Malignant Peripheral Nerve Sheath Tumors in Children with Neurofibromatosis Type 1

Purpose. Malignant peripheral nerve sheath tumors (MPNSTs) are rare in children and account for approximately 5–10% of all soft tissue sarcomas in adults. MPNSTs may occur independently but individuals with neurofibromatosis type 1 (NF1) have a significantly increased risk. Our aim is to present pa...

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Main Authors:	Pourtsidis, Apostolos, Doganis, Dimitrios, Baka, Margarita, Bouhoutsou, Despina, Varvoutsi, Maria, Synodinou, Maria, Giamarelou, Panagiota, Kosmidis, Helen
Format:	Online
Language:	English
Published:	Hindawi Publishing Corporation 2014
Online Access:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4182008/

id	pubmed-4182008
recordtype	oai_dc
spelling	pubmed-41820082014-10-14 Malignant Peripheral Nerve Sheath Tumors in Children with Neurofibromatosis Type 1 Pourtsidis, Apostolos Doganis, Dimitrios Baka, Margarita Bouhoutsou, Despina Varvoutsi, Maria Synodinou, Maria Giamarelou, Panagiota Kosmidis, Helen Case Report Purpose. Malignant peripheral nerve sheath tumors (MPNSTs) are rare in children and account for approximately 5–10% of all soft tissue sarcomas in adults. MPNSTs may occur independently but individuals with neurofibromatosis type 1 (NF1) have a significantly increased risk. Our aim is to present patients with MPNST treated in our department. Cases and Results. In this report we present 4 cases of MPNSTs (3 females: 13, 12, and 13 years old and 1 male: 10 years old) arising in patients with NF1. All of them presented with an enlarging mass and pain at diagnosis. Tumor was located in the buttock, the spinal cord, the trunk, and the left leg proximal to the heel. Wide excision of the tumor and radiotherapy were applied to all and adjuvant chemotherapy was given to three of them after the disease was progressed. All four died 32, 18, 10, and 22 months after diagnosis with progressive disease locally and pulmonary metastases in two of them. Conclusions. In conclusion, MPNSTs arising in patients with NF1 are high grade sarcomas with short survival. Individuals with NF1 should be followed closely in order to identify early the development of MPNSTs. Aggressive surgery and complete excision significantly improves disease-free survival. The usefulness of radiation therapy in MPNSTs is not determined although all patients will receive radiation therapy at some stage of the disease. The role of chemotherapy is unclear. Hindawi Publishing Corporation 2014 2014-09-16 /pmc/articles/PMC4182008/ /pubmed/25317349 http://dx.doi.org/10.1155/2014/843749 Text en Copyright © 2014 Apostolos Pourtsidis et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
repository_type	Open Access Journal
institution_category	Foreign Institution
institution	US National Center for Biotechnology Information
building	NCBI PubMed
collection	Online Access
language	English
format	Online
author	Pourtsidis, Apostolos Doganis, Dimitrios Baka, Margarita Bouhoutsou, Despina Varvoutsi, Maria Synodinou, Maria Giamarelou, Panagiota Kosmidis, Helen
spellingShingle	Pourtsidis, Apostolos Doganis, Dimitrios Baka, Margarita Bouhoutsou, Despina Varvoutsi, Maria Synodinou, Maria Giamarelou, Panagiota Kosmidis, Helen Malignant Peripheral Nerve Sheath Tumors in Children with Neurofibromatosis Type 1
author_facet	Pourtsidis, Apostolos Doganis, Dimitrios Baka, Margarita Bouhoutsou, Despina Varvoutsi, Maria Synodinou, Maria Giamarelou, Panagiota Kosmidis, Helen
author_sort	Pourtsidis, Apostolos
title	Malignant Peripheral Nerve Sheath Tumors in Children with Neurofibromatosis Type 1
title_short	Malignant Peripheral Nerve Sheath Tumors in Children with Neurofibromatosis Type 1
title_full	Malignant Peripheral Nerve Sheath Tumors in Children with Neurofibromatosis Type 1
title_fullStr	Malignant Peripheral Nerve Sheath Tumors in Children with Neurofibromatosis Type 1
title_full_unstemmed	Malignant Peripheral Nerve Sheath Tumors in Children with Neurofibromatosis Type 1
title_sort	malignant peripheral nerve sheath tumors in children with neurofibromatosis type 1
description	Purpose. Malignant peripheral nerve sheath tumors (MPNSTs) are rare in children and account for approximately 5–10% of all soft tissue sarcomas in adults. MPNSTs may occur independently but individuals with neurofibromatosis type 1 (NF1) have a significantly increased risk. Our aim is to present patients with MPNST treated in our department. Cases and Results. In this report we present 4 cases of MPNSTs (3 females: 13, 12, and 13 years old and 1 male: 10 years old) arising in patients with NF1. All of them presented with an enlarging mass and pain at diagnosis. Tumor was located in the buttock, the spinal cord, the trunk, and the left leg proximal to the heel. Wide excision of the tumor and radiotherapy were applied to all and adjuvant chemotherapy was given to three of them after the disease was progressed. All four died 32, 18, 10, and 22 months after diagnosis with progressive disease locally and pulmonary metastases in two of them. Conclusions. In conclusion, MPNSTs arising in patients with NF1 are high grade sarcomas with short survival. Individuals with NF1 should be followed closely in order to identify early the development of MPNSTs. Aggressive surgery and complete excision significantly improves disease-free survival. The usefulness of radiation therapy in MPNSTs is not determined although all patients will receive radiation therapy at some stage of the disease. The role of chemotherapy is unclear.
publisher	Hindawi Publishing Corporation
publishDate	2014
url	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4182008/
_version_	1613139738385973248

Malignant Peripheral Nerve Sheath Tumors in Children with Neurofibromatosis Type 1

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