Division of Medical Oncology, Fondazione IRCCS Policlinico San Matteo and University of Pavia, Italy.
Division of Medical Oncology, Fondazione IRCCS Policlinico San Matteo and University of Pavia, Italy.
Semin Oncol. 2019 Apr;46(2):107-111. doi: 10.1053/j.seminoncol.2019.04.001. Epub 2019 Apr 24.
Germ cell tumors (GCTs) are a group of neoplasms usually arising in the gonads, and very rarely in sites outside the gonads, mainly in the retroperitoneum, the anterior mediastinum, and pineal gland or the coccyx (mainly in childhood). The prognosis of nonseminoma primary mediastinal GCTs (PMGCTs), is considered to be poorer compared to its retroperitoneal or gonadal counterparts and, according to the International Germ Cell Cancer Collaborative Group, it is considered by definition as a "poor risk" disease.
Our review highlights the clinical features, prognostic factors, and therapeutic strategies in PMGCTs, as described in medical literature. So far available data were obtained through a Medline search of English-language papers.
Due to the rarity of the disease, there are no prospective studies comparing different treatment strategies. For this reason, oncology societies recommend treating PMGCTs like other GCTs, according to general risk categories.
Because of the rarity of the disease, poor categorization, and the lack of novel therapeutic strategies, an International database is required to obtain more information on these tumors. Additional efforts should be done, with the aims to find effective novel therapeutic agents.
生殖细胞肿瘤（GCTs）是一组通常发生在性腺的肿瘤，非常罕见于性腺外部位，主要位于腹膜后、前纵隔、松果体或尾骨（主要在儿童中）。与腹膜后或性腺来源的生殖细胞肿瘤相比，非精原细胞瘤性原发性纵隔生殖细胞肿瘤（PMGCTs）的预后被认为更差，并且根据国际生殖细胞癌协作组的定义，它被认为是一种“不良风险”疾病。
我们的综述强调了 PMGCTs 的临床特征、预后因素和治疗策略，这些内容在医学文献中有描述。迄今为止，通过对英文文献的 Medline 搜索获得了现有数据。
由于该疾病罕见，因此没有比较不同治疗策略的前瞻性研究。出于这个原因，肿瘤学会建议根据一般风险类别，像治疗其他 GCTs 一样治疗 PMGCTs。
由于该疾病罕见、分类不良以及缺乏新的治疗策略，因此需要建立一个国际数据库以获取更多关于这些肿瘤的信息。应做出更多努力，旨在寻找有效的新型治疗药物。
