Malignant peripheral nerve sheath tumours: an outcome analysis of 32 patients in Lerdsin Hospital, Thailand
Objectives Malignant peripheral nerve sheath tumours (MPNST) are rare soft tissue sarcoma which exhibit a biologically aggressive behaviour with propensity for rapid progression and high mortality rate. Our objectives were to identify the prognostic factors for local recurrence and distant metastasi...
| Main Authors: | , , , |
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| Format: | Conference or Workshop Item |
| Language: | English English |
| Published: |
2018
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| Subjects: | |
| Online Access: | http://irep.iium.edu.my/66822/ http://irep.iium.edu.my/66822/ http://irep.iium.edu.my/66822/1/Abstract%20oral.pdf http://irep.iium.edu.my/66822/2/Goh%20Kian%20Liang%201112-1118.pdf |
| Summary: | Objectives Malignant peripheral nerve sheath tumours (MPNST) are rare soft tissue sarcoma which exhibit a biologically aggressive behaviour with propensity for rapid progression and high mortality rate. Our objectives were to identify the prognostic factors for local recurrence and distant metastasis, to analyse the survival outcome and to identify the prognostic factors related to patients’ survival.
Methods We performed a retrospective review of 32 patients diagnosed with MPNST from 2009 to 2017 in Lerdsin Hospital. The mean age of presentation was 51 years old with 14 males and 18 females. The most common affected site was lower limb (56%) compared to upper limb (13%) and central location (31%). Thirty-one percent of patients had underlying neurofibromatosis type 1 (NF-1). Most patients had high grade tumour (81%) and mean tumour size was 12.2 cm (range, 1-25 cm). All except one patient underwent tumour excision whilst 31% had positive margin. Adjuvant treatment consisted of radiation (61%), chemotherapy (18%) or both (9%).
Results The local recurrence rate was 26% with a median survival time after surgery of 56 months (95% CI 31.7-79.5) and 5-year overall survival rate was 39%. High tumour grade and male patients were significantly associated with distant metastasis on univariate analysis but no predictors for local recurrence were identified. Age <45, high tumour grade, triton tumour subtype, diagnosis of NF-1, local recurrence, metastasis and use of adjuvant chemotherapy were all associated with disease specific survival (DSS). Multivariate analysis identified local recurrence (HR=20.035, 95% CI 1.8-222.8) as the single poor prognostic factor for DSS.
Conclusions High tumour grade, local recurrence and distant metastasis all adversely affects the survival in MPNST. However, adjuvant chemotherapy or radiotherapy did not appear to improve survival. Limitations of this study include retrospective design and small sample size. |
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