Ashrafganjoei Tahereh, Sourati Ainaz, Mohamadianamiri Mahdiss
Department of Obstetrics and Gynecology, Preventative Gynecology Research Center (PGRC), Imam Hossein Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Department of Radiotherapy and Oncology, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Int J Reprod Biomed. 2016 Dec;14(12):783-786.
Neuroendocine carcinoma of the gynecologic tract is rare and poses a significant clinical challenge because of tumor heterogeneity and lack of standardized guidelines for treatment. Ovotestis refers to the histology of a gonad that contains both ovarian follicles and testicular tubular elements. Ovotesticular disorder of sexual development occurs in fewer than 10% of all disorders of sexual development. Gonadal tumors with malignant potential occur in 2.6% of all cases of ovotesticular disorder of sexual development.
Here we represent a 77-year-old woman with primary amenorrhea, infertility and 10cm solid mass in left adnex with 46 XY in karyotype with ovotestis neuroendocrine neoplasm in pathology report which was treated with a multi-modality manner including surgery and chemotherapy but she came back with pulmonary metastasis after 2 cycles of chemotherapy. For women who present with a stage 1 primary ovarian neuroendocrine tumor the prognosis is excellent with greater than 90% survival. Neuroendocrine tumor of the ovary represents 3 % of all neuroendocrine tumors. The prevalence of ovotestis is 1/20000 births. For women with more advanced disease, the prognosis is poor. Neuroendocrine carcinoma of the ovary is a rare and aggressive tumor commonly associated with other surface epithelial and germ cell neoplasms. The prevalence of ovotestis is 1/20000 births and gonadal malignancies are the most reported neoplasm affected the ovotestis. Here we report a case of ovotestis which is presented with neuroendocrine carcinoma and poor prognosis.
Neuroendocrine carcinoma of the ovary is a rare and aggressive tumor commonly associated with other surface epithelial and germ cell neoplasms. The prevalence of ovotestis is rare and gonadal malignancies are the most reported neoplasm affected the ovotestis.
妇科神经内分泌癌较为罕见，由于肿瘤异质性以及缺乏标准化治疗指南，给临床带来了重大挑战。卵睾是指性腺的组织学表现，其中同时包含卵巢滤泡和睾丸管状结构。性发育异常中的卵睾性疾病在所有性发育异常疾病中所占比例不到10%。具有恶性潜能的性腺肿瘤在所有卵睾性性发育异常病例中占2.6%。
我们在此报告一例77岁女性，表现为原发性闭经、不孕，左侧附件区有一个10厘米的实性肿块，核型为46 XY，病理报告显示为卵睾神经内分泌肿瘤，接受了包括手术和化疗在内的多模式治疗，但化疗2个周期后出现肺转移。对于患有1期原发性卵巢神经内分泌肿瘤的女性，预后极佳，生存率超过90%。卵巢神经内分泌肿瘤占所有神经内分泌肿瘤的3%。卵睾的发病率为1/20000活产。对于病情更严重的女性，预后较差。卵巢神经内分泌癌是一种罕见且侵袭性强的肿瘤，通常与其他表面上皮和生殖细胞肿瘤相关。卵睾的发病率为1/20000活产，性腺恶性肿瘤是报道最多的累及卵睾的肿瘤。我们在此报告一例伴有神经内分泌癌且预后不良的卵睾病例。
卵巢神经内分泌癌是一种罕见且侵袭性强的肿瘤，通常与其他表面上皮和生殖细胞肿瘤相关。卵睾较为罕见，性腺恶性肿瘤是报道最多的累及卵睾的肿瘤。
