Case Report: Malignant transformation of ganglioneuroma in the rectovaginal septum to malignant peripheral nerve sheath tumor based on neurofibromas
Malignant peripheral nerve sheath tumor (MPNST) is a type of soft tissue sarcoma that commonly occurs in the trunk, limbs, and head and neck regions, but rarely in the pelvic area. Nearly half of MPNST cases are secondary to neurofibromatosis type 1 (NF1). This case report discusses a 29-year-old wo...
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| Format: | Article |
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Frontiers Media S.A.
2025-06-01
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| Series: | Frontiers in Oncology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fonc.2025.1566385/full |
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| author | Xiaoxiao Xi Hui Cheng Shanyu Huang Chen Wang Mingzhu Ye Xin Sun |
| author_facet | Xiaoxiao Xi Hui Cheng Shanyu Huang Chen Wang Mingzhu Ye Xin Sun |
| author_sort | Xiaoxiao Xi |
| collection | DOAJ |
| description | Malignant peripheral nerve sheath tumor (MPNST) is a type of soft tissue sarcoma that commonly occurs in the trunk, limbs, and head and neck regions, but rarely in the pelvic area. Nearly half of MPNST cases are secondary to neurofibromatosis type 1 (NF1). This case report discusses a 29-year-old woman diagnosed with NF1 and pseudohermaphroditism, who presented with a large mass in the rectovaginal septum. Several years prior, a biopsy had identified the mass as a ganglioneuroma (GN). The mass was surgically removed via a vaginal approach, and postoperative pathology confirmed MPNST arising from NF1. In addition, this report provides a brief review of the relevant literature. |
| format | Article |
| id | doaj-art-32a6aadfb26e465ab793225cdf7d3ba2 |
| institution | OA Journals |
| issn | 2234-943X |
| language | English |
| publishDate | 2025-06-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Oncology |
| spelling | doaj-art-32a6aadfb26e465ab793225cdf7d3ba22025-08-20T02:36:01ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2025-06-011510.3389/fonc.2025.15663851566385Case Report: Malignant transformation of ganglioneuroma in the rectovaginal septum to malignant peripheral nerve sheath tumor based on neurofibromasXiaoxiao XiHui ChengShanyu HuangChen WangMingzhu YeXin SunMalignant peripheral nerve sheath tumor (MPNST) is a type of soft tissue sarcoma that commonly occurs in the trunk, limbs, and head and neck regions, but rarely in the pelvic area. Nearly half of MPNST cases are secondary to neurofibromatosis type 1 (NF1). This case report discusses a 29-year-old woman diagnosed with NF1 and pseudohermaphroditism, who presented with a large mass in the rectovaginal septum. Several years prior, a biopsy had identified the mass as a ganglioneuroma (GN). The mass was surgically removed via a vaginal approach, and postoperative pathology confirmed MPNST arising from NF1. In addition, this report provides a brief review of the relevant literature.https://www.frontiersin.org/articles/10.3389/fonc.2025.1566385/fullmalignant peripheral nerve sheath tumorneurofibromatosis type 1ganglioneuromarectovaginal septumdisorder of sex developmenttransvaginal surgery |
| spellingShingle | Xiaoxiao Xi Hui Cheng Shanyu Huang Chen Wang Mingzhu Ye Xin Sun Case Report: Malignant transformation of ganglioneuroma in the rectovaginal septum to malignant peripheral nerve sheath tumor based on neurofibromas Frontiers in Oncology malignant peripheral nerve sheath tumor neurofibromatosis type 1 ganglioneuroma rectovaginal septum disorder of sex development transvaginal surgery |
| title | Case Report: Malignant transformation of ganglioneuroma in the rectovaginal septum to malignant peripheral nerve sheath tumor based on neurofibromas |
| title_full | Case Report: Malignant transformation of ganglioneuroma in the rectovaginal septum to malignant peripheral nerve sheath tumor based on neurofibromas |
| title_fullStr | Case Report: Malignant transformation of ganglioneuroma in the rectovaginal septum to malignant peripheral nerve sheath tumor based on neurofibromas |
| title_full_unstemmed | Case Report: Malignant transformation of ganglioneuroma in the rectovaginal septum to malignant peripheral nerve sheath tumor based on neurofibromas |
| title_short | Case Report: Malignant transformation of ganglioneuroma in the rectovaginal septum to malignant peripheral nerve sheath tumor based on neurofibromas |
| title_sort | case report malignant transformation of ganglioneuroma in the rectovaginal septum to malignant peripheral nerve sheath tumor based on neurofibromas |
| topic | malignant peripheral nerve sheath tumor neurofibromatosis type 1 ganglioneuroma rectovaginal septum disorder of sex development transvaginal surgery |
| url | https://www.frontiersin.org/articles/10.3389/fonc.2025.1566385/full |
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