Malignant peripheral nerve sheath tumours: an outcome analysis of 32 patients in Lerdsin Hospital, Thailand
Background 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. Currently, the data for this tumour is lacking especially with regards to the Asian population....
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
ResearchOne Publishing House
2018
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| Subjects: | |
| Online Access: | http://irep.iium.edu.my/65489/ http://irep.iium.edu.my/65489/ http://irep.iium.edu.my/65489/1/20181009_00001.pdf |
| Summary: | Background
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. Currently, the data for this tumour is lacking especially with regards to the Asian population.
Methods
A retrospective analysis was performed in 32 patients diagnosed with MPNST from 2009 to 2017 in Lerdsin Hospital. Prognostic factors such as patient demographic factors, tumour and treatment characteristics were evaluated for statistical significance. The mean age of presentation was 51 years old with 66% patients above 44 years old. Of the 32 patients, 14 were males and 18 were 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). The largest proportion of patients had high grade tumour (81%) and the mean size of tumour was 12.2 cm (range, 1-25 cm). All except one patient underwent tumour excision whilst 31% had positive margin. Adjuvant treatment was initiated in 50% of the patients which 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. Other associated factors include presence of NF-1 (HR=7.962, 95% CI 0.9-69.9) and high tumour grade (HR=16.945, 95% CI 0.9-321.8) although not reaching statistical significance.
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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