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Primary Intraosseous Malignant Peripheral Nerve Sheath Tumor of the Humerus: Report of a Rare Case
Malignant peripheral nerve sheath tumors (MPNSTs) usually arise in the soft tissues. Intraosseous MPNSTs are rare. They may arise de novo but are typically associated with neurofibromatosis type 1 (NF1) and radiation therapy. Our patient is a 58-year-old female patient that presented with right shou...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9886069/ https://www.ncbi.nlm.nih.gov/pubmed/36726883 http://dx.doi.org/10.7759/cureus.33178 |
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author | Ortiz, Wendolin J Salazar, Maria S Eager, John J Sajid, Saleha Cervantes, Mario |
author_facet | Ortiz, Wendolin J Salazar, Maria S Eager, John J Sajid, Saleha Cervantes, Mario |
author_sort | Ortiz, Wendolin J |
collection | PubMed |
description | Malignant peripheral nerve sheath tumors (MPNSTs) usually arise in the soft tissues. Intraosseous MPNSTs are rare. They may arise de novo but are typically associated with neurofibromatosis type 1 (NF1) and radiation therapy. Our patient is a 58-year-old female patient that presented with right shoulder pain. An MRI showed a shoulder mass, and percutaneous bone biopsy demonstrated morphology suggestive of an MPNST; besides, on immunohistochemistry, SOX10 was positive, and H3K27me3 expression was entirely lost. The patient underwent total resection of the right proximal humerus and endoprosthetic shoulder reconstruction, followed by radiation therapy and chemotherapy. Only a few cases in the mandible, spine, maxilla, ulna, metacarpal, tarsal, and one in the humerus have been published. In this paper, we contribute with an additional case of primary intraosseous MPNST in the humerus and a brief literature review. |
format | Online Article Text |
id | pubmed-9886069 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Cureus |
record_format | MEDLINE/PubMed |
spelling | pubmed-98860692023-01-31 Primary Intraosseous Malignant Peripheral Nerve Sheath Tumor of the Humerus: Report of a Rare Case Ortiz, Wendolin J Salazar, Maria S Eager, John J Sajid, Saleha Cervantes, Mario Cureus Pathology Malignant peripheral nerve sheath tumors (MPNSTs) usually arise in the soft tissues. Intraosseous MPNSTs are rare. They may arise de novo but are typically associated with neurofibromatosis type 1 (NF1) and radiation therapy. Our patient is a 58-year-old female patient that presented with right shoulder pain. An MRI showed a shoulder mass, and percutaneous bone biopsy demonstrated morphology suggestive of an MPNST; besides, on immunohistochemistry, SOX10 was positive, and H3K27me3 expression was entirely lost. The patient underwent total resection of the right proximal humerus and endoprosthetic shoulder reconstruction, followed by radiation therapy and chemotherapy. Only a few cases in the mandible, spine, maxilla, ulna, metacarpal, tarsal, and one in the humerus have been published. In this paper, we contribute with an additional case of primary intraosseous MPNST in the humerus and a brief literature review. Cureus 2022-12-31 /pmc/articles/PMC9886069/ /pubmed/36726883 http://dx.doi.org/10.7759/cureus.33178 Text en Copyright © 2022, Ortiz et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Pathology Ortiz, Wendolin J Salazar, Maria S Eager, John J Sajid, Saleha Cervantes, Mario Primary Intraosseous Malignant Peripheral Nerve Sheath Tumor of the Humerus: Report of a Rare Case |
title | Primary Intraosseous Malignant Peripheral Nerve Sheath Tumor of the Humerus: Report of a Rare Case |
title_full | Primary Intraosseous Malignant Peripheral Nerve Sheath Tumor of the Humerus: Report of a Rare Case |
title_fullStr | Primary Intraosseous Malignant Peripheral Nerve Sheath Tumor of the Humerus: Report of a Rare Case |
title_full_unstemmed | Primary Intraosseous Malignant Peripheral Nerve Sheath Tumor of the Humerus: Report of a Rare Case |
title_short | Primary Intraosseous Malignant Peripheral Nerve Sheath Tumor of the Humerus: Report of a Rare Case |
title_sort | primary intraosseous malignant peripheral nerve sheath tumor of the humerus: report of a rare case |
topic | Pathology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9886069/ https://www.ncbi.nlm.nih.gov/pubmed/36726883 http://dx.doi.org/10.7759/cureus.33178 |
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