PDF(Pubmed)
Abstract：
Primitive neuroectodermal tumor (PNET) is a general term used in scientific literature for a heterogeneous group of small round-cell malignant tumors primarily arising from neural crest cells. These are extremely aggressive neoplasms which usually occur within soft tissue or bone of young adults. Ovarian tumors composed of primitive neuroectodermal elements are extremely rare, with only few case reports in scientific literature. Due to being so exceedingly rare, PNETs are frequently misdiagnosed and there are no standard therapeutic guidelines. Young patients seem to have better prognoses and individualized strategy is recommended. Limited data suggests that various gene deletions as well as amplifications may be crucial factors for tumorigenesis and the aggressive behavior of PNET. In this paper, we performed a brief review of all cases of primary ovarian PNETs published in the scientific literature to date, in regard to their clinical, histopathological, and therapeutic aspects, with the aim to provide a more comprehensive understanding of this exceedingly rare pathology.
摘要：
原始神经外胚层肿瘤（PNET）是科学文献中使用的通用术语，用于主要由神经c细胞引起的一组异质的小圆形细胞恶性肿瘤。这些是极具侵袭性的肿瘤，通常发生在年轻人的软组织或骨骼中。由原始神经外胚层成分组成的卵巢肿瘤极为罕见，科学文献中只有很少的病例报告。由于非常罕见，PNETs经常被误诊，并且没有标准的治疗指南。年轻患者的预后似乎更好，建议采用个性化策略。有限的数据表明，各种基因缺失以及扩增可能是肿瘤发生和PNET攻击行为的关键因素。在本文中,我们对迄今为止发表在科学文献中的所有原发性卵巢PNETs病例进行了简要回顾,关于他们的临床，组织病理学,和治疗方面，目的是对这种极其罕见的病理学有更全面的了解。
