Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2006 Aug 22:4:55.
doi: 10.1186/1477-7819-4-55.

Malignant peripheral nerve sheath tumors (MPNST)--clinicopathological study and treatment outcome of twenty-four cases

Madhabananda Kar  1 , S V Suryanarayana Deo, Nootan Kumar Shukla, Ajay Malik, Sidharth DattaGupta, Bidhu Kumar Mohanti, Sanjay Thulkar
Affiliations

Malignant peripheral nerve sheath tumors (MPNST)--clinicopathological study and treatment outcome of twenty-four cases

Madhabananda Kar et al. World J Surg Oncol. .

Abstract

Background: Malignant peripheral nerve sheath tumor (MPNST) is biologically an aggressive tumor for which the treatment of choice is the surgery. We reviewed the clinical profile, diagnostic methods, treatment patterns, and outcome of twenty-four MPNST patients in this study.

Patients and methods: A retrospective analysis of 24 MPNST patients, treated from 1994 to 2002, in the department of Surgical Oncology at All India Institute of Medical Sciences, New Delhi, was done. A combination of gross, histopathological and immunohistochemical findings, and proliferation markers (MIB1) were considered for diagnosis and grade of the MPNST. Survival analysis was done by the Kaplan-Meier method and differences were evaluated with the log-rank test. Multivariate analysis was carried out by using Cox's proportional hazards model by using SPSS (Version 9, Chicago, Illinois) software.

Results: MPNST constituted 12% of all soft tissue sarcomas, where 21% (5/24) of patients had associated Von Recklinghausen's disease (VRHD). A higher incidence of male preponderance and multifocal MPNST were noted in the present series. At a mean follow-up of 38 months, 13 (54 %) patients had relapse of disease and 5-year over all and disease free survival were 58% and 35% respectively. In univariate analysis, sex (p = 0.05), tumor depth (p < 0.03), and cellular differentiation (p < 0.002) were shown to be adverse prognostic factors for disease free survival and sex (p = 0.04), cellular differentiation (p < 0.0004), and tumor grade (p = 0.05) for overall survival. However, in multivariate analysis, cellular differentiation (p < 0.005) and tumor grade (p < 0.01) emerged as independent prognostic factors for both disease free and overall survival, respectively. Postoperative radiotherapy (RT) has shown a definite role in both disease free and overall survival in this study.

Conclusion: MPNSTs constituted a significant proportion (12%) of soft tissue sarcoma in our medical center. Heterogeneous differentiation and multifocality of the tumor were few distinct features of MPNST. Sex and cellular differentiation were noticed as the new adverse prognostic factors and adjuvant radiotherapy has been proved to be a significant treatment tool in the current series.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Shows Kaplan Meier survival curve indicating disease free survival in months.
Figure 2
Figure 2
Shows Kaplan Meier survival curve indicating overall survival in months.
Figure 3
Figure 3
Shows Kaplan Meier Curve indicating the effect of postoperative radiotherapy on disease free survival.
Figure 4
Figure 4
Shows Kaplan Meier Curve indicating the effect of post operative radiotherapy on overall survival.
Figure 5
Figure 5
Kaplan Meier Curve indicating the impact of cellular differentiation on disease free survival.
Figure 6
Figure 6
Kaplan Meier Curve indicating the impact of cellular differentiation on overall survival.
Figure 7
Figure 7
Shows Kaplan Meier projected survival curve indicating the influence of relapse of the disease on overall survival of the patients.
See this image and copyright information in PMC

References

    1. Hruban RH, Shiu MH, Senie RT, Woodruff JM. Malignant peripheral nerve sheath tumors of the buttock and lower extremity A study of 43 cases. Cancer. 1990;66:1253–1265. doi: 10.1002/1097-0142(19900915)66:6<1253::AID-CNCR2820660627>3.0.CO;2-R. - DOI - PubMed
    1. Angelov L, Guha A. Peripheral Nerve Tumors. In: Berstein M, Berger MS, editor. Neuro oncology Essentials. 1. New York Theme Publishers; 2000. pp. 434–444.
    1. Wanebo JE, Malik JM, VandenBerg SR, Wanebo JH, Driesen N, Persing JA. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 28 cases. Cancer. 1993;71:1247–1253. doi: 10.1002/1097-0142(19930215)71:4<1247::AID-CNCR2820710413>3.0.CO;2-S. - DOI - PubMed
    1. Dasgupta TK, Choudhuri PK. Tumors of soft tissue. 2. Connecticut Appleton & Lange; 1998. pp. 127–395.
    1. Ducatman SB, Bernd WS, David GP, Herbert MR, Duane MI. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer. 1986;57:2006–2021. doi: 10.1002/1097-0142(19860515)57:10<2006::AID-CNCR2820571022>3.0.CO;2-6. - DOI - PubMed

LinkOut - more resources