Nuclear Medicine Unit, Department of Endocrinology Department of Endocrinology, Oncological Endocrinology and Nuclear Medicine, University Hospital, Kraków, Poland.
Chair and Department of Endocrinology, Jagiellonian University Medical College, Kraków, Poland.
Front Endocrinol (Lausanne). 2021 Nov 30;12:770266. doi: 10.3389/fendo.2021.770266. eCollection 2021.
Neuroendocrine neoplasms are a heterogeneous group of cancers that develop from enterochromaffin cells of the diffuse endocrine system, with an increase in incidents over the last years. Ovarian neuroendocrine tumors (NET) are rare neoplasms, comprising 0.1% of all ovarian neoplasms and less than 5% of all neuroendocrine tumors. They may arise alone (as monodermal, specialized teratoma - ovarian carcinoid) or as a part of other ovarian lesion: cystic mature or immature teratomas. Due to the rarity and limited amount of such cases reported in the literature, there is no consensus on diagnostic and therapeutic procedures in this group of patients.
The group of 10 patients at the age of 19 to 77 years (mean 42.8 ± 17.9), diagnosed with unilateral NET within ovarian teratoma were analyzed. The histopathological type of tumor, progression free survival after surgical treatment and presence of hormonally active syndrome were assessed.
70% (n=7) of patients was diagnosed with mature cystic teratomas containing NET component and 30% (n=3) with monodermal teratoma (strumal carcinoid). All cases of monodermal teratomas were found in women at premenopausal age. Determined Ki67 ranged from 2% to 9%. Ninety percent of lesions (n=9) stained positive for synaptophysin and chromogranin, while markers: CK20, CK7, TTF-1 and CDX2 were negative in all cases, which ruled out their metastatic nature. None of the patients presented with carcinoid syndrome. All followed-up patients remain progression-free, which confirms surgical intervention being a crucial and sufficient method of treatment.
The prognosis and clinical behavior of NETs associated with ovarian teratomas are good with long progression-free survival.
神经内分泌肿瘤是一组起源于弥散内分泌系统肠嗜铬细胞的异质性癌症，近年来发病率有所增加。卵巢神经内分泌肿瘤（NET）是罕见的肿瘤，占所有卵巢肿瘤的 0.1%，不到所有神经内分泌肿瘤的 5%。它们可以单独发生（作为单胚层、特化的畸胎瘤-卵巢类癌），也可以作为其他卵巢病变的一部分：囊性成熟或未成熟畸胎瘤。由于此类病例在文献中的报道数量有限且罕见，因此对于这组患者，目前尚无诊断和治疗程序的共识。
分析了年龄在 19 至 77 岁之间（平均 42.8 ± 17.9）的 10 例单侧 NET 患者。评估了肿瘤的组织病理学类型、手术后无进展生存期以及是否存在激素活性综合征。
70%（n=7）的患者被诊断为含有 NET 成分的成熟囊性畸胎瘤，30%（n=3）为单胚层畸胎瘤（Strumal carcinoid）。所有单胚层畸胎瘤均发生在绝经前妇女中。测定的 Ki67 范围为 2%至 9%。90%的病变（n=9）对突触素和嗜铬粒蛋白呈阳性染色，而所有病例的标志物：CK20、CK7、TTF-1 和 CDX2 均为阴性，排除了其转移性。没有患者出现类癌综合征。所有随访患者均无进展，这证实了手术干预是一种关键且充分的治疗方法。
与卵巢畸胎瘤相关的 NET 预后和临床行为良好，无进展生存期长。
