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 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.

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Publication Details

Journal
Frontiers in Oncology
Published
2025-06-19
DOI
https://doi.org/10.3389/fonc.2025.1566385
Citations
2
Primary Topic
Neurofibromatosis and Schwannoma Cases
Type
article
Field-Weighted Citation Impact
2.78
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article

Case Report: Malignant transformation of ganglioneuroma in the rectovaginal septum to malignant peripheral nerve sheath tumor based on neurofibromas

Mingzhu Ye, Shanyu Huang, Chen Wang, Hui Cheng et al.
2 citations
Frontiers in Oncology
Neurofibromatosis and Schwannoma Cases
2.78
article

Case Report: Malignant transformation of ganglioneuroma in the rectovaginal septum to malignant peripheral nerve sheath tumor based on neurofibromas

Mingzhu Ye, Shanyu Huang, Chen Wang, Hui Cheng, Xin Sun, Xiaoxiao Xi
article en
2 citations

Abstract

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.

Frontiers in OncologyVol. 15
Central South University (CN), Third Xiangya Hospital (CN)
Good health and well-being
Openalex Percentile: Top 10%
Neurofibromatosis and Schwannoma Cases
2.78
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