Clinical Analysis of Primary Primitive Neuroectodermal Tumors in the Female Genital Tract

Xiao, Changji MD*; Zhao, Jing MD*; Guo, Peng MD*; Wang, Dan MD*; Zhao, Dachun MD; Ren, Tong MD*; Yang, Jiaxin MD*; Shen, Keng MD*; Lang, Jinghe MD*; Xiang, Yang MD*; Cui, Quancai MD

International Journal of Gynecological Cancer: March 2014 - Volume 24 - Issue 3 - p 404–409
doi: 10.1097/IGC.0000000000000082
Review Articles

Objective: The aim of the study was to investigate the clinical manifestations, diagnosis, treatment, and prognosis of primitive neuroectodermal tumors (PNETs) in the female genital tract.

Methods: From April 2001 to May 2013, the clinicopathologic characteristics, treatments, outcomes, and prognosis of 11 patients with PNET in the female genital tract were analyzed retrospectively at our hospital.

Results: The location of PNET in the 11 patients presented here included vulva (2 patients), cervix (2 patients), uterus and its ligament (5 patients), and the ovaries (2 patients). Ages ranged from 18 to 59 years (median, 31 years).The main clinical manifestations of PNET in the female genital tract are irregular vaginal bleeding (6 patients), pelvic mass, uterine enlargement, and rapidly increasing vulvar mass (8 patients), and vulvar pain and lower abdominal pain (5 patients). The CA125 levels of 8 patients were elevated before the operations and reduced to normal when the diseases were controlled, while the levels increased as the tumor was progressive. Results for the most commonly used immunohistochemistry studies revealed CD99 in 11 of the 11 tumors, synaptophysin in 6 of the 7 positive tumors, and neuron-specific enolase in 6 of the 6 tumors. Ten patients underwent surgical resection. Nine of them underwent preoperative or/and postoperative combination chemotherapy. The follow-up of 10 patients were available and ranged from 1 to 145 months (median, 30.5 months), 3 of whom experiencing recurrence.

Conclusions: Primitive neuroectodermal tumor is very rare and can originate from any part of the female genital tract. The tumors had different manifestations but the same pathologic features. CA125 may be an important marker for prognosis and follow-up of PNET of the female internal genital tract.

Departments of *Obstetrics and Gynecology, and †Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People’s Republic of China.

Address correspondence and reprint requests to Yang Xiang, MD, Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Shuai Fu Yuan 1#, Dongcheng District, Beijing 100730, China. E-mail: xiangyang65@gmail.com.

This work was supported by a grant from the National Nature and Science Foundation of China (grant no. 81272890).

The authors declare no conflicts of interest.

Received October 1, 2013

Accepted December 2, 2013

© 2014 by the International Gynecologic Cancer Society and the European Society of Gynaecological Oncology.