Złośliwe nowotwory osłonek nerwowych obwodowych – Rokowania, prognozy i postęp choroby

Złośliwe nowotwory osłonek nerwowych obwodowych
Rokowania, prognozy i postęp choroby

Złośliwe nowotwory osłonek nerwowych obwodowych (MPNST) stanowią około 5-10% mięsaków tkanek miękkich u dorosłych i charakteryzują się agresywnym przebiegiem oraz niekorzystnym rokowaniem. 5-letnie przeżycie całkowite (OS) waha się między 30% a 69,5%, a mediana przeżycia wynosi około 6 lat. Obecność zespołu nerwiakowłókniakowatości typu 1 (NF1) znacząco pogarsza rokowanie (HR 1,63 dla zgonu z wszystkich przyczyn), obniżając 5-letnie OS do około 32%. Inne negatywne czynniki prognostyczne to wielkość guza >5 cm, obecność przerzutów (5-letnie przeżycie spada do 18% przy przerzutach vs 74% bez), wiek >60 lat, wysoki stopień złośliwości histologicznej według FNCLCC oraz lokalizacja głęboka i w obrębie tułowia, głowy i szyi. Ekspresja markerów molekularnych, takich jak surwiwina, S-100, Ki67 oraz utrata trimetylacji H3K27, również koreluje z gorszym rokowaniem.

Prognostyka złośliwych nowotworów osłonek nerwowych obwodowych (MPNST)

Złośliwe nowotwory osłonek nerwowych obwodowych (MPNST) są wysoce agresywnymi nowotworami tkanek miękkich, stanowiącymi około 5-10% wszystkich mięsaków tkanek miękkich u dorosłych.¹² Rokowanie w przypadku MPNST jest ogólnie niekorzystne, a wskaźniki przeżycia pozostają na niskim poziomie w ciągu ostatnich dekad.³

Wskaźniki przeżycia w MPNST

Dane dotyczące przeżycia pacjentów z MPNST wskazują na jego niekorzystne rokowanie:

5-letnie przeżycie całkowite (OS) waha się między 30% a 69,5%, w zależności od badanej populacji⁴⁵⁶
3-letnie przeżycie całkowite wynosi około 50%⁷
5-letnie przeżycie wolne od choroby (EFS) wynosi około 37%⁸
5-letnie przeżycie wolne od wznowy miejscowej (LRFS) u pacjentów z chorobą zlokalizowaną, którzy przeszli zabieg chirurgiczny, wynosi około 78,2%⁹
5-letnie przeżycie wolne od przerzutów (MFS) wynosi około 70,3%¹⁰
Mediana przeżycia w MPNST wynosi około 6 lat¹¹

W przypadku pacjentów z przerzutami odległymi rokowanie jest zdecydowanie gorsze – 5-letnie przeżycie spada do około 18% w porównaniu z 74% u pacjentów bez przerzutów.¹²

Kluczowe czynniki prognostyczne

Zidentyfikowano liczne czynniki wpływające na rokowanie w MPNST. Poniższe czynniki w istotny sposób korelują z gorszym rokowaniem:

Czynniki kliniczne

Obecność zespołu nerwiakowłókniakowatości typu 1 (NF1) jest istotnym negatywnym czynnikiem prognostycznym. Pacjenci z MPNST związanym z NF1 wykazują znacząco wyższe ryzyko zgonu z wszystkich przyczyn (HR 1,63) oraz zgonu związanego z chorobą (HR 1,52) w porównaniu z przypadkami sporadycznymi.¹³ Chorzy z NF1 charakteryzują się gorszą odpowiedzią na chemioterapię (17,6% vs 55,3%) oraz niższym 5-letnim przeżyciem całkowitym wynoszącym około 32%.¹⁴¹⁵

Wielkość guza jest istotnym czynnikiem prognostycznym – guzy większe niż 5 cm znacząco zmniejszają przeżycie specyficzne dla choroby (DSS) oraz przeżycie całkowite (OS).¹⁶¹⁷

Obecność przerzutów przy rozpoznaniu lub w trakcie obserwacji istotnie pogarsza rokowanie. Pacjenci bez przerzutów mają lepsze 5-letnie przeżycie specyficzne dla choroby (38,3%) w porównaniu z pacjentami z przerzutami (5,1%).¹⁸ Metaanaliza potwierdziła, że obecność przerzutów jest niezależnym negatywnym czynnikiem rokowniczym.¹⁹

Wiek pacjenta powyżej 60 lat jest również uważany za niezależny negatywny czynnik prognostyczny, choć rzadziej był opisywany w literaturze.²⁰

Czynniki patologiczne

Stopień złośliwości histologicznej silnie koreluje z wynikami przeżycia. Wysoki stopień złośliwości według klasyfikacji FNCLCC jest związany z gorszym rokowaniem, szczególnie w kontekście przeżycia wolnego od przerzutów (MFS).²¹²²

Ekspresja markerów molekularnych również ma znaczenie prognostyczne. Badania wykazały, że:

Nadekspresja mRNA surwiwiny wiąże się z wyższym stopniem złośliwości FNCLCC i niższym prawdopodobieństwem przeżycia²³
Status ekspresji białka S-100 jest niezależnym czynnikiem wpływającym na przeżycie całkowite²⁴²⁵
Ekspresja Ki67 jest niezależnym czynnikiem wpływającym na przeżycie wolne od guza²⁶
Utrata trimetylacji H3K27 jest częstsza w nowotworach związanych z NF1 oraz w guzach o wyższym stopniu złośliwości²⁷

Lokalizacja i głębokość guza również wpływają na rokowanie. Guzy położone głęboko pod powięzią oraz zlokalizowane w obrębie tułowia, głowy i szyi wiążą się z gorszym rokowaniem.²⁸²⁹

Czynniki związane z leczeniem

Leczenie chirurgiczne pozostaje najważniejszym elementem terapii wpływającym na rokowanie. W analizie wieloczynnikowej przeprowadzenie zabiegu operacyjnego guza pierwotnego było istotnie związane z korzystnym OS.³⁰ Uzyskanie ujemnych marginesów chirurgicznych (resekcja R0) znacząco poprawia rokowanie w zakresie OS i LRFS.³¹³²

Rola radioterapii i chemioterapii w poprawie przeżycia w MPNST pozostaje przedmiotem dyskusji.³³ Ogólnie uważa się, że pacjenci z MPNST słabo odpowiadają na leczenie adjuwantowe, co podkreśla potrzebę opracowania nowych opcji terapeutycznych.³⁴

Porównanie z łagodnymi zmianami osłonek nerwowych

W kontekście diagnostyki różnicowej warto podkreślić istotne różnice w rokowaniu między MPNST a łagodnymi guzami osłonek nerwowych, takimi jak schwannoma komórkowy:

5-letnie przeżycie wolne od progresji wynosi 100% dla schwannoma komórkowego w porównaniu z 18% dla MPNST³⁵
5-letnie przeżycie specyficzne dla choroby wynosi 100% dla schwannoma komórkowego w porównaniu z 32% dla MPNST³⁶
W dużej kohorcie 41% pacjentów z pierwotnie zlokalizowanym MPNST rozwinęło przerzuty odległe w medianie czasu 12 miesięcy³⁷

Strategie leczenia i ich wpływ na rokowanie

Pełna resekcja chirurgiczna z ujemnymi marginesami pozostaje jedynym udowodnionym leczeniem prowadzącym do wyleczenia.³⁸ Pomimo stosowania mniej radykalnych procedur z naciskiem na oszczędzanie kończyn i całkowitą (R0) resekcję guza, można osiągnąć porównywalne wskaźniki przeżycia.³⁹

Amputacja powinna być ostatecznością, ponieważ szeroka resekcja chirurgiczna nie wykazuje korzyści w ogólnym przeżyciu w mięsakach tkanek miękkich, a prowadzi do niezwykle okaleczających operacji.⁴⁰

W przypadku pacjentów z przerzutami odległymi, zabieg chirurgiczny i/lub chemioterapia systemowa dla zmian przerzutowych mogą zwiększyć przeżycie pacjentów, którzy przeszli resekcję chirurgiczną guza pierwotnego.⁴¹

Zalecenia kliniczne

Biorąc pod uwagę zgromadzone dane dotyczące rokowania w MPNST, należy rozważyć następujące podejścia:

Uwzględnienie statusu NF1 przy określaniu stopnia zaawansowania choroby i zalecanie ściślejszego monitorowania pacjentów z NF1 zagrożonych rozwojem MPNST⁴²
Wielodyscyplinarne leczenie pacjentów z MPNST z agresywnymi cechami w celu zmaksymalizowania dobrego rokowania⁴³
Dążenie do całkowitej resekcji chirurgicznej z odpowiednimi marginesami, co stanowi najważniejszy ochronny czynnik prognostyczny dla pacjentów z MPNST⁴⁴
Rozważenie terapii adjuwantowych w razie potrzeby, szczególnie w przypadkach wysokiego ryzyka⁴⁵

Obecne wyzwania w prognozowaniu MPNST

Mimo postępów w rozumieniu biologii MPNST, nadal istnieją ograniczenia w prognozowaniu przebiegu klinicznego tego nowotworu:⁴⁶

Cechy histologiczne i stopień zaawansowania guza nie zawsze precyzyjnie przewidują zachowanie kliniczne i potencjał przerzutowy MPNST
Istnieje potrzeba bardziej dokładnych biomarkerów prognostycznych, które mogłyby stratyfikować pacjentów według ryzyka i kierować decyzjami terapeutycznymi
Wartość prognostyczna niektórych czynników (np. utrata trimetylacji H3K27) jako predyktorów odpowiedzi na terapie oparte na mechanizmach epigenetycznych wymaga dalszego określenia⁴⁷

W związku z ogólnie złym rokowaniem MPNST, szczególnie w przypadkach z przerzutami lub związanych z NF1, istnieje pilna potrzeba opracowania nowych opcji terapeutycznych, które mogłyby poprawić wyniki leczenia tej agresywnej choroby.⁴⁸ Niezbędne są duże badania retrospektywne w celu poprawy słabej bazy dowodów dla schematów leczenia MPNST.⁴⁹

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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). […] 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% […] Unfortunately, histological features and tumor grade do not predict the clinical behavior and metastatic potential for MPNST. […] High levels of survivin mRNA were significantly associated with FNCLCC tumor grade 3 and a high mitotic rate.
#2 Diagnosis, Treatment and Survival of 65 Patients with Malignant Peripheral Nerve Sheath Tumors | Anticancer Research
https://ar.iiarjournals.org/content/34/2/777
Background: Malignant peripheral nerve sheath tumors (MPNST) account for up to 10% of all malignant soft tissue tumors in adults. Insufficient data are available on diagnosis, differential diagnosis and treatment modalities as well as prognosis. […] The 5-year disease-free survival rate was 49%. Overall, 27% of patients first operated on at our Institution experienced local recurrence. The only significant negative prognostic factor for survival was occurrence of metastases. […] The 5-year overall survival was 49%. […] Only metastasis was found to be a significant independent prognostic factor for survival. […] Existing metastasis had a considerable influence on the survival of patients. Without verified metastases, the overall survival was 126 months; with metastasis, it was only 36 months; the 5-year survival was 74% versus 18%.
#3 Survival outcomes of malignant peripheral nerve sheath tumors (MPNSTs) with and without neurofibromatosis type I (NF1): a meta-analysis | World Journal of Surgical Oncology | Full Text
https://wjso.biomedcentral.com/articles/10.1186/s12957-023-03296-z
NF1 is associated with a substantially higher risk of all-cause and disease-specific mortality. This finding suggests that closer surveillance is required for NF1 patients at risk of developing MPNSTs. […] The prognosis for MPNST is poor and has remained abysmal in the last few decades with 5-year survival rates ranging between 16 and 62%. Some studies have reported poorer survival rates in the NF1 group, while others have suggested that there is little difference. […] Given that NF1 has been established as a significant predictor of mortality, we propose considering NF1 status when staging MPNSTs and advocate closer monitoring of these patients. […] The results of this study show that NF1 is associated with a substantially higher risk of all-cause and disease-specific mortality for MPNSTs. We found no significant change in the association of NF1 as a poor prognostic indicator of mortality with time of publication or with the anatomical location of the tumor. As such, clinicians should consider NF1 status in staging the disease and closer monitoring of NF1 patients at risk of developing MPNSTs to enhance their survival rates through timely intervention.
#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 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. […] 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.
#6 Loss of H3K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics | Modern Pathology
https://www.nature.com/articles/modpathol2015134
The diagnosis of malignant peripheral nerve sheath tumor is challenging, particularly in the sporadic setting. […] Most malignant peripheral nerve sheath tumors pursue an aggressive clinical course with a 5-year survival rate of 35-50%. […] The lifetime risk of developing malignant peripheral nerve sheath tumor is estimated to be 5-10% for patients with neurofibromatosis type 1. […] Our findings suggest that PRC2 inactivation in malignant peripheral nerve sheath tumor may occur during progression to higher grades. […] 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. […] The value of H3K27me3 as a predictive marker for sensitivity to epigenetic-based therapies remains to be determined.
#7 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). 3- and 5-year tumor-free survival rates for 140 completely resected patients were 40.0% and 34.0%, respectively. Multivariate analysis showed that AJCC stage, S-100 and Ki67 staining were independent factors of tumor-free survival (p 0.05 for all). […] 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 OS rates were 50.0% and 43.0%, respectively for the whole group, and 54.0% and 45.0%, respectively for patients who received tumor resection.
#8 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. This systematic review and meta-analysis aimed to investigate 5-year OS rate, 5-year EFS rate, and LR rate for MPNST, and to assess potential risk factors for prognosis. […] The pooled 5-year OS rate, 5-year EFS rate, and LR rate were 49%, 37%, and 38%, respectively. The significant prognostic factors for survival were NF1 status, tumor size, depth, location, malignant grade, margin status, chemotherapy, and radiotherapy. Age and sex were not associated with survival. […] 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.
#9 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.
#10 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.
#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. This review summarized the current understanding of the pathogenic mechanisms behind MPNST and the latest concepts in clinical management from diagnosis to therapeutic intervention. Additionally, the developments in molecular diagnosis and targeted therapies for MPNST are highlighted. It concluded with the challenges and prospects of MPNST management. […] MPNST has a poor prognosis on average. Previous studies have shown that the five-year overall survival rate is 50â60%, 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. In contrast, the low-grade MPNST was rarely studied.
#12 Diagnosis, Treatment and Survival of 65 Patients with Malignant Peripheral Nerve Sheath Tumors | Anticancer Research
https://ar.iiarjournals.org/content/34/2/777
Background: Malignant peripheral nerve sheath tumors (MPNST) account for up to 10% of all malignant soft tissue tumors in adults. Insufficient data are available on diagnosis, differential diagnosis and treatment modalities as well as prognosis. […] The 5-year disease-free survival rate was 49%. Overall, 27% of patients first operated on at our Institution experienced local recurrence. The only significant negative prognostic factor for survival was occurrence of metastases. […] The 5-year overall survival was 49%. […] Only metastasis was found to be a significant independent prognostic factor for survival. […] Existing metastasis had a considerable influence on the survival of patients. Without verified metastases, the overall survival was 126 months; with metastasis, it was only 36 months; the 5-year survival was 74% versus 18%.
#13 Survival outcomes of malignant peripheral nerve sheath tumors (MPNSTs) with and without neurofibromatosis type I (NF1): a meta-analysis | World Journal of Surgical Oncology | Full Text
https://wjso.biomedcentral.com/articles/10.1186/s12957-023-03296-z
Malignant peripheral nerve sheath tumors (MPNSTs) are malignancies that demonstrate nerve sheath differentiation in the peripheral nervous system. They can occur sporadically or be associated with neurofibromatosis type 1 (NF1), an autosomal dominant neurocutaneous disorder, with up to 13% of patients developing MPNSTs in their lifetimes. Previous studies have suggested conflicting findings regarding the prognosis of NF1 for patients with MPNSTs. The elucidation of NF1 as an independent prognostic factor on mortality has implications for clinical management. We aim to investigate the role of NF1 status as an independent prognostic factor of overall survival (OS) and disease-specific survival (DSS) in MPNSTs. […] A total of 59 retrospective studies involving 3602 patients fulfilled the inclusion criteria for OS analysis, and 23 studies involving 704 MPNST patients were included to evaluate DSS outcomes. There was a significant increase in the hazard of all-cause mortality (HR 1.63, 95% CI 1.45 to 1.84) and disease-specific mortality (HR 1.52, 95% CI 1.24 to 1.88) among NF1 as compared to sporadic cases.
#14 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). […] 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% […] Unfortunately, histological features and tumor grade do not predict the clinical behavior and metastatic potential for MPNST. […] High levels of survivin mRNA were significantly associated with FNCLCC tumor grade 3 and a high mitotic rate.
#15 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management
https://www.mdpi.com/2072-6694/15/4/1077
NF1 mutation is associated with worse survival than sporadic MPNST. Further studies are needed as the optimal treatment regimen for NF1-associated, and sporadic MPNST may differ. 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 value of radiotherapy and chemotherapy for MPNST survival is still being debated.
#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 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management
https://www.mdpi.com/2072-6694/15/4/1077
NF1 mutation is associated with worse survival than sporadic MPNST. Further studies are needed as the optimal treatment regimen for NF1-associated, and sporadic MPNST may differ. 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 value of radiotherapy and chemotherapy for MPNST survival is still being debated.
#18 Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas | Modern Pathology
https://www.nature.com/articles/modpathol2014109
Patients without metastasis had better 5-year disease-specific survival (38.3%) relative to those with metastasis (5.1%, P=0.003). […] Our study confirmed previously proposed morphologic features of cellular schwannoma including well-defined capsules, macrophage-rich areas, cellular Schwannian whorls and hyalinized thick blood vessels. […] 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%.
#19 Diagnosis, Treatment and Survival of 65 Patients with Malignant Peripheral Nerve Sheath Tumors | Anticancer Research
https://ar.iiarjournals.org/content/34/2/777
Background: Malignant peripheral nerve sheath tumors (MPNST) account for up to 10% of all malignant soft tissue tumors in adults. Insufficient data are available on diagnosis, differential diagnosis and treatment modalities as well as prognosis. […] The 5-year disease-free survival rate was 49%. Overall, 27% of patients first operated on at our Institution experienced local recurrence. The only significant negative prognostic factor for survival was occurrence of metastases. […] The 5-year overall survival was 49%. […] Only metastasis was found to be a significant independent prognostic factor for survival. […] Existing metastasis had a considerable influence on the survival of patients. Without verified metastases, the overall survival was 126 months; with metastasis, it was only 36 months; the 5-year survival was 74% versus 18%.
#20 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management
https://www.mdpi.com/2072-6694/15/4/1077
NF1 mutation is associated with worse survival than sporadic MPNST. Further studies are needed as the optimal treatment regimen for NF1-associated, and sporadic MPNST may differ. 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 value of radiotherapy and chemotherapy for MPNST survival is still being debated.
#21 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.
#22 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management
https://www.mdpi.com/2072-6694/15/4/1077
Malignant peripheral nerve sheath tumor (MPNST) is a soft tissue sarcoma with limited therapeutic interventions and a poor prognosis. This review summarized the current understanding of the pathogenic mechanisms behind MPNST and the latest concepts in clinical management from diagnosis to therapeutic intervention. Additionally, the developments in molecular diagnosis and targeted therapies for MPNST are highlighted. It concluded with the challenges and prospects of MPNST management. […] MPNST has a poor prognosis on average. Previous studies have shown that the five-year overall survival rate is 50â60%, 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. In contrast, the low-grade MPNST was rarely studied.
#23 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). […] 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% […] Unfortunately, histological features and tumor grade do not predict the clinical behavior and metastatic potential for MPNST. […] High levels of survivin mRNA were significantly associated with FNCLCC tumor grade 3 and a high mitotic rate.
#24 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). 3- and 5-year tumor-free survival rates for 140 completely resected patients were 40.0% and 34.0%, respectively. Multivariate analysis showed that AJCC stage, S-100 and Ki67 staining were independent factors of tumor-free survival (p 0.05 for all). […] 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 OS rates were 50.0% and 43.0%, respectively for the whole group, and 54.0% and 45.0%, respectively for patients who received tumor resection.
#25 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 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). […] 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 the series, negative margins could improve both the OS (p = 0.002) and the TFS (p = 0.003) on analyses of univariable Cox proportions. But it was not an independent prognostic factor via multivariate analysis (p 0.05). […] Despite combined multimodality therapy, MPNST behaves as an aggressive sarcoma with a propensity to recur locally or to metastasize to distant sites.
#26 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 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). […] 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 the series, negative margins could improve both the OS (p = 0.002) and the TFS (p = 0.003) on analyses of univariable Cox proportions. But it was not an independent prognostic factor via multivariate analysis (p 0.05). […] Despite combined multimodality therapy, MPNST behaves as an aggressive sarcoma with a propensity to recur locally or to metastasize to distant sites.
#27 Loss of H3K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics | Modern Pathology
https://www.nature.com/articles/modpathol2015134
The diagnosis of malignant peripheral nerve sheath tumor is challenging, particularly in the sporadic setting. […] Most malignant peripheral nerve sheath tumors pursue an aggressive clinical course with a 5-year survival rate of 35-50%. […] The lifetime risk of developing malignant peripheral nerve sheath tumor is estimated to be 5-10% for patients with neurofibromatosis type 1. […] Our findings suggest that PRC2 inactivation in malignant peripheral nerve sheath tumor may occur during progression to higher grades. […] 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. […] The value of H3K27me3 as a predictive marker for sensitivity to epigenetic-based therapies remains to be determined.
#28 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. This systematic review and meta-analysis aimed to investigate 5-year OS rate, 5-year EFS rate, and LR rate for MPNST, and to assess potential risk factors for prognosis. […] The pooled 5-year OS rate, 5-year EFS rate, and LR rate were 49%, 37%, and 38%, respectively. The significant prognostic factors for survival were NF1 status, tumor size, depth, location, malignant grade, margin status, chemotherapy, and radiotherapy. Age and sex were not associated with survival. […] 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.
#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 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). […] 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 the series, negative margins could improve both the OS (p = 0.002) and the TFS (p = 0.003) on analyses of univariable Cox proportions. But it was not an independent prognostic factor via multivariate analysis (p 0.05). […] Despite combined multimodality therapy, MPNST behaves as an aggressive sarcoma with a propensity to recur locally or to metastasize to distant sites.
#30 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.
#31 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.
#32 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 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). […] 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 the series, negative margins could improve both the OS (p = 0.002) and the TFS (p = 0.003) on analyses of univariable Cox proportions. But it was not an independent prognostic factor via multivariate analysis (p 0.05). […] Despite combined multimodality therapy, MPNST behaves as an aggressive sarcoma with a propensity to recur locally or to metastasize to distant sites.
#33 Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management
https://www.mdpi.com/2072-6694/15/4/1077
NF1 mutation is associated with worse survival than sporadic MPNST. Further studies are needed as the optimal treatment regimen for NF1-associated, and sporadic MPNST may differ. 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 value of radiotherapy and chemotherapy for MPNST survival is still being debated.
#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. […] 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.
#35 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%. […] In a large cohort, 41% of patients who initially presented with a localized malignant peripheral nerve sheath tumor developed distant metastasis in a median time of 12 months.
#36 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%. […] In a large cohort, 41% of patients who initially presented with a localized malignant peripheral nerve sheath tumor developed distant metastasis in a median time of 12 months.
#37 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%. […] In a large cohort, 41% of patients who initially presented with a localized malignant peripheral nerve sheath tumor developed distant metastasis in a median time of 12 months.
#38 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.
#39 Diagnosis, Treatment and Survival of 65 Patients with Malignant Peripheral Nerve Sheath Tumors | Anticancer Research
https://ar.iiarjournals.org/content/34/2/777
Despite using less radical procedures with focus on limb salvage and complete (R0) tumor resection, we achieved comparable survival rates. […] Amputation should be the last resort because wide surgical resection shows no benefit in overall survival in soft tissue sarcoma and leads to extremely mutilating operations. […] Large retrospective studies are necessary to improve the poor evidence for treatment regimes of MPNSTs.
#40 Diagnosis, Treatment and Survival of 65 Patients with Malignant Peripheral Nerve Sheath Tumors | Anticancer Research
https://ar.iiarjournals.org/content/34/2/777
Despite using less radical procedures with focus on limb salvage and complete (R0) tumor resection, we achieved comparable survival rates. […] Amputation should be the last resort because wide surgical resection shows no benefit in overall survival in soft tissue sarcoma and leads to extremely mutilating operations. […] Large retrospective studies are necessary to improve the poor evidence for treatment regimes of MPNSTs.
#41 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.
#42 Survival outcomes of malignant peripheral nerve sheath tumors (MPNSTs) with and without neurofibromatosis type I (NF1): a meta-analysis | World Journal of Surgical Oncology | Full Text
https://wjso.biomedcentral.com/articles/10.1186/s12957-023-03296-z
NF1 is associated with a substantially higher risk of all-cause and disease-specific mortality. This finding suggests that closer surveillance is required for NF1 patients at risk of developing MPNSTs. […] The prognosis for MPNST is poor and has remained abysmal in the last few decades with 5-year survival rates ranging between 16 and 62%. Some studies have reported poorer survival rates in the NF1 group, while others have suggested that there is little difference. […] Given that NF1 has been established as a significant predictor of mortality, we propose considering NF1 status when staging MPNSTs and advocate closer monitoring of these patients. […] The results of this study show that NF1 is associated with a substantially higher risk of all-cause and disease-specific mortality for MPNSTs. We found no significant change in the association of NF1 as a poor prognostic indicator of mortality with time of publication or with the anatomical location of the tumor. As such, clinicians should consider NF1 status in staging the disease and closer monitoring of NF1 patients at risk of developing MPNSTs to enhance their survival rates through timely intervention.
#43 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.
#44 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. This systematic review and meta-analysis aimed to investigate 5-year OS rate, 5-year EFS rate, and LR rate for MPNST, and to assess potential risk factors for prognosis. […] The pooled 5-year OS rate, 5-year EFS rate, and LR rate were 49%, 37%, and 38%, respectively. The significant prognostic factors for survival were NF1 status, tumor size, depth, location, malignant grade, margin status, chemotherapy, and radiotherapy. Age and sex were not associated with survival. […] 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.
#45 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. This systematic review and meta-analysis aimed to investigate 5-year OS rate, 5-year EFS rate, and LR rate for MPNST, and to assess potential risk factors for prognosis. […] The pooled 5-year OS rate, 5-year EFS rate, and LR rate were 49%, 37%, and 38%, respectively. The significant prognostic factors for survival were NF1 status, tumor size, depth, location, malignant grade, margin status, chemotherapy, and radiotherapy. Age and sex were not associated with survival. […] 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.
#46 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). […] 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% […] Unfortunately, histological features and tumor grade do not predict the clinical behavior and metastatic potential for MPNST. […] High levels of survivin mRNA were significantly associated with FNCLCC tumor grade 3 and a high mitotic rate.
#47 Loss of H3K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics | Modern Pathology
https://www.nature.com/articles/modpathol2015134
The diagnosis of malignant peripheral nerve sheath tumor is challenging, particularly in the sporadic setting. […] Most malignant peripheral nerve sheath tumors pursue an aggressive clinical course with a 5-year survival rate of 35-50%. […] The lifetime risk of developing malignant peripheral nerve sheath tumor is estimated to be 5-10% for patients with neurofibromatosis type 1. […] Our findings suggest that PRC2 inactivation in malignant peripheral nerve sheath tumor may occur during progression to higher grades. […] 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. […] The value of H3K27me3 as a predictive marker for sensitivity to epigenetic-based therapies remains to be determined.
#48 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. […] 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.
#49 Diagnosis, Treatment and Survival of 65 Patients with Malignant Peripheral Nerve Sheath Tumors | Anticancer Research
https://ar.iiarjournals.org/content/34/2/777
Despite using less radical procedures with focus on limb salvage and complete (R0) tumor resection, we achieved comparable survival rates. […] Amputation should be the last resort because wide surgical resection shows no benefit in overall survival in soft tissue sarcoma and leads to extremely mutilating operations. […] Large retrospective studies are necessary to improve the poor evidence for treatment regimes of MPNSTs.