卵巢原始神经外胚层肿瘤一例并文献复习

doi:10.12280/gjszjk.20230035

摘要/Abstract

摘要：

发生在女性生殖系统的原始神经外胚层肿瘤（primitive neuroectodermal tumor，PNET）临床上罕见，目前尚无标准的治疗方案，预后差。报告1例极为罕见的卵巢PNET病例，以月经异常为主要症状，有子宫平滑肌瘤病史，血清肿瘤标志物未见明显升高，影像学未见明显异常。于2022年10月20日在气管插管全身麻醉下行腹腔镜下全子宫切除术+双侧输卵管切除术+双侧卵巢切除术，术后病理确诊为卵巢小圆细胞恶性肿瘤。术后给予长春新碱、多柔比星脂质体、环磷酰胺-异环磷酰胺、依托泊苷交替化疗5个周期，未见复发。总结该类罕见肿瘤的临床特点，旨在提高临床医生对该病的认识，从而早期明确诊断、探索有效的治疗，提高患者的生存率。

关键词: 神经外胚瘤，原始, 卵巢肿瘤, 治疗, 预后, 病例报告

Abstract:

The primitive neuroectodermal tumor (PNET) in female reproductive system is clinically rare. There is no standard treatment regimen, and the prognosis is very poor. We report one case of unusual ovarian PNET, menstrual abnormality was the main symptom. There was a history of uterine leiomyoma. The serum tumor markers were not significantly increased. Meanwhile, there was no abnormal imaging findings. On October 20, 2022, the laparoscopic total hysterectomy, bilateral salpingectomy and bilateral ovariectomy was given under endotracheal general anesthesia. Postoperative pathology confirmed the diagnosis of ovarian small round cell malignant tumor. After surgery, the alternating chemotherapy with vincristine, doxorubicin liposomes, cyclophosphamide-ifosfamide and etoposide was given 5 cycles and showed no recurrence. By summarizing the clinical characteristics of such a rare tumor, it aims to improve clinicians′ understanding of the disease, so as to provide early diagnosis and effective treatment, and to improve the survival of patients.

Key words: Neuroectodermal tumors, primitive, Ovarian neoplasms, Therapy, Prognosis, Case reports

安荣, 王晓慧. 卵巢原始神经外胚层肿瘤一例并文献复习[J]. 国际生殖健康/计划生育杂志, 2023, 42(5): 376-379.

AN Rong, WANG Xiao-hui. Ovarian Primitive Neuroectodermal Tumor: A Case Report and Literature Review[J]. Journal of International Reproductive Health/Family Planning, 2023, 42(5): 376-379.

图/表 2

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