A case series of patients with gonadal dysgenesis-associated mixed malignant ovarian germ cell tumor

Abstract

Introduction: Malignant germ cell tumors (MGCT) can occur in both genders. In this study, we present eight cases of mixed ovarian MGCT in patients. Most patients reported in the current study are young women, among whom clinical characteristics of gonadal dysgenesis associated MGCT were rarely reported.

Methods: Comprehensive information of eight patients with mixed ovarian MGCTs, including patients’ age, clinical features, tumor markers, imaging findings, surgical records, pathology, karyotyping tests, chemotherapy and follow-up were collected. Surgical specimens were evaluated by two specialized gynecologic pathologists.

Results: All patients received surgery, while seven received chemotherapy. Among them, two received a second surgery and three patients received hormone replacement therapy (HRT) after gonadectomy. Four of five patients with amenorrhea were found to have 46, XY karyotype. All patients showed no sign of recurrence at the latest follow-up.

Discussion: Karyotyping or genetics testing in patients with amenorrhea is necessary, especially for patients with pelvic mass, which can help surgeons to evaluate the necessity of gonadectomy before surgery. The patients with gonadal dysgenesis associated mixed ovarian MGCT seem to have better prognosis and long survival time. Thus, HRT, an option that can improve life quality, is worth considering for these patients after gonadectomy.

Chinese abstract

目的：恶性生殖细胞肿瘤(MGCT)在男性和女性中均有发生。在这项研究中, 我们报道了8例混合型卵巢MGCT患者。目前研究中的大多数患者是年轻女性, 其中与MGCT相关的性腺发育不全的临床特点很少报道。

方法：收集8例混合型卵巢MGCT患者的年龄、临床特征、肿瘤标志物、影像学表现、手术记录、病理、核型分析、化疗及随访等综合资料。手术标本由两位专业的妇科病理学家进行评估。

结果：所有患者都进行了手术治疗, 其中7名患者进行了化疗。两名患者进行了第二次手术, 三名患者在性腺切除后接受了激素替代疗法(HRT)。5例闭经患者中有4例染色体核型为46, XY。所有患者在近期的随访中都没有复发的表现。

讨论：对闭经患者, 特别是对有盆腔肿块患者进行染色体核型或遗传学检测是有必要的, 它可以帮助医生在手术前评估性腺切除的必要性。性腺发育不全合并混合型卵巢MGCT患者预后较好, 生存期较长。因此, 对于这些性腺切除术后的患者来说, HRT可以改善生活质量, 是一个值得考虑的治疗方法。

Keywords:

• Mixed malignant germ cell tumors
• gonadal dysgenesis
• gonadectomy
• hormone replacement therapy

Disclosure statement

The authors report that they have no conflicts of interest.

Author contributions

Yu Z was responsible for the design of the research, conceptualization and final approval of the manuscript. YT was responsible for the design of the research and final approval of the manuscript. WY was responsible for the design of the research, data analysis and drafting. LW reviewed the manuscript. QH was responsible for pathologic testing and diagnosis. Yi Z was responsible for treatment strategy and management.

Additional information

Funding

This study was supported by Natural Science Foundation of Hunan Province of China [No. 2018JJ3857]. The authors have not declared other specific grants for this research from any funding agency in the public, commercial or not-for-profit sectors.