Analysis of clinical features, treatment methods, and prognostic influence factors in patients with malignant peripheral nerve sheath tumor_Chinese Journal of Reparative and Reconstructive Surgery

Authors：

SHI Bochong ^1,2 , ZHENG Haoyu ^1,2 ^△ , WU Huajian ³ , HU Xianglin ^1,2 ,  YAN Wangjun ^1,2

1. Department of Musculoskeletal Oncology, Fudan University Shanghai Cancer Center, Shanghai, 200032, P. R. China;
2. Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, 200032, P. R. China;
3. Department of Musculoskeletal Oncology, Xiamen Branch, Fudan University Shanghai Cancer Center, Xiamen Fujian, 361003, P. R. China;

Corresponding author：

YAN Wangjun, Email: yanwj@fudan.edu.cn

Keywords：

Malignant peripheral nerve sheath tumor; neurofibromatosis type 1; local recurrence; COX proportional hazards regression analysis

DOI：

10.7507/1002-1892.202406040

Video：

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Objective To investigate the clinical features, treatment methods, and prognostic influence factors of patients with malignant peripheral nerve sheath tumor (MPNST). Methods A retrospective analysis was conducted on 96 MPNST patients treated between January 1, 2015 and December 31, 2021. There were 46 males and 50 females, aged between 15 and 87 years (mean, 48.2 years). The tumors were located in the trunk in 50 cases, extremities in 39 cases, and head and neck in 7 cases. The maximum tumor diameter was <5 cm in 49 cases, ≥5 cm in 32 cases, with 15 cases missing data. Tumor depth was deep in 77 cases and superficial in 19 cases. The Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) histological grading was G1 in 9 cases, G2 in 12 cases, and G3 in 34 cases, with 41 cases missing data. There were 37 recurrent MPNST cases, 32 cases with neurofibromatosis type 1 (NF1), and 26 cases in stage Ⅳ. Postoperative adjuvant radiotherapy was administered to 25 patients, perioperative chemotherapy to 45 patients, and anlotinib-targeted therapy to 30 patients. R₀ resection was achieved in 73 cases. Patients were divided into groups based on the presence or absence of NF1, and baseline data between the two groups were compared. Kaplan-Meier curves were generated to assess disease-free survival (DFS) and overall survival (OS) based on various factors (age, gender, presence of NF1, recurrent MPNST, stage Ⅳ MPNST, FNCLCC grade, R₀ resection, tumor location, tumor size, tumor depth, perioperative chemotherapy, postoperative adjuvant radiotherapy, and anlotinib-targeted therapy), and differences between survival curves were analyzed using the Log-Rank test. Multivariate COX proportional hazards regression was used to identify independent prognostic factors for MPNST. Results Patients with NF1 had a significantly higher proportion of superficial tumors and lower FNCLCC grade compared to those without NF1 (P<0.05); no significant difference was found for other variables (P<0.05). Kaplan-Meier analysis showed that recurrent MPNST, stage Ⅳ MPNST, FNCLCC grade, R₀ resection, perioperative chemotherapy, and anlotinib-targeted therapy were factors influencing 1-year DFS (P<0.05), while stage Ⅳ MPNST, FNCLCC grade, and perioperative chemotherapy were factors affecting 3-year OS (P<0.05). Multivariate COX proportional hazards regression analysis revealed that recurrent MPNST and high-grade FNCLCC (G3) were independent prognostic factors for 1-year DFS (P<0.05), while stage Ⅳ MPNST, superficial tumor depth, age over 60 years, postoperative adjuvant radiotherapy, and anlotinib-targeted therapy were independent prognostic factors for 3-year OS (P<0.05). Conclusion MPNST patients with NF1 tend to have more superficial tumors and lower FNCLCC grades. FNCLCC grade, R₀ resection, and adjuvant therapies, including radiotherapy and anlotinib-targeted therapy, are closely associated with MPNST prognosis. Complete surgical resection should be prioritized in clinical management, along with adjuvant treatments such as radiotherapy and targeted therapy of anlotinib to improve patient outcomes.

Citation： SHI Bochong, ZHENG Haoyu, WU Huajian, HU Xianglin, YAN Wangjun. Analysis of clinical features, treatment methods, and prognostic influence factors in patients with malignant peripheral nerve sheath tumor. Chinese Journal of Reparative and Reconstructive Surgery, 2024, 38(10): 1193-1201. doi: 10.7507/1002-1892.202406040 Copy

1.	Staedtke V, Bai RY, Blakeley JO. Cancer of the peripheral nerve in neurofibromatosis type 1. Neurotherapeutics, 2017, 14(2): 298-306.
2.	Widemann BC, Lu Y, Reinke D, et al. Targeting sporadic and neurofibromatosis type 1 (NF1) related refractory malignant peripheral nerve sheath tumors (MPNST) in a phase Ⅱ study of everolimus in combination with bevacizumab (SARC016). Sarcoma, 2019, 2019: 7656747. doi: 10.1155/2019/7656747.
3.	Korf B. A phase 1/2 trial of the MEK inhibitor selumetinib and bromodomain inhibitor with durvalumab (MEDI4736), a PD-L1 antibody for sarcomas including malignant peripheral nerve sheath tumors (NF112): NCT05253131[R/OL]. 2024[2024-01-01]. https://clinicaltrials.gov/study/NCT05253131.
4.	中国临床试验注册中心——世界卫生组织国际临床试验注册平台一级注册机构 [EB/OL]. [2024-06-07]. https://www.chictr.org.cn/showproj.html?proj=191666.
5.	Somatilaka BN, Sadek A, McKay RM, et al. Malignant peripheral nerve sheath tumor: models, biology, and translation. Oncogene, 2022, 41(17): 2405-2421.
6.	Trojani M, Contesso G, Coindre JM, et al. Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer, 1984, 33(1): 37-42.
7.	Gupta G, Maniker A. Malignant peripheral nerve sheath tumors. Neurosurg Focus, 2007, 22(6): E12. doi: 10.3171/foc.2007.22.6.13.
8.	Miao R, Wang H, Jacobson A, et al. Radiation-induced and neurofibromatosis-associated malignant peripheral nerve sheath tumors (MPNST) have worse outcomes than sporadic MPNST. Radiother Oncol, 2019, 137: 61-70.
9.	Kolberg M, Høland M, Agesen TH, et al. Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1. Neuro Oncol, 2013, 15(2): 135-147.
10.	Martin E, Coert JH, Flucke UE, et al. A nationwide cohort study on treatment and survival in patients with malignant peripheral nerve sheath tumours. Eur J Cancer, 2020, 124: 77-87.
11.	Rad E, Tee AR. Neurofibromatosis type 1: Fundamental insights into cell signalling and cancer. Semin Cell Dev Biol, 2016, 52: 39-46.
12.	Yao C, Zhou H, Dong Y, et al. Malignant peripheral nerve sheath tumors: latest concepts in disease pathogenesis and clinical management. Cancers (Basel), 2023, 15(4): 1077. doi: 10.3390/cancers15041077.
13.	Kroep JR, Ouali M, Gelderblom H, et al. First-line chemotherapy for malignant peripheral nerve sheath tumor (MPNST) versus other histological soft tissue sarcoma subtypes and as a prognostic factor for MPNST: an EORTC soft tissue and bone sarcoma group study. Ann Oncol, 2011, 22(1): 207-214.
14.	Carli M, Ferrari A, Mattke A, et al. Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group. J Clin Oncol, 2005, 23(33): 8422-8430.
15.	Higham CS, Steinberg SM, Dombi E, et al. SARC006: Phase Ⅱ trial of chemotherapy in sporadic and neurofibromatosis type 1 associated chemotherapy-naive malignant peripheral nerve sheath tumors. Sarcoma, 2017, 2017: 8685638. doi: 10.1155/2017/8685638.
16.	Kar M, Deo SV, Shukla NK, et al. Malignant peripheral nerve sheath tumors (MPNST)—clinicopathological study and treatment outcome of twenty-four cases. World J Surg Oncol, 2006, 4: 55. doi: 10.1186/1477-7819-4-55.
17.	Arshi A, Tajudeen BA, St John M. Malignant peripheral nerve sheath tumors of the head and neck: Demographics, clinicopathologic features, management, and treatment outcomes. Oral Oncol, 2015, 51(12): 1088-1094.
18.	Kahn J, Gillespie A, Tsokos M, et al. Radiation therapy in management of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Front Oncol, 2014, 4: 324. doi: 10.3389/fonc.2014.00324.
19.	Sun D, Xie XP, Zhang X, et al. Stem-like cells drive NF1-associated MPNST functional heterogeneity and tumor progression. Cell Stem Cell, 2021, 28(8): 1397-1410.
20.	Joseph NM, Mukouyama YS, Mosher JT, et al. Neural crest stem cells undergo multilineage differentiation in developing peripheral nerves to generate endoneurial fibroblasts in addition to Schwann cells. Development, 2004, 131(22): 5599-5612.
21.	Sarcoma Alliance for Research Through Collaboration. SARC031: A phase 2 trial of the MEK inhibitor selumetinib (AZD6244 hydrogen sulfate) in combination with the mTOR inhibitor sirolimus for patients with unresectable or metastatic malignant peripheral nerve sheath tumors: NCT03433183[R/OL]. 2023[2024-01-01]. https://clinicaltrials.gov/study/NCT03433183.
22.	Kohlmeyer JL, Lingo JJ, Kaemmer CA, et al. CDK4/6-MEK inhibition in MPNSTs causes plasma cell infiltration, sensitization to PD-L1 blockade, and tumor regression. Clin Cancer Res, 2023, 29(17): 3484-3497.
23.	Hiemcke-Jiwa LS, Meister MT, Martin E, et al. NTRK rearrangements in a subset of NF1-related malignant peripheral nerve sheath tumors as novel actionable target. Acta Neuropathol, 2023, 145(1): 149-152.
24.	Manji GA, Van Tine BA, Lee SM, et al. A phase Ⅰ study of the combination of pexidartinib and sirolimus to target tumor-associated macrophages in unresectable sarcoma and malignant peripheral nerve sheath tumors. Clin Cancer Res, 2021, 27(20): 5519-5527.
25.	Gampala S, Moon HR, Wireman R, et al. New Ref-1/APE1 targeted inhibitors demonstrating improved potency for clinical applications in multiple cancer types. Pharmacol Res, 2024, 201: 107092. doi: 10.1016/j.phrs.2024.107092.
26.	Wang J, Pollard K, Calizo A, et al. Activation of receptor tyrosine kinases mediates acquired resistance to MEK inhibition in malignant peripheral nerve sheath tumors. Cancer Res, 2021, 81(3): 747-762.
27.	Wang J, Pollard K, Allen AN, et al. Combined inhibition of SHP2 and MEK is effective in models of NF1-deficient malignant peripheral nerve sheath tumors. Cancer Res, 2020, 80(23): 5367-5379.
28.	Wang J, Calizo A, Zhang L, et al. CDK4/6 inhibition enhances SHP2 inhibitor efficacy and is dependent upon RB function in malignant peripheral nerve sheath tumors. Sci Adv, 2023, 9(47): eadg8876. doi: 10.1126/sciadv.adg8876.
29.	Kochat V, Raman AT, Landers SM, et al. Enhancer reprogramming in PRC2-deficient malignant peripheral nerve sheath tumors induces a targetable de-differentiated state. Acta Neuropathol, 2021, 142(3): 565-590.
30.	Gampala S, Shah F, Zhang C, et al. Exploring transcriptional regulators Ref-1 and STAT3 as therapeutic targets in malignant peripheral nerve sheath tumours. Br J Cancer, 2021, 124(9): 1566-1580.
31.	Zhang X, Lou HE, Gopalan V, et al. Single-cell sequencing reveals activation of core transcription factors in PRC2-deficient malignant peripheral nerve sheath tumor. Cell Rep, 2022, 40(12): 111363. doi: 10.1016/j.celrep.2022.111363.
32.	Second Affiliated Hospital, School of Medicine, Zhejiang University. Efficacy and safety of cadonilimab combined with anlotinibin in the treatment of advanced or metastatic soft tissue sarcoma with previous first-line standard treatment failure: NCT05926700[R/OL]. 2024[2024-01-01]. https://clinicaltrials.gov/study/NCT05926700.
33.	Chia Tai Tianqing Pharmaceutical Group Co. , Ltd. A registered randomized, double-blind, placebo-controlled (2∶1), multi-centered clinical trial of anlotinib as a treatment for soft tissue sarcoma: NCT02449343[R/OL]. 2017[2024-01-01]. https://clinicaltrials.gov/study/NCT02449343.
34.	Chia Tai Tianqing Pharmaceutical Group Co. , Ltd. Phase 2 study of anlotinib in advanced soft tissue sarcoma: NCT01878448[R/OL]. 2019[2024-01-01]. https://clinicaltrials.gov/study/NCT01878448.
35.	Sobczuk P, Teterycz P, Czarnecka AM, et al. Malignant peripheral nerve sheath tumors—Outcomes and prognostic factors based on the reference center experience. Surg Oncol, 2020, 35: 276-284.
36.	Zou C, Smith KD, Liu J, et al. Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Ann Surg, 2009, 249(6): 1014-1022.
37.	Okada K, Hasegawa T, Tajino T, et al. Clinical relevance of pathological grades of malignant peripheral nerve sheath tumor: a multi-institution TMTS study of 56 cases in Northern Japan. Ann Surg Oncol, 2007, 14(2): 597-604.
38.	Baehring JM, Betensky RA, Batchelor TT. Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment. Neurology, 2003, 61(5): 696-698.
39.	Fan Q, Yang J, Wang G. Clinical and molecular prognostic predictors of malignant peripheral nerve sheath tumor. Clin Transl Oncol, 2014, 16(2): 191-199.
40.	Valentin T, Le Cesne A, Ray-Coquard I, et al. Management and prognosis of malignant peripheral nerve sheath tumors: The experience of the French Sarcoma Group (GSF-GETO). Eur J Cancer, 2016, 56: 77-84.
41.	王天景, 黄润之, 贾景一, 等. 基于SEER数据库的不同年龄组恶性周围神经鞘膜瘤预后的研究. 同济大学学报 (医学版), 2021, 42(3): 381-387.
42.	Italiano A, Delva F, Mathoulin-Pelissier S, et al. Effect of adjuvant chemotherapy on survival in FNCLCC grade 3 soft tissue sarcomas: a multivariate analysis of the French Sarcoma Group Database. Ann Oncol, 2010, 21(12): 2436-2441.
43.	Penel N, Le Cesne A, Bui BN, et al. Imatinib for progressive and recurrent aggressive fibromatosis (desmoid tumors): an FNCLCC/French Sarcoma Group phase Ⅱ trial with a long-term follow-up. Ann Oncol, 2011, 22(2): 452-457.
44.	Wakeman KM, Zhang QS, Bandhlish A, et al. Fédération Nationale Des Centres de Lutte Contre Le Cancer (FNCLCC) grading, margin status and tumor location associate with survival outcomes in malignant peripheral nerve sheath tumors. Am J Clin Oncol, 2022, 45(1): 28-35.

1. Staedtke V, Bai RY, Blakeley JO. Cancer of the peripheral nerve in neurofibromatosis type 1. Neurotherapeutics, 2017, 14(2): 298-306.
2. Widemann BC, Lu Y, Reinke D, et al. Targeting sporadic and neurofibromatosis type 1 (NF1) related refractory malignant peripheral nerve sheath tumors (MPNST) in a phase Ⅱ study of everolimus in combination with bevacizumab (SARC016). Sarcoma, 2019, 2019: 7656747. doi: 10.1155/2019/7656747.
3. Korf B. A phase 1/2 trial of the MEK inhibitor selumetinib and bromodomain inhibitor with durvalumab (MEDI4736), a PD-L1 antibody for sarcomas including malignant peripheral nerve sheath tumors (NF112): NCT05253131[R/OL]. 2024[2024-01-01]. https://clinicaltrials.gov/study/NCT05253131.
4. 中国临床试验注册中心——世界卫生组织国际临床试验注册平台一级注册机构 [EB/OL]. [2024-06-07]. https://www.chictr.org.cn/showproj.html?proj=191666.
5. Somatilaka BN, Sadek A, McKay RM, et al. Malignant peripheral nerve sheath tumor: models, biology, and translation. Oncogene, 2022, 41(17): 2405-2421.
6. Trojani M, Contesso G, Coindre JM, et al. Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer, 1984, 33(1): 37-42.
7. Gupta G, Maniker A. Malignant peripheral nerve sheath tumors. Neurosurg Focus, 2007, 22(6): E12. doi: 10.3171/foc.2007.22.6.13.
8. Miao R, Wang H, Jacobson A, et al. Radiation-induced and neurofibromatosis-associated malignant peripheral nerve sheath tumors (MPNST) have worse outcomes than sporadic MPNST. Radiother Oncol, 2019, 137: 61-70.
9. Kolberg M, Høland M, Agesen TH, et al. Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1. Neuro Oncol, 2013, 15(2): 135-147.
10. Martin E, Coert JH, Flucke UE, et al. A nationwide cohort study on treatment and survival in patients with malignant peripheral nerve sheath tumours. Eur J Cancer, 2020, 124: 77-87.
11. Rad E, Tee AR. Neurofibromatosis type 1: Fundamental insights into cell signalling and cancer. Semin Cell Dev Biol, 2016, 52: 39-46.
12. Yao C, Zhou H, Dong Y, et al. Malignant peripheral nerve sheath tumors: latest concepts in disease pathogenesis and clinical management. Cancers (Basel), 2023, 15(4): 1077. doi: 10.3390/cancers15041077.
13. Kroep JR, Ouali M, Gelderblom H, et al. First-line chemotherapy for malignant peripheral nerve sheath tumor (MPNST) versus other histological soft tissue sarcoma subtypes and as a prognostic factor for MPNST: an EORTC soft tissue and bone sarcoma group study. Ann Oncol, 2011, 22(1): 207-214.
14. Carli M, Ferrari A, Mattke A, et al. Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group. J Clin Oncol, 2005, 23(33): 8422-8430.
15. Higham CS, Steinberg SM, Dombi E, et al. SARC006: Phase Ⅱ trial of chemotherapy in sporadic and neurofibromatosis type 1 associated chemotherapy-naive malignant peripheral nerve sheath tumors. Sarcoma, 2017, 2017: 8685638. doi: 10.1155/2017/8685638.
16. Kar M, Deo SV, Shukla NK, et al. Malignant peripheral nerve sheath tumors (MPNST)—clinicopathological study and treatment outcome of twenty-four cases. World J Surg Oncol, 2006, 4: 55. doi: 10.1186/1477-7819-4-55.
17. Arshi A, Tajudeen BA, St John M. Malignant peripheral nerve sheath tumors of the head and neck: Demographics, clinicopathologic features, management, and treatment outcomes. Oral Oncol, 2015, 51(12): 1088-1094.
18. Kahn J, Gillespie A, Tsokos M, et al. Radiation therapy in management of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Front Oncol, 2014, 4: 324. doi: 10.3389/fonc.2014.00324.
19. Sun D, Xie XP, Zhang X, et al. Stem-like cells drive NF1-associated MPNST functional heterogeneity and tumor progression. Cell Stem Cell, 2021, 28(8): 1397-1410.
20. Joseph NM, Mukouyama YS, Mosher JT, et al. Neural crest stem cells undergo multilineage differentiation in developing peripheral nerves to generate endoneurial fibroblasts in addition to Schwann cells. Development, 2004, 131(22): 5599-5612.
21. Sarcoma Alliance for Research Through Collaboration. SARC031: A phase 2 trial of the MEK inhibitor selumetinib (AZD6244 hydrogen sulfate) in combination with the mTOR inhibitor sirolimus for patients with unresectable or metastatic malignant peripheral nerve sheath tumors: NCT03433183[R/OL]. 2023[2024-01-01]. https://clinicaltrials.gov/study/NCT03433183.
22. Kohlmeyer JL, Lingo JJ, Kaemmer CA, et al. CDK4/6-MEK inhibition in MPNSTs causes plasma cell infiltration, sensitization to PD-L1 blockade, and tumor regression. Clin Cancer Res, 2023, 29(17): 3484-3497.
23. Hiemcke-Jiwa LS, Meister MT, Martin E, et al. NTRK rearrangements in a subset of NF1-related malignant peripheral nerve sheath tumors as novel actionable target. Acta Neuropathol, 2023, 145(1): 149-152.
24. Manji GA, Van Tine BA, Lee SM, et al. A phase Ⅰ study of the combination of pexidartinib and sirolimus to target tumor-associated macrophages in unresectable sarcoma and malignant peripheral nerve sheath tumors. Clin Cancer Res, 2021, 27(20): 5519-5527.
25. Gampala S, Moon HR, Wireman R, et al. New Ref-1/APE1 targeted inhibitors demonstrating improved potency for clinical applications in multiple cancer types. Pharmacol Res, 2024, 201: 107092. doi: 10.1016/j.phrs.2024.107092.
26. Wang J, Pollard K, Calizo A, et al. Activation of receptor tyrosine kinases mediates acquired resistance to MEK inhibition in malignant peripheral nerve sheath tumors. Cancer Res, 2021, 81(3): 747-762.
27. Wang J, Pollard K, Allen AN, et al. Combined inhibition of SHP2 and MEK is effective in models of NF1-deficient malignant peripheral nerve sheath tumors. Cancer Res, 2020, 80(23): 5367-5379.
28. Wang J, Calizo A, Zhang L, et al. CDK4/6 inhibition enhances SHP2 inhibitor efficacy and is dependent upon RB function in malignant peripheral nerve sheath tumors. Sci Adv, 2023, 9(47): eadg8876. doi: 10.1126/sciadv.adg8876.
29. Kochat V, Raman AT, Landers SM, et al. Enhancer reprogramming in PRC2-deficient malignant peripheral nerve sheath tumors induces a targetable de-differentiated state. Acta Neuropathol, 2021, 142(3): 565-590.
30. Gampala S, Shah F, Zhang C, et al. Exploring transcriptional regulators Ref-1 and STAT3 as therapeutic targets in malignant peripheral nerve sheath tumours. Br J Cancer, 2021, 124(9): 1566-1580.
31. Zhang X, Lou HE, Gopalan V, et al. Single-cell sequencing reveals activation of core transcription factors in PRC2-deficient malignant peripheral nerve sheath tumor. Cell Rep, 2022, 40(12): 111363. doi: 10.1016/j.celrep.2022.111363.
32. Second Affiliated Hospital, School of Medicine, Zhejiang University. Efficacy and safety of cadonilimab combined with anlotinibin in the treatment of advanced or metastatic soft tissue sarcoma with previous first-line standard treatment failure: NCT05926700[R/OL]. 2024[2024-01-01]. https://clinicaltrials.gov/study/NCT05926700.
33. Chia Tai Tianqing Pharmaceutical Group Co. , Ltd. A registered randomized, double-blind, placebo-controlled (2∶1), multi-centered clinical trial of anlotinib as a treatment for soft tissue sarcoma: NCT02449343[R/OL]. 2017[2024-01-01]. https://clinicaltrials.gov/study/NCT02449343.
34. Chia Tai Tianqing Pharmaceutical Group Co. , Ltd. Phase 2 study of anlotinib in advanced soft tissue sarcoma: NCT01878448[R/OL]. 2019[2024-01-01]. https://clinicaltrials.gov/study/NCT01878448.
35. Sobczuk P, Teterycz P, Czarnecka AM, et al. Malignant peripheral nerve sheath tumors—Outcomes and prognostic factors based on the reference center experience. Surg Oncol, 2020, 35: 276-284.
36. Zou C, Smith KD, Liu J, et al. Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Ann Surg, 2009, 249(6): 1014-1022.
37. Okada K, Hasegawa T, Tajino T, et al. Clinical relevance of pathological grades of malignant peripheral nerve sheath tumor: a multi-institution TMTS study of 56 cases in Northern Japan. Ann Surg Oncol, 2007, 14(2): 597-604.
38. Baehring JM, Betensky RA, Batchelor TT. Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment. Neurology, 2003, 61(5): 696-698.
39. Fan Q, Yang J, Wang G. Clinical and molecular prognostic predictors of malignant peripheral nerve sheath tumor. Clin Transl Oncol, 2014, 16(2): 191-199.
40. Valentin T, Le Cesne A, Ray-Coquard I, et al. Management and prognosis of malignant peripheral nerve sheath tumors: The experience of the French Sarcoma Group (GSF-GETO). Eur J Cancer, 2016, 56: 77-84.
41. 王天景, 黄润之, 贾景一, 等. 基于SEER数据库的不同年龄组恶性周围神经鞘膜瘤预后的研究. 同济大学学报 (医学版), 2021, 42(3): 381-387.
42. Italiano A, Delva F, Mathoulin-Pelissier S, et al. Effect of adjuvant chemotherapy on survival in FNCLCC grade 3 soft tissue sarcomas: a multivariate analysis of the French Sarcoma Group Database. Ann Oncol, 2010, 21(12): 2436-2441.
43. Penel N, Le Cesne A, Bui BN, et al. Imatinib for progressive and recurrent aggressive fibromatosis (desmoid tumors): an FNCLCC/French Sarcoma Group phase Ⅱ trial with a long-term follow-up. Ann Oncol, 2011, 22(2): 452-457.
44. Wakeman KM, Zhang QS, Bandhlish A, et al. Fédération Nationale Des Centres de Lutte Contre Le Cancer (FNCLCC) grading, margin status and tumor location associate with survival outcomes in malignant peripheral nerve sheath tumors. Am J Clin Oncol, 2022, 45(1): 28-35.

Chinese Journal of Reparative and Reconstructive Surgery

Analysis of clinical features, treatment methods, and prognostic influence factors in patients with malignant peripheral nerve sheath tumor

Abstract Full text Figures/Tables Video References Cited by

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