AUTHOR=Xi Xiaoxiao , Cheng Hui , Huang Shanyu , Wang Chen , Ye Mingzhu , Sun Xin 

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

JOURNAL=Frontiers in Oncology

VOLUME=Volume 15 - 2025

YEAR=2025

URL=https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2025.1566385

DOI=10.3389/fonc.2025.1566385

ISSN=2234-943X

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.