Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1
ABSTRACT Objective In this study, we review the institution’s experience in treating malignant peripheral nerve sheath tumors (MPNSTs). A secondary aim was to compare outcomes between MPNSTs with and without neurofibromatosis type 1 (NF1). Methods Ninety-two patients with MPNSTs, over a period o...
Saved in:
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Thieme Revinter Publicações
|
| Series: | Arquivos de Neuro-Psiquiatria |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2017000600366&lng=en&tlng=en |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1849693806641807360 |
|---|---|
| author | Roberto André Torres de Vasconcelos Pedro Guimarães Coscarelli Regina Papais Alvarenga Marcus André Acioly |
| author_facet | Roberto André Torres de Vasconcelos Pedro Guimarães Coscarelli Regina Papais Alvarenga Marcus André Acioly |
| author_sort | Roberto André Torres de Vasconcelos |
| collection | DOAJ |
| description | ABSTRACT Objective In this study, we review the institution’s experience in treating malignant peripheral nerve sheath tumors (MPNSTs). A secondary aim was to compare outcomes between MPNSTs with and without neurofibromatosis type 1 (NF1). Methods Ninety-two patients with MPNSTs, over a period of 20 years, were reviewed. A retrospective chart review was performed. The median age was 43.5 years (range, 3–84 years) and 55.4% were female; 41 patients (44.6%) had NF1-associated tumors. Results Mean tumor sizes were 15.8 ± 8.2 cm and 10.8 ± 6.3 cm for patients with and without NF1, respectively. Combined two- and five-year overall survival was 48.5% and 29%. Multivariate analysis confirmed the association of tumor size greater than 10 cm (hazard ratio (HR) 2.99; 95% confidence interval (CI) 1.14–7.85; p = 0.0258) and presence of NF1 (HR 3.41; 95%CI 1.88–6.19; p < 0.001) with a decreased overall survival. Conclusion Tumor size and NF1 status were the most important predictors of overall survival in our population. |
| format | Article |
| id | doaj-art-4fa3d1867f8f4a42b8f586e39f938ddc |
| institution | DOAJ |
| issn | 1678-4227 |
| language | English |
| publisher | Thieme Revinter Publicações |
| record_format | Article |
| series | Arquivos de Neuro-Psiquiatria |
| spelling | doaj-art-4fa3d1867f8f4a42b8f586e39f938ddc2025-08-20T03:20:17ZengThieme Revinter PublicaçõesArquivos de Neuro-Psiquiatria1678-422775636637110.1590/0004-282x20170052S0004-282X2017000600366Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1Roberto André Torres de VasconcelosPedro Guimarães CoscarelliRegina Papais AlvarengaMarcus André AciolyABSTRACT Objective In this study, we review the institution’s experience in treating malignant peripheral nerve sheath tumors (MPNSTs). A secondary aim was to compare outcomes between MPNSTs with and without neurofibromatosis type 1 (NF1). Methods Ninety-two patients with MPNSTs, over a period of 20 years, were reviewed. A retrospective chart review was performed. The median age was 43.5 years (range, 3–84 years) and 55.4% were female; 41 patients (44.6%) had NF1-associated tumors. Results Mean tumor sizes were 15.8 ± 8.2 cm and 10.8 ± 6.3 cm for patients with and without NF1, respectively. Combined two- and five-year overall survival was 48.5% and 29%. Multivariate analysis confirmed the association of tumor size greater than 10 cm (hazard ratio (HR) 2.99; 95% confidence interval (CI) 1.14–7.85; p = 0.0258) and presence of NF1 (HR 3.41; 95%CI 1.88–6.19; p < 0.001) with a decreased overall survival. Conclusion Tumor size and NF1 status were the most important predictors of overall survival in our population.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2017000600366&lng=en&tlng=enneoplasias de bainha neuralneurofibromatose tipo 1sobrevivência |
| spellingShingle | Roberto André Torres de Vasconcelos Pedro Guimarães Coscarelli Regina Papais Alvarenga Marcus André Acioly Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1 Arquivos de Neuro-Psiquiatria neoplasias de bainha neural neurofibromatose tipo 1 sobrevivência |
| title | Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1 |
| title_full | Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1 |
| title_fullStr | Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1 |
| title_full_unstemmed | Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1 |
| title_short | Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1 |
| title_sort | malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1 |
| topic | neoplasias de bainha neural neurofibromatose tipo 1 sobrevivência |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2017000600366&lng=en&tlng=en |
| work_keys_str_mv | AT robertoandretorresdevasconcelos malignantperipheralnervesheathtumorwithandwithoutneurofibromatosistype1 AT pedroguimaraescoscarelli malignantperipheralnervesheathtumorwithandwithoutneurofibromatosistype1 AT reginapapaisalvarenga malignantperipheralnervesheathtumorwithandwithoutneurofibromatosistype1 AT marcusandreacioly malignantperipheralnervesheathtumorwithandwithoutneurofibromatosistype1 |