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

被引:0
作者
Xi, Xiaoxiao [1 ]
Cheng, Hui [1 ]
Huang, Shanyu [1 ]
Wang, Chen [1 ]
Ye, Mingzhu [1 ]
Sun, Xin [1 ]
机构
[1] Cent South Univ, Xiangya Hosp 3, Dept Obstet & Gynecol, Changsha, Peoples R China
关键词
malignant peripheral nerve sheath tumor; neurofibromatosis type 1; ganglioneuroma; rectovaginal septum; disorder of sex development; transvaginal surgery; SURVIVAL; MARKER;
D O I
10.3389/fonc.2025.1566385
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
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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页数:7
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