treatment For Patients With Malignant Peripheral Nerve S
    https://www.longdom.org/open-access/adjuvant-treatment-for-patients-with-malignant-peripheral-nerve-sheathtumours-22489.html
    In current clinical practice, there are no curative treatment options for the aggressive neuroectodermal cancer malignant peripheral nerve sheath tumor (MPNST) beyond surgical resection. Nearly one half of the patients have neurofibromatosis type 1 (NF1), a cancer predisposition syndrome that increases the risk of MPNST almost 5000 times as compared to the general population, and MPNST patients with NF1 have been reported to have worse prognosis, although the differences in outcome seem to have diminished in recent years. For both NF1 and non-NF1 patients, the 5-year overall survival after MPNST diagnosis is between 30 and 50%. […] The general clinical experience with MPNST is that these patients respond poorly to adjuvant treatment, and there is a great need for new treatment options for this patient group. We have recently described an IHC profile in MPNST identifying prognostic factors that are also targets to the well-known cytostatic drugs, etoposide and 5-FU rarely used in the treatment of MPNST. To further explore these findings, we suggest a clinical trial guiding therapy in MPNST based on the expression of these biomarkers.
  • #64 Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8635905/
    Complete surgical resection with negative surgical margins is necessary for a successful MPNST treatment. […] The 5-year OS rate of all patients with MPNST was 69.5%. […] In the multivariate analysis, surgery of the primary tumour was significantly associated with favourable OS. […] The 5-year OS, LRFS, and MFS rates of patients with localised disease at diagnosis who underwent surgery of their primary tumour at our institutions were 81.1%, 78.2%, and 70.3%, respectively. […] Negative surgical margin was significantly associated with better OS and LRFS, and patients with high-grade tumours exhibited more unfavourable MFS. […] Surgery and/or systemic chemotherapy for metastatic lesions could increase the survival of patients with distant metastases who underwent surgical resection of the primary tumour. […] Complete surgical excision of the primary tumour with negative margins remains the only proven curative treatment. […] We recommend a multidisciplinary treatment for patients with MPNST with aggressive features to maximise a good prognosis.
  • #65 Adjuvant Treatment For Patients With Malignant Peripheral Nerve S
    https://www.longdom.org/open-access/adjuvant-treatment-for-patients-with-malignant-peripheral-nerve-sheathtumours-22489.html
    In current clinical practice, there are no curative treatment options for the aggressive neuroectodermal cancer malignant peripheral nerve sheath tumor (MPNST) beyond surgical resection. Nearly one half of the patients have neurofibromatosis type 1 (NF1), a cancer predisposition syndrome that increases the risk of MPNST almost 5000 times as compared to the general population, and MPNST patients with NF1 have been reported to have worse prognosis, although the differences in outcome seem to have diminished in recent years. For both NF1 and non-NF1 patients, the 5-year overall survival after MPNST diagnosis is between 30 and 50%. […] The general clinical experience with MPNST is that these patients respond poorly to adjuvant treatment, and there is a great need for new treatment options for this patient group. We have recently described an IHC profile in MPNST identifying prognostic factors that are also targets to the well-known cytostatic drugs, etoposide and 5-FU rarely used in the treatment of MPNST. To further explore these findings, we suggest a clinical trial guiding therapy in MPNST based on the expression of these biomarkers.
  • #66 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management
    https://www.mdpi.com/2072-6694/15/4/1077
    Malignant peripheral nerve sheath tumor (MPNST) is a soft tissue sarcoma with limited therapeutic interventions and a poor prognosis. […] MPNST is still difficult to diagnose and treat, and the overall prognosis is poor. Although MPNST is a rare disease, the mortality rate is high. The median survival time is largely based on subtypes of MPNST and molecular variations. […] MPNST has a poor prognosis on average. Previous studies have shown that the five-year overall survival rate is 50–60%, and the median survival of MPNST is six years. […] The identification of prognostic predictors is necessary for accurate diagnosis and treatment selection. […] Recent studies have revealed a strong correlation between high FNCLCC grade MPNST and survival outcomes. […] NF1 mutation is associated with worse survival than sporadic MPNST.

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nowotwór neuroektodermalny terapia uzupełniająca przeżycie bez przerzutów mięsak tkanek miękkich wznowa miejscowa schwannoma komórkowy stratyfikacja ryzyka przeżycie całkowite MPNST terapia wielomodalna marker Ki67 przerzut odległy białko S-100 leczenie uzupełniające złośliwość histologiczna radioterapia diagnostyka różnicowa margines chirurgiczny H3K27me3 resekcja chirurgiczna klasyfikacja FNCLCC neurofibromatoza typu 1 surwiwina przeżycie bez wznowy miejscowej klasyfikacja AJCC zmiana przerzutowa biomarker modyfikacja epigenetyczna