Kuc-Rajca Małgorzata, Dańska-Bidzińska Anna
Klinika Nowotworów Narzadów Płciowych Kobiecych, Centrum Onkologii--Instytut im. M. Skłodowskiej-Curie w Warszawie, Polska.
Ginekol Pol. 2011 Sep;82(9):685-9.
Neuroendocrine neoplasms are a rare and heterogeneous group of diseases that account for only 2% of all gynecologic malignancies. The most common types are ovarian carcinoid tumor and small cell neuroendocrine carcinoma of the cervix. The tumors are staged according to FIGO clinical staging system. The diagnosis is usually made retrospectively after obtaining the results of histopathological evaluation of the primary tumor They rarely cause syndromes related to hormone overexpression. Neuroendocrine neoplasms are characterized by aggressive behaviour Even at an early stage there is high incidence of nodal and distant metastases. Survival is poor regardless of stage at diagnosis. The most important is to diagnose the neuroendocrine tumor accurately and treat it in multimodal, aggressive approach to control the disease better and reduce the incidence of reccurences. Apart from typical therapeutic approach, treatment may encompass isotope therapy using radiolabeled somatostatin analogs. This method should be reserved for patients with expression of somatostatin receptors detected by the somatostatin receptor scyntygraphy. Data concerning the management of neuroendocrin tumors are based mainly on retrospective studies and clinical case series. Lack of randomized trials makes it impossible to select the best treatment option. Better understanding of the biology of neuroendocrine tumors, especially the molecular genetics, will in the future help to determine the optimal treatment strategies for these tumors.
神经内分泌肿瘤是一类罕见且异质性的疾病,仅占所有妇科恶性肿瘤的2%。最常见的类型是卵巢类癌肿瘤和宫颈小细胞神经内分泌癌。这些肿瘤根据国际妇产科联盟(FIGO)临床分期系统进行分期。诊断通常在获得原发性肿瘤的组织病理学评估结果后回顾性做出。它们很少引起与激素过度表达相关的综合征。神经内分泌肿瘤具有侵袭性,即使在早期,淋巴结转移和远处转移的发生率也很高。无论诊断时处于何阶段,生存率都很低。最重要的是准确诊断神经内分泌肿瘤,并采用多模式、积极的方法进行治疗,以更好地控制疾病并降低复发率。除了典型的治疗方法外,治疗可能包括使用放射性标记的生长抑素类似物进行同位素治疗。这种方法应仅用于通过生长抑素受体闪烁显像检测到生长抑素受体表达的患者。关于神经内分泌肿瘤管理的数据主要基于回顾性研究和临床病例系列。缺乏随机试验使得无法选择最佳治疗方案。更好地了解神经内分泌肿瘤的生物学特性,尤其是分子遗传学,未来将有助于确定这些肿瘤的最佳治疗策略。