Results of surgical treatment of patients with malignant peripheral nerve sheath tumors: a retrospective and prospective study

封面

如何引用文章

全文:

详细

Background. Peripheral nerve sheath malignancies (PNSM) are a rare and aggressive group of sarcomas that can occur sporadically, after radiation therapy, or related to neurofibromatosis type 1. Loss of function of NF1, TP53, and CDKN2A genes is thought to contribute to the progression of benign neurofibroma to PNSM and is a poor prognostic sign. The optimal management of patients with this disease is currently unresolved, and specific prognostic factors have not been established.

Aim. To analyze the results of surgical treatment in patients with PNSM and establish prognostic factors of overall and recurrence-free survival rate.

Materials and methods. The retrospective and prospective studies enrolled adult patients with histologically confirmed PNSM treated between 1998 and 2021 at the N.N. Blokhin National Medical Research Center for Oncology. Surgical intervention was performed on 61 patients, 38 (62%) females and 23 (38%) males. The most common PNSM localization was paravertebral (22 [36%] patients), followed by retroperitoneal (14 [23%] patients), and upper and lower extremities (13 [21%] and 12 [20%] patients, respectively).

Results. For PNSM patients with a history of surgery, the median overall survival (OS) was 46 months (95% confidence interval 26.1–65.9). The 3-year and 5-year OS was 46% and 31%, respectively. The median recurrence-free survival (RFS) was 27 months (8.7–45.3), and 3-year and 5-year RFS was 26% and 13%, respectively. Median OS for grade 1 malignancies was not achieved, while grade 2 and grade 3 malignancy was 53 and 33 months, respectively (p=0.033). The median RFS for grade 1, 2, and 3 tumors was 119, 43, and 15 months, respectively (p=0.078). Patients who underwent radical (R0) surgery had higher RFS (p=0.006) and OS (p<0.0001). After radical (R0) surgery, the median OS was not achieved; after nonradical (R1/R2) surgery, the median was 34 months. The median RFS was 124 months after R0-resection and 48 months after R1/R2.

Conclusion. The most significant prognostic factors in PNSM patients are the radicality of the surgery performed and the malignancy grade. In our study, tumor size and localization did not affect the long-term treatment results.

作者简介

Adil Abdulzhaliev

Blokhin National Medical Research Center of Oncology

编辑信件的主要联系方式.
Email: AdilNGY@yandex.ru
ORCID iD: 0000-0001-9156-9822

Graduate Student

俄罗斯联邦, Moscow

Evgeny Sushentsov

Blokhin National Medical Research Center of Oncology

Email: crcspine@rambler.ru
ORCID iD: 0000-0003-3672-1742
SPIN 代码: 9946-5494

Cand. Sci. (Med.)

俄罗斯联邦, Moscow

Irena Boulytcheva

Blokhin National Medical Research Center of Oncology

Email: irena@boulytcheva.com
ORCID iD: 0000-0001-7592-4249

D. Sci. (Med.)

俄罗斯联邦, Moscow

Anastasia Senderovich

Central Clinical Hospital with Polyclinic

Email: a.senderovich@mail.ru
ORCID iD: 0000-0003-2213-5785
SPIN 代码: 4602-7378

Cand. Sci. (Med.)

俄罗斯联邦, Moscow

Maxim Nikulin

Blokhin National Medical Research Center of Oncology

Email: maximpetrovich@mail.ru
ORCID iD: 0000-0002-9608-4696
SPIN 代码: 9455-5566

Cand. Sci. (Med.)

俄罗斯联邦, Moscow

Denis Sofronov

Blokhin National Medical Research Center of Oncology

Email: mdsofronov@mail.ru
ORCID iD: 0000-0001-9557-3685
SPIN 代码: 1159-3665

Cand. Sci. (Med.)

俄罗斯联邦, Moscow

Vladislav Bugaev

Blokhin National Medical Research Center of Oncology

Email: vladbugaev@mail.ru
ORCID iD: 0000-0002-2410-7801
SPIN 代码: 7913-4919

Cand. Sci. (Med.)

俄罗斯联邦, Moscow

参考

  1. Hwang IK, Hahn SM, Kim HS, et al. Outcomes of treatment for malignant peripheral nerve sheath tumors: Different clinical features associated with neurofibromatosis type 1. Cancer Res Treat. 2017;49(3):717-26.
  2. 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):135-47.
  3. Casali PG, Abecassis N, Aro HT, et al. Soft tissue and visceral sarcomas: ESMO-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018;29:iv51-67.
  4. von Mehren M, Randall RL, Benjamin RS, et al. Soft tissue sarcoma, version 2.2018: Clinical practice guidelines in oncology. J Natl Compr Cancer Netw. 2018;16(5):536-63.
  5. Khu KJ, Midha R. Malignant Peripheral Nerve Sheath Tumors. World Neurosurg. 2016;94:566-7.
  6. 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.
  7. LaFemina J, Qin LX, Moraco NH, et al. Oncologic outcomes of sporadic, neurofibromatosis-associated, and radiation-induced malignant peripheral nerve sheath tumors. Ann Surg Oncol. 2013;20(1):66-72.
  8. Cai Z, Tang X, Liang H, et al. Prognosis and risk factors for malignant peripheral nerve sheath tumor: A systematic review and meta-analysis. World J Surg Oncol. 2020;18(1):1-12.
  9. James AW, Shurell E, Singh A, et al. Malignant Peripheral Nerve Sheath Tumor. Surg Oncol Clin N Am. 2016;25(4):789-802.
  10. Goertz O, Langer S, Uthoff D, et al. Diagnosis, treatment and survival of 65 patients with malignant peripheral nerve sheath tumors. Anticancer Res. 2014;34(2):777-84.
  11. Pervaiz N, Colterjohn N, Farrokhyar F, et al. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer. 2008;113(3):573-81.
  12. 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):1014-22.
  13. Mowery A, Clayburgh D. Malignant peripheral nerve sheath tumors: Analysis of the national cancer database. Oral Oncol. 2019;98:13-9.
  14. Hirbe AC, Gutmann DH. Neurofibromatosis type 1: A multidisciplinary approach to care. Lancet Neurol. 2014;13(8):834-43.
  15. 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.
  16. Stucky CCH, Johnson KN, Gray RJ, et al. Malignant Peripheral Nerve Sheath Tumors (MPNST): The Mayo Clinic experience. Ann Surg Oncol. 2012;19(3):878-85.
  17. Anghileri M, Miceli R, Fiore M, et al. Malignant peripheral nerve sheath tumors: Prognostic factors and survival in a series of patients treated at a single institution. Cancer. 2006;107(5):1065-74.
  18. Porter DE, Prasad V, Foster L, et al. Survival in malignant peripheral nerve sheath tumours: A comparison between sporadic and neurofibromatosis type 1-associated tumours. Sarcoma. 2009;2009:756395. doi: 10.1155/2009/756395
  19. Cashen DV, Parisien RC, Raskin K,et al. Survival data for patients with malignant schwannoma. Clin Orthop Relat Res. 2004;426(426):69-73.
  20. Martin E, Muskens IS, Coert JH, et al. Treatment and survival differences across tumor sites in malignant peripheral nerve sheath tumors: A SEER database analysis and review of the literature. Neuro-Oncology Pract. 2019;6(2):134-43.
  21. Yuan ZN, Xu LB, Zhao ZG, et al. Clinicopathological features and prognosis of malignant peripheral nerve sheath tumor: a retrospective study of 140 cases. Zhonghua Zhong Liu Za Zhi. 2017;39(6):439-44.
  22. Kim AR, Stewart DR, Reilly KM, et al. Malignant Peripheral Nerve Sheath Tumors State of the Science: Leveraging Clinical and Biological Insights into Effective Therapies. Sarcoma. 2017;2017:7429697. doi: 10.1155/2017/7429697
  23. 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.
  24. Desai KI. The surgical management of symptomatic benign peripheral nerve sheath tumors of the neck and extremities: An experience of 442 cases. Clin Neurosurg. 2017;81(4):568-80.
  25. Kim SM, Seo SW, Lee JY, Sung KS. Surgical outcome of Schwannomas arising from major peripheral nerves in the lower limb. Int Orthop. 2012;36(8):1721-5.
  26. Hajiabadi MM, Campos B, Sedlaczek O, et al. Interdisciplinary approach allows minimally invasive, nerve-sparing removal of retroperitoneal peripheral nerve sheath tumors. Langenbecks Arch Surg. 2020;405(2):199-205.
  27. Safaee MM, Lyon R, Barbaro NM, et al. Neurological outcomes and surgical complications in 221 spinal nerve sheath tumors. J Neurosurg Spine. 2017;26(1):103-11.
  28. Montano N, D'Alessandris QG, D'Ercole M, et al. Tumors of the peripheral nervous system: Analysis of prognostic factors in a series with long-term follow-up and review of the literature. J Neurosurg. 2016;125(2):363-71.
  29. Chen J, Zheng YF, Tang SC, et al. Long-term outcomes of surgical resection with or without adjuvant therapy for treatment of primary spinal peripheral primitive neuroectodermal tumors. Clin Neurol Neurosurg. 2018;175:25-33.
  30. Longhi A, Errani C, Magagnoli G, et al. High grade malignant peripheral nerve sheath tumors: Outcome of 62 patients with localized disease and review of the literature. J Chemother. 2010;22(6):413-8.

补充文件

附件文件
动作
1. JATS XML
下载
2. Fig. 1. Localization of the primary tumor (%); n.

下载 (43KB)
索引源数据
3. Fig. 2. Clinical manifestations (%).

下载 (45KB)
索引源数据
4. Fig. 3. Type of disease progression (n).

下载 (41KB)
索引源数据
5. Fig. 4. The number of R1/R2 resections depending on the location of the tumor (%).

下载 (53KB)
索引源数据
6. Fig. 5. OS and DFS depending on the radicality of surgical treatment.

下载 (58KB)
索引源数据
7. Fig. 6. OS and DFS depending on the location of the primary MPNST.

下载 (81KB)
索引源数据
8. Fig. 7. OS and DFS depending on the degree of tumor malignancy.

下载 (68KB)
索引源数据
9. Fig. 8. OS and DFS depending on the status of NF-1.

下载 (52KB)
索引源数据
10. Fig. 9. OS and DFS depending on the size of the tumor.

下载 (56KB)
索引源数据
11. Fig. 10. The effect of adjuvant chemotherapy on OS and DFS.

下载 (65KB)
索引源数据

版权所有 © Consilium Medicum, 2023

Creative Commons License
此作品已接受知识共享署名-非商业性使用-相同方式共享 4.0国际许可协议的许可。
 


##common.cookie##