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Sporadic malignant peripheral nerve sheath tumour in a 3-year-old girl: A diagnostic challenge

Introduction: Malignant peripheral nerve sheath tumour (MPNST) is an uncommon malignant neoplasm of childhood with unfavourable prognosis. Only 1.7% of the cases have been reported in children less than five years of age and approximately one-half arise from a benign peripheral nerve sheath tumour e...

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Main Authors: Mustapar, Nurulhasanah, Ismail, Ismaliza, Wan Sulaiman, Wan Azman, Wan Ismail, Wan Faisham Nu’man, Tuan Sharif, Sharifah Emilia
Format: Conference or Workshop Item
Language:English
Published: Malaysian Journal of Pathology 2018
Subjects:
RB Pathology
Online Access:http://irep.iium.edu.my/97297/7/97297_Sporadic%20malignant%20peripheral%20nerve%20sheath%20tumour%20in%20a%203-year-old%20girl.pdf
http://irep.iium.edu.my/97297/
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spelling my.iium.irep.972972022-04-04T03:38:10Z http://irep.iium.edu.my/97297/ Sporadic malignant peripheral nerve sheath tumour in a 3-year-old girl: A diagnostic challenge Mustapar, Nurulhasanah Ismail, Ismaliza Wan Sulaiman, Wan Azman Wan Ismail, Wan Faisham Nu’man Tuan Sharif, Sharifah Emilia RB Pathology Introduction: Malignant peripheral nerve sheath tumour (MPNST) is an uncommon malignant neoplasm of childhood with unfavourable prognosis. Only 1.7% of the cases have been reported in children less than five years of age and approximately one-half arise from a benign peripheral nerve sheath tumour especially in background of neurofibromatosis type 1 (NF1). Primary MPNST in children are even rarer. Case Report: A 3-year-old Malay girl presented with painful right axillary swelling for 6 months duration, initially treated as axillary lymphadenitis and she defaulted follow up. She came back 4 months later with enlargement of the swelling. The biopsy was reported as Schwannoma which correlates with MRI findings of benign peripheral nerve sheath tumour. Final diagnosis after debulking surgery was consistent with MPNST. She succumbed to death 20 months after her initial diagnosis of advanced MPNST and lung metastasis. Pathological findings: Grossly, a huge partlycircumscribed soft tissue mass noted arising from a nerve with solid greyish yellowish myxoid cut surface. Spindle shaped cells arranged in herringbone pattern with marked pleomorphism, brisk mitosis and extensive necrosis are seen microscopically. Immunohistochemistry shows patchy S100 protein staining with loss of expression of H3K27 trimethylation. Conclusion: Although MPNST is rare in paediatric age group, diagnosis should be considered in children without NF1 with rapidly evolving and painful mass in the distribution of a peripheral nerve. In this case, the diagnosis was delayed and made after surgery. Due to its morphologic heterogeneity and lack of specific immunohistochemical markers, MPNST remains a diagnostic challenge. Malaysian Journal of Pathology 2018-12 Conference or Workshop Item NonPeerReviewed application/pdf en http://irep.iium.edu.my/97297/7/97297_Sporadic%20malignant%20peripheral%20nerve%20sheath%20tumour%20in%20a%203-year-old%20girl.pdf Mustapar, Nurulhasanah and Ismail, Ismaliza and Wan Sulaiman, Wan Azman and Wan Ismail, Wan Faisham Nu’man and Tuan Sharif, Sharifah Emilia (2018) Sporadic malignant peripheral nerve sheath tumour in a 3-year-old girl: A diagnostic challenge. In: The 5th International Academy of Pathology Malaysian Division, Annual Scientific Meeting, 27th - 28th October 2018, Shangri-la’s Tanjung Aru Resort and Spa, Kota Kinabalu, Sabah. http://www.mjpath.org.my/2018/v40n3/index.php
institution Universiti Islam Antarabangsa Malaysia
building IIUM Library
collection Institutional Repository
continent Asia
country Malaysia
content_provider International Islamic University Malaysia
content_source IIUM Repository (IREP)
url_provider http://irep.iium.edu.my/
language English
topic RB Pathology
spellingShingle RB Pathology
Mustapar, Nurulhasanah
Ismail, Ismaliza
Wan Sulaiman, Wan Azman
Wan Ismail, Wan Faisham Nu’man
Tuan Sharif, Sharifah Emilia
Sporadic malignant peripheral nerve sheath tumour in a 3-year-old girl: A diagnostic challenge
description Introduction: Malignant peripheral nerve sheath tumour (MPNST) is an uncommon malignant neoplasm of childhood with unfavourable prognosis. Only 1.7% of the cases have been reported in children less than five years of age and approximately one-half arise from a benign peripheral nerve sheath tumour especially in background of neurofibromatosis type 1 (NF1). Primary MPNST in children are even rarer. Case Report: A 3-year-old Malay girl presented with painful right axillary swelling for 6 months duration, initially treated as axillary lymphadenitis and she defaulted follow up. She came back 4 months later with enlargement of the swelling. The biopsy was reported as Schwannoma which correlates with MRI findings of benign peripheral nerve sheath tumour. Final diagnosis after debulking surgery was consistent with MPNST. She succumbed to death 20 months after her initial diagnosis of advanced MPNST and lung metastasis. Pathological findings: Grossly, a huge partlycircumscribed soft tissue mass noted arising from a nerve with solid greyish yellowish myxoid cut surface. Spindle shaped cells arranged in herringbone pattern with marked pleomorphism, brisk mitosis and extensive necrosis are seen microscopically. Immunohistochemistry shows patchy S100 protein staining with loss of expression of H3K27 trimethylation. Conclusion: Although MPNST is rare in paediatric age group, diagnosis should be considered in children without NF1 with rapidly evolving and painful mass in the distribution of a peripheral nerve. In this case, the diagnosis was delayed and made after surgery. Due to its morphologic heterogeneity and lack of specific immunohistochemical markers, MPNST remains a diagnostic challenge.
format Conference or Workshop Item
author Mustapar, Nurulhasanah
Ismail, Ismaliza
Wan Sulaiman, Wan Azman
Wan Ismail, Wan Faisham Nu’man
Tuan Sharif, Sharifah Emilia
author_facet Mustapar, Nurulhasanah
Ismail, Ismaliza
Wan Sulaiman, Wan Azman
Wan Ismail, Wan Faisham Nu’man
Tuan Sharif, Sharifah Emilia
author_sort Mustapar, Nurulhasanah
title Sporadic malignant peripheral nerve sheath tumour in a 3-year-old girl: A diagnostic challenge
title_short Sporadic malignant peripheral nerve sheath tumour in a 3-year-old girl: A diagnostic challenge
title_full Sporadic malignant peripheral nerve sheath tumour in a 3-year-old girl: A diagnostic challenge
title_fullStr Sporadic malignant peripheral nerve sheath tumour in a 3-year-old girl: A diagnostic challenge
title_full_unstemmed Sporadic malignant peripheral nerve sheath tumour in a 3-year-old girl: A diagnostic challenge
title_sort sporadic malignant peripheral nerve sheath tumour in a 3-year-old girl: a diagnostic challenge
publisher Malaysian Journal of Pathology
publishDate 2018
url http://irep.iium.edu.my/97297/7/97297_Sporadic%20malignant%20peripheral%20nerve%20sheath%20tumour%20in%20a%203-year-old%20girl.pdf
http://irep.iium.edu.my/97297/
http://www.mjpath.org.my/2018/v40n3/index.php
_version_ 1729702917946998784
score 13.211869

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