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...

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Main Authors:	Goh, Kian Liang, Kiatisevi, Piya, Sukanthanak, Bhasanan, Piakong, Pongsiri
Format:	Conference or Workshop Item
Language:	English English
Published:	2018
Subjects:	RD701 Orthopedics
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

id	iium-66822
recordtype	eprints
spelling	iium-668222018-10-24T01:31:36Z http://irep.iium.edu.my/66822/ Malignant peripheral nerve sheath tumours: an outcome analysis of 32 patients in Lerdsin Hospital, Thailand Goh, Kian Liang Kiatisevi, Piya Sukanthanak, Bhasanan Piakong, Pongsiri RD701 Orthopedics 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. 2018-10-07 Conference or Workshop Item NonPeerReviewed application/pdf en http://irep.iium.edu.my/66822/1/Abstract%20oral.pdf application/pdf en http://irep.iium.edu.my/66822/2/Goh%20Kian%20Liang%201112-1118.pdf Goh, Kian Liang and Kiatisevi, Piya and Sukanthanak, Bhasanan and Piakong, Pongsiri (2018) Malignant peripheral nerve sheath tumours: an outcome analysis of 32 patients in Lerdsin Hospital, Thailand. In: 12th Asia Pacific Musculo Skeletal Tumor Society (APMSTS 2018), 4th-7th Oct. 2018, Fairmont Hotel, Jaipur, India. (Unpublished) http://apmsts2018.com/
repository_type	Digital Repository
institution_category	Local University
institution	International Islamic University Malaysia
building	IIUM Repository
collection	Online Access
language	English English
topic	RD701 Orthopedics
spellingShingle	RD701 Orthopedics Goh, Kian Liang Kiatisevi, Piya Sukanthanak, Bhasanan Piakong, Pongsiri Malignant peripheral nerve sheath tumours: an outcome analysis of 32 patients in Lerdsin Hospital, Thailand
description	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.
format	Conference or Workshop Item
author	Goh, Kian Liang Kiatisevi, Piya Sukanthanak, Bhasanan Piakong, Pongsiri
author_facet	Goh, Kian Liang Kiatisevi, Piya Sukanthanak, Bhasanan Piakong, Pongsiri
author_sort	Goh, Kian Liang
title	Malignant peripheral nerve sheath tumours: an outcome analysis of 32 patients in Lerdsin Hospital, Thailand
title_short	Malignant peripheral nerve sheath tumours: an outcome analysis of 32 patients in Lerdsin Hospital, Thailand
title_full	Malignant peripheral nerve sheath tumours: an outcome analysis of 32 patients in Lerdsin Hospital, Thailand
title_fullStr	Malignant peripheral nerve sheath tumours: an outcome analysis of 32 patients in Lerdsin Hospital, Thailand
title_full_unstemmed	Malignant peripheral nerve sheath tumours: an outcome analysis of 32 patients in Lerdsin Hospital, Thailand
title_sort	malignant peripheral nerve sheath tumours: an outcome analysis of 32 patients in lerdsin hospital, thailand
publishDate	2018
url	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
first_indexed	2023-09-18T21:34:53Z
last_indexed	2023-09-18T21:34:53Z
_version_	1777412754659868672

Malignant peripheral nerve sheath tumours: an outcome analysis of 32 patients in Lerdsin Hospital, Thailand

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