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70 (); 196-201
doi:
10.1016/j.jor.2025.05.054

Prognostic factors and treatment outcomes of malignant peripheral nerve sheath tumors (MPNST) of the extremities: A tertiary cancer institutional analysis

, , , , , , , , ,
Department of Surgical Oncology, The Gujarat Cancer and Research Institute, Ahmedabad, India

⁎Corresponding author: Abhijeet Ashok Salunke. drabhijeetsalunke@gmail.com

Received: , Accepted: ,
Professor P K Surendran Memorial Education Foundation
Disclaimer:
This article was originally published by Reed Elsevier India Pvt. Ltd. and was migrated to Scientific Scholar after the change of Publisher.

Abstract

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is an uncommon and aggressive ectomesenchymal soft tissue sarcoma that usually originates from peripheral nerves or pre-existing neurofibromas. These tumors are recognised for their high metastatic risk and challenging cases to treat. The purpose of this study was to analyse the factors impacting patient prognosis and treatment options by analysing treatment results and prognostic factors in patients with MPNST of the extremities.

This retrospective longitudinal study included 53 patients with histologically and immunohistochemically confirmed MPNST of the extremities, treated at a tertiary care cancer centre from 2011 to 2018. Data were collected on demographics, clinical presentation, treatment modalities (surgery, radiotherapy, chemotherapy, palliative care), and follow-up. Follow-up included physical exams, imaging, and CECT thorax at 3–6 month intervals for two years, then biannually for three more years.Variables assessed for prognostic impact included age, sex, NF1 status, tumor grade, size, location, margin status, metastasis at diagnosis, and treatment intent. Disease-free survival (DFS) was measured from the date of surgery, and overall survival (OS) from the date of diagnosis.

Among 53 patients with MPNST of the extremities, 52.83 % were over 45 years of age, and 63 % were male and 37 % were females. Most tumors (86.79 %) were sporadic, with NF1-associated tumors accounting for 13.20 %. Primary disease was more common (66.03 %) than recurrent (33.96 %). High-grade tumors were predominant, with 24.52 % classified as Grade 2 and 45.28 % as Grade 3. Metastatic disease was present in 20.75 % of cases. Tumor size was variable, with the largest proportion (32 %) in the 5–10 cm range. Curative treatment was administered to 67.92 % of patients, while 32.07 % received palliative care. Adjuvant radiotherapy was given to 35.64 % of patients, and chemotherapy (doxorubicin + ifosfamide) was administered to those with metastases. The overall survival rate was 58.46 %. Tumor grade (G2, G3), metastatic presentation, and intent of treatment were significantly associated with survival outcomes. NF1 status did not significantly impact overall survival, though NF1-positive patients benefited significantly from adjuvant radiation. Tumor grade was a strong predictor of survival, with Grade 3 tumors showing poorer outcomes than Grades 1 and 2.

Our current study focuses on the predictive importance of treatment intent, tumor grade, and metastatic manifestation in patients with malignant peripheral nerve sheath tumors (MPNST) of the extremities. While variables such as age, gender, tumor size, surgical margin status, and NF1 status exhibited varying correlations, the most accurate predictors of survival were tumor biology, namely histological grade and metastatic occurrence.The cornerstone of curative treatment continues to be postoperative radiation after wide local excision with negative surgical margins. The results highlight the necessity of aggressive care and early detection, particularly in high-grade or NF1-associated patients.

Keywords

Malignant peripheral nerve sheath tumor
MPNST
Extremity
Neurofibromatosis
Soft tissue sarcoma
1

1 Introduction

Malignant peripheral nerve sheath tumour (MPNST) is a connective tissue sarcoma that surrounds the nerves and has a poor prognosis, a high mortality rate, and low morbidity. Although the precise origin is yet unknown, MPNST is a kind of soft tissue sarcoma that may have a genetic relationship within families.1,2 MPNST is frequently seen in the limbs and trunk, while it is uncommon in the head and neck. In 2002, the World Health Organization (WHO) classified neurosarcoma, neurofibrosarcoma, malignant schwannoma, and malignant neurilemmoma as MPNST. Later, in 2013, the World Health Organization (WHO) defined MPNST as soft tissue tumors of nervous system tumour classes III to IV, which encompasses both malignant peripheral epithelioid nerve sheath tumour and malignant triton tumour.3

MPNST primarily affects individuals between the ages of 20 and 60. The etiology of MPNST includes associations with neurofibromatosis type 1 (NF1), previous radiation therapy, or it may occur sporadically.4 MPNST in the extremities include peripheral edema, numbness, burning sensations, loss of balance, and difficulty moving the limbs.5 It is estimated that 20–50 % of MPNST cases are associated with NF1, and 8–13 % of patients with NF1 are at risk for developing MPNST.5 Disease-free survival for patients with MPNST and NF1 has been reported to be lower than 5 years, with survival rates ranging from 35 % to 50 %.10–12

There are no standard approaches to treat MPNST due to low incident rates and as MPNST is very aggressive, first-line treatment of MPNST is surgical resection and adjuvant treatment include chemotherapy and radiotherapy. NF1 is present in 20–50 % of MPNST diagnoses, and 8–13 % of NF1 patients are at risk for MPNST.6–9 In cases of MPNST with NF-1, disease-free survival has been found to be between 35 and 50 percent.10–12 Due to the rarity of the disease and the aggressive nature of MPNST, there are no established treatment protocols. Surgical resection is the first line of treatment, followed by adjuvant chemotherapy and radiation therapy.1–14 MPNST has a poor prognosis and a significant risk of metastasising. Tumor size, tumour grade, location, surgical margin status, and recurrence are the most important prognostic factors, while histologic grading is one of these major prognostic markers and indicators of metastatic risk.15–17

In this study we analysed the patients diagnosed with malignant peripheral nerve sheath tumors (MPNST) of the extremities to investigate their clinicopathological features, therapeutic result, overall survival, and other prognostic variables.

2

2 Materials and Methods

This retrospective longitudinal study included 53 patients with histologically and immunohistochemically confirmed MPNST of the extremities, treated at a tertiary care cancer centre from 2011 to 2018. Clinical information was gathered from previous patient records. CECT and MRI were used to staging lesions in the head and neck, limbs, thorax, abdomen, and pelvis.Tumors were graded using the French grading method. The eighth edition of the American Joint Committee on Cancer (AJCC) was used to determine tumour size (T size). The following criteria were used to establish margin status: R0 = microscopically negative margins, R1 = microscopically positive margins, and R2 = macroscopically positive margins.

Data on the patients' multidisciplinary treatments, including surgery, radiation therapy, chemotherapy, and palliative care, were gathered. For the first two years, follow-up surveillance consisted of a history and physical examination, local part imaging where necessary, and CECT thorax every 3–6 months, then every 6 months for the following three years.Age, sex, the presence of NF1, the location and presentation of the tumor, the existence of metastatic disease at presentation, the intention to treat, the grade, size, margin, and adjuvant radiation were all examined for their effects on overall survival. Disease-free survival was measured starting from the date of surgery, and overall survival was measured starting from the date of histological diagnosis (see Table 1).

Table 1 Demographic, tumor, and treatment characteristics of the MPNST cohort (n = 53).
Parameters N % of total
Age
<45 25 47.16 %
>45 28 52.83 %
Sex
Male 33 62.26 %
Female 20 37.73 %
Associated with NF1
Yes (2) 07 13.20 %
No (1) 46 86.79 %
Presentation
Recurrent (2) 18 33.96 %
Primary(1) 35 66.03 %
Grade(G)
G1 13 24.52 %
G2 13 24.52 %
G3 24 45.28 %
Unknown 03 5.66 %
Tumor size
T1 (<5 cm) 6 11.32 %
T2 (5–10 cm) 17 32.07 %
T3 (10–15 cm) 10 18.86 %
T4 (>15 cm) 11 20.75 %
Unknown 09 16.98 %
Metastasis on presentation
Yes 11 20.75 %
No 42 79.24 %
Intent of treatment
Curative 36 67.92 %
Palliative 17 32.07 %
Margin status (n = 53)
R0 24 45.28 %
R1 05 9.43 %
R2 02 3.77 %
Unknown 22 41.50 %
Adjuvant radiotherapy (n = 53)
Yes 19 35.84 %
No 10 18.86 %
Unknown 17 32.07 %
Not applicable(for amputation) 7 13.20 %
Survival (n = 53)
Death 22 41.50 %
Alive 31 58.49 %

Based on tumour presentation, NF1 status, and death event, clinical and demographic characteristics were separated. Univariate analysis was used to assess the relationship between various clinical and demographic data in patients and factors such NF1 status, tumour presentation, and overall survival.For commuting the odds ratios and p values between groups, we utilised the medcalc software. The effect of clinicopathological variables statistically on patients' overall survival was extrapolated using Kaplan-Meier (Mantel-Cox) survival statistics. Log-rank analysis was used to analyse the clinicopathological characteristics such as neurofibromatosis-1 status (NF1+ versus NF1), tumour grade (G1/G2/G3), and clinical presentation [primary/recurrence/metastasis] in relation to overall survival of the patients. For all analyses, a p value of 0.05 was considered statistically significant.

3

3 Results

3.1

3.1 Demographic factors

Out of the 53 cases of malignant peripheral nerve sheath tumors (MPNST) involving the extremities, 52.83 % were older than 45 years, while 47.16 % were younger. The male population accounted for 62.26 % of the cases, whereas females made up 37.73 %.

3.2

3.2 Tumor characteristics

The majority of the tumors (86.79 %) were sporadic, with 46 patients falling into this category. The remaining 13.20 % of cases (7 patients) were associated with Neurofibromatosis Type 1 (NF-1). Among the 53 patients, 24.52 % had Grade 2 tumors, while 45.28 % had Grade 3 tumors. Metastasis was found in 20.75 % of the patients. When considering tumor size, 20.75 % had tumors larger than 15 cm, 32 % had tumors between 5 and 10 cm, and 11 % had tumors smaller than 5 cm. Another 18 % had tumors measuring greater than 15 cm (see Table 2).

Table 2 Distribution of variables with respect to Survival status.
Parameters Scoring Alive Death OR(95 % CI) p-Value
Age <45=1>45 = 2 1615 913 0.65(0.21–1.95) 0.44
Sex Female = 1Male = 2 1219 814 0.90(0.29–2.80) 0.86
Association with NF1 No = 1Yes = 2 292 175 0.23(0.04–1.34) 0.10
Presentation Primary = 1Recurrent = 2 2011 157 1.18(0.37–3.76) 0.78
Grade(G) G1 = 1G2 = 2G3 = 3 12611 1713 0.07(0.07–0.72)0.07(0.007–0.63) 0.02∗ 0.01∗
Grade(G) Low = 1High = 2 1415 120 0.05(0.006–0.45) 0.007∗
Tumor sizeT1 (<5 cm)T2 (5–10 cm)T3 (10–15 cm)T4 (>15 cm) T1 = 1T2 = 2T3 = 3T4 = 4 51055 1756 0.28(0.02–3.01)0.20(0.01–2.38) 0.290.200.15
Metastasis on Presentation No = 1Yes = 2 310 1111 0.16(0.01–1.94) 0.005∗
Intent of treatment Curative = 1Palliative = 2 274 913 0.10(0.02–0.39) 0.001∗
Presented after resection from outside? No = 1Yes = 2 1710 72 2.06(0.35–11.90) 0.42
Margin status R0 = 1R1 = 2 204 43 0.27(0.04–1.68) 0.15
Adjuvant radiotherapy No = 1Yes = 2 715 34 1.61(0.28–9.20) 0.59
DFS DFS = 0Event = 1 207 09 0.01(0.001–0.37) 0.009∗
3.3

3.3 Treatment-related factors

Of the 53 patients, 66.03 % had primary cases, while 33.96 % had recurrent tumors. Regarding surgical margins, 45.28 % had negative margins, 9.43 % underwent R1 resections, and 3.77 % had R2 resections. In terms of treatment intent, 67.92 % of the patients received curative therapy, while 32.07 % were treated palliatively, typically due to metastatic disease or an inoperable condition. Adjuvant radiation therapy was administered to 35.64 % (19 patients), while 18.86 % (10 patients) did not receive it.

Postoperative external beam radiation therapy (EBRT) was given to 19 patients, utilizing a total dose of 50 Gy in 20–25 fractions, delivered through either a linear accelerator or cobalt. This radiation therapy was focused on the surgical scar and tumor bed, with a margin extending 5 cm craniocaudally. No patients in the study received preoperative radiation therapy. Adjuvant chemotherapy (doxorubicin and ifosfamide) was given to 11 patients with metastatic disease, following institutional protocol for soft tissue sarcoma.

3.4

3.4 Survival analysis

The overall survival rate was 58.46 % (31 patients), while 41.50 % (22 patients) experienced mortality. Upon examining survival correlations, no significant relationship was found between overall survival and age, gender, NF-1 status, tumor size, recurrence, resection margin status, or adjuvant radiotherapy. However, high tumor grade, metastatic disease, treatment intent, and disease-free survival (DFS) were strongly correlated with survival status.

For high-grade tumors, Grade 2 and Grade 3 showed significant correlations with survival outcomes, with Grade 2 tumors having an odds ratio (OR) of 0.07 (95 % CI: 0.007–0.63, p = 0.01) and Grade 3 tumors an OR of 0.07 (95 % CI: 0.07–0.72, p = 0.02). Non-metastatic cases had a significantly higher survival rate compared to metastatic cases (OR: 0.16, 95 % CI: 0.01–1.94, p = 0.005). Patients who received curative care had better survival outcomes compared to those who received palliative care, with an odds ratio of 0.10 (95 % CI: 0.022–0.39, p = 0.001) (see Table 3).

Table 3 Distribution of variables with respect to Presentation status.
Parameters Scoring Primary Recurrent OR(95 % CI) p-Value
Age <45=1>45 = 2 1718 810 0.84(0.27–2.65) 0.78
Sex Female = 1Male = 2 1718 312 0.21(0.05–0.86) 0.03∗
Association with NF1 No = 1Yes = 2 305 162 1.33(0.23–7.66) 0.75
Grade(G) G1 = 1G2 = 2G3 = 3 8817 557 1.00(0.21–4.86)1.52(0.36–6.29) 1.000.56
Grade(G) Low = 1High = 2 924 611 1.45(0.41–5.10) 0.56
Tumor sizeT1 (<5 cm)T2 (5–10 cm)T3 (10–15 cm)T4 (>15 cm) T1 = 1T2 = 2T3 = 3T4 = 4 411510 2651 0.91(0.12–6.56)0.50(0.06–4.09)0.05(0.03–0.71) 0.930.510.02∗
Metastasis on Presentation No = 1Yes = 2 278 153 3.33(0.8413.19) 0.08#
Intent of Treatment Curative = 1Palliative = 2 2312 135 1.36(0.40–4.71) 0.63
Presented after resection from outside? No = 1Yes = 2 149 103 2.14(0.46–9.98) 0.33
Margin status R0 = 1R1 = 2 137 110 12.77(0.66–248377) 0.09#
Adjuvant Radiotherapy No = 1Yes = 2 714 35 1.20(0.22–6.53) 0.83
Survival Alive = 1Death = 2 2015 117 1.18(0.37–3.76) 0.78
DFS DFS = 0Event = 1 1211 85 1.47(0.36–5.85) 0.59
3.5

3.5 Disease features and mode of presentation

Tumor size did not significantly differ between initial and recurrent cases. Although margin status was associated with an increased risk of recurrence (OR: 12.77, 95 % CI: 0.66–24.83, p = 0.09), this was not statistically significant. Additionally, no significant associations were found between age, NF-1 history, grade, treatment intent, recurrence, adjuvant radiotherapy, and disease presentation (see Table 4).

Table 4 Distribution of variables with respect to the presence of tumor.
Parameters Scoring NF-ve NF + ve OR(95 % CI) p-Value
Age <45=1>45 = 2 1927 61 8.52(0.95–76.71) 0.06∗
Sex Female = 1Male = 2 1630 43 2.50(0.50–12.57) 0.26
Presentation Primary = 1Recurrent = 2 3016 52 1.33(0.23–7.66) 0.75
Grade(G) G1 = 1G2 = 2G3 = 3 121021 133 0.28(0.02–3.10) 0.30
Grade(G) Low = 1High = 2 1429 16 0.35(0.03–3.15) 0.35
Tumor size T1 (<5 cm)T2 (5–10 cm)T3 (10–15 cm)T4 (>15 cm) 61699 0212 0.51(0.02–12.07)0.48(0.01–13.92)0.29(0.01–7.14) 0.680.670.45
Metastasis on presentation No = 1Yes = 2 379 52 0.61(0.10–3.66) 0.58
Intent of treatment Curative = 1Palliative = 2 3214 43 0.58(0.12–2.96) 0.52
Presented after resection from outside? No = 1Yes = 2 2111 31 1.58(0.15–16.94) 0.71
Margin status R0 = 1R1 = 2 226 21 0.54(0.04–7.09) 0.64
Adjuvant Radiotherapy No = 1Yes = 2 917 12 0.01(0.001–0.16) 0.0008∗
Survival Alive = 0Death = 1 2012 25 0.24(0.04–1.44) 0.12
DFS DFS = 0Event = 1 2817 04 0.06(0.00–1.29) 0.07#
3.6

3.6 NF-1 status and survival

NF-1 positivity showed a trend toward higher disease risk in patients over 45 years of age (OR: 8.52, p = 0.06), though this was not statistically significant. In NF-1 positive patients, adjuvant radiation therapy significantly improved survival outcomes (OR: 0.01, p = 0.0008). NF-1 negative status was associated with improved disease-free survival, though this was not statistically significant (OR: 0.06, p = 0.07). Median survival rates between NF-1 positive and negative patients did not differ significantly (p = 0.08, CI: 0.007–1.125) (see Fig. 1).

Kaplan–Meier curve of overall survival differences in MPNST of extremity cases with neurofibromatosis-1 status (NF1+ versus NF1).
Fig. 1 Kaplan–Meier curve of overall survival differences in MPNST of extremity cases with neurofibromatosis-1 status (NF1+ versus NF1).
3.7

3.7 Tumor grading and survival

A significant difference in median survival was observed between Grade 1 (undefined) and Grade 3 tumors, with Grade 3 tumors having a median survival of 15 months (p = 0.015, CI: 0.08–0.69). For Grade 2 tumors, the median survival was 21 months, and for Grade 3 tumors, it was 15 months, though the difference was not statistically significant (p = 0.53). A significant difference was found when comparing Grade 1 (undefined) to Grade 2 tumors, with Grade 2 tumors having a median survival of 21 months (p = 0.02, 95 % CI: 0.04–0.68) (see Fig. 2).

Kaplan–Meier curve of overall survival differences with Tumor Grade.
Fig. 2 Kaplan–Meier curve of overall survival differences with Tumor Grade.
3.8

3.8 Primary vs. recurrent disease

No significant difference in survival was observed between primary and recurrent diseases, with a median survival of 53 months for primary disease and 51 months for recurrent disease (p = 0.85, CI: 0.47–2.78). However, a significant difference was noted when comparing primary disease to metastatic disease, with primary disease having a median survival of 53 months, while metastatic disease had a median survival of just 8 months (p = 0.001, 95 % CI: 0.05–0.42) (see Fig. 3).

Kaplan–Meier curve of overall survival differences with Metastasis on presentation.
Fig. 3 Kaplan–Meier curve of overall survival differences with Metastasis on presentation.
4

4 Discussion

Malignant peripheral nerve sheath tumor (MPNST) is a rare and aggressive subtype of soft tissue sarcoma. MPNSTs arising in the extremities are particularly uncommon and heterogeneous, occurring either sporadically or in association with neurofibromatosis type 1 (NF1). This study presents clinical data and treatment outcomes for both sporadic and NF1-associated MPNSTs specifically localised to the extremities. A key strength of this study is its stringent inclusion criteria, which ensured a homogenous and well-defined patient population.

In our cohort, the five-year overall survival (OS) rate was 58.49 %. This aligns with findings by Kolberg et al., who reported no significant survival difference between sporadic and NF1-associated MPNSTs.19 Similarly, Anghileri et al. noted better outcomes for extremity MPNSTs compared to tumors in the trunk, head, and neck regions.18 A notable 45 % (n = 24) of patients had grade 3 tumors. Previous studies, such as those by Vauthey et al., have identified extremity MPNSTs as typically high-grade and deep-seated.21 Our findings similarly indicate that high-grade tumors (grade 2 and 3) are associated with reduced survival rates and higher mortality compared to low-grade (grade 1) tumors, which were linked to improved outcomes. These findings are consistent with prior reports emphasising the prognostic impact of tumor grade.22,23

Tumor size also played a critical role in prognosis. Although survival status did not show statistically significant correlation with size, larger tumors were consistently associated with worse outcomes (T1: 16 %, T2: 41 %, T3: 50.5 %, T4: 54 %). Martin et al. and others have previously demonstrated that large tumor size independently predicts poorer survival.18,23–25 No significant difference in survival was observed between patients undergoing R0 (clear margins) versus R1 (microscopically positive margins) resections, consistent with findings by Bernthal et al. and other literature on margin status and outcome.24,26

Survival analysis also revealed that male patients and those above 45 years had relatively poorer outcomes, with mortality rates of 42 % and 46 %, respectively, though these differences were not statistically significant. Similar trends were noted in a study by Martin et al., which identified male sex and age ≥60 as independent predictors of worse survival.24 Nevertheless, previous studies have supported the role of adjuvant radiotherapy in improving local control and potentially survival.20,22,23,27,28

NF1 status was explored as a prognostic factor.29 Although not statistically significant, NF1-positive patients showed associations with adverse disease features, including high tumor grade, recurrence, R1 resection, metastatic disease, and poor survival. These trends echo previous findings suggesting that NF1 is a marker of increased MPNST risk and inferior outcomes.2,11,25,30–34 Importantly, adjuvant radiation demonstrated a statistically significant protective effect (p = 0.0008) across both NF1-positive and negative groups.Several previous studies have shown that positive surgical margins increase the risk of local recurrence by 2.4 times and disease-specific mortality by 1.8 times.34–49

This study has several limitations, primarily due to its retrospective design, which may introduce bias and limit control over confounding variables. The small sample size reduces statistical power and limits the generalisability of findings. Additionally, the ability to explore underlying disease mechanisms and accurately predict patient outcomes is constrained by the limited cohort size.We were unable to establish a statistically significant association between surgical margin status and disease recurrence or survival. Nonetheless, our findings suggest that factors such as age, gender, tumor grade, tumor size, resection margins, and NF1 status may influence both disease-free and overall survival in MPNSTs of the extremities.

To better understand these associations and improve clinical outcomes, future multicenter studies with larger sample sizes are strongly recommended.

5

5 Conclusion

Our research focuses on the predictive importance of treatment intent, tumor grade, and metastatic manifestation in patients with malignant peripheral nerve sheath tumors (MPNST) of the extremities. While variables such as age, gender, tumor size, surgical margin status, and NF1 status exhibited varying correlations, the most accurate predictors of survival were tumor biology, namely histological grade and metastatic occurrence.The cornerstone of curative treatment continues to be postoperative radiation after wide local excision with negative surgical margins.

The results highlight the necessity of aggressive care and early detection, particularly in high-grade or NF1-associated patients.To further understand the function of molecular markers and create tailored treatments to improve outcomes in this uncommon and aggressive cancer, larger prospective studies are necessary.

CRediT authorship contribution statement

Abhijeet Ashok Salunke: Conceptualization, Methodology, Software. Sanjay Singh: Data curation, Writing – original draft. Tarun Barnalla: Visualization, Investigation. Nandlal Bharwani: Conceptualization, Methodology, Software. Dhruv Patel: Software, Validation. Keval Patel: Writing – review & editing. Vikas Warikoo: Conceptualization, Methodology, Software. Mohit Sharma: Conceptualization, Methodology, Software. Ketul Puj: Writing – review & editing. Shashank Pandya: Supervision.

Ethical statement

Ethical committee approval obtained.

Financial disclosures

Nil.

Funding

Nil.

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