CASE REPORT article
Front. Oncol.
Sec. Gynecological Oncology
Volume 15 - 2025 | doi: 10.3389/fonc.2025.1566385
This article is part of the Research TopicAdvancements in Diagnostic and Management Strategies for Gynecological PathologiesView all 17 articles
Case Report: Malignant Transformation of Ganglioneuroma in the Rectovaginal Septum to Malignant Peripheral Nerve Sheath Tumor Based on Neurofibromas
Provisionally accepted- Third Xiangya Hospital, Central South University, Changsha, China
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Malignant peripheral nerve sheath tumor (MPNST) is a type of soft tissue sarcoma that commonly 10occurs in the trunk, limbs, and head and neck regions, but rarely in the pelvic area. Nearly half of 11MPNST cases are secondary to neurofibromatosis type 1 (NF1). This case report discusses a 29-year- 12old woman diagnosed with NF1 and pseudohermaphroditism, who presented with a large mass in the 13rectovaginal septum. Several years prior, a biopsy had identified the mass as a ganglioneuroma (GN). 14The mass was surgically removed via a vaginal approach, and postoperative pathology confirmed 15MPNST arising from NF1. In addition, this report provides a brief review of the relevant literature.
Keywords: Malignant peripheral nerve sheath tumor, neurofibromatosis type 1, Ganglioneuroma, Rectovaginal septum, disorder of sex development
Received: 24 Jan 2025; Accepted: 03 Jun 2025.
Copyright: © 2025 Xi, Cheng, Huang, Wang, Ye and Sun. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Xin Sun, Third Xiangya Hospital, Central South University, Changsha, China
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