Mesquita Filho Paulo Moacir, Santos Fabio Pires, Köhler Luiza Rech, Manfroi Gregori, De Carli Fernanda, Augusto de Araujo Moises, Schwingel Daniela
Department of Neurosurgery, Hospital da Cidade de Passo Fundo, Passo Fundo, Brazil; Neurology and Neurosurgery Service (SNN), Passo Fundo, Brazil; Academic League of SNN (LASNN), Passo Fundo, Brazil; Medical School, University of Passo Fundo, Passo Fundo, Brazil.
Department of ENT Surgery, Hospital da Cidade de Passo Fundo, Passo Fundo, Brazil; Medical School, University of Passo Fundo, Passo Fundo, Brazil.
World Neurosurg. 2018 Sep;117:165-171. doi: 10.1016/j.wneu.2018.06.041. Epub 2018 Jun 15.
Germinomas are rare malignant central nervous system tumors, a type of germ cell tumor, according to the 2016 World Health Organization Classification of Brain Tumors. Most of these tumors develop along the midline, most often from the pineal gland, followed by tumors arising in the suprasellar cisterns. Suprasellar germinomas commonly manifest with diabetes insipidus, visual impairment, and hypothalamic-pituitary failure. We present a literature review from the PubMed database and report 2 cases of suprasellar germinomas.
Both pediatric patients presented with progressive visual loss; one did not show endocrinologic impairment, and the other presented with hypothyroidism, diabetes insipidus, and generalized edema. Magnetic resonance imaging showed expansive suprasellar masses in both cases, confirmed as germinomas at histopathologic examination. The patients were treated with the same surgical approach, although their outcomes were different because of endocrinologic and post surgical complications.
Suprasellar germinomas are highly curable lesions when diagnosed early. The treatment remains controversial and should be individualized, but the association of chemotherapy and radiotherapy allows reduce adverse effects and shows great results.
根据2016年世界卫生组织脑肿瘤分类,生殖细胞瘤是罕见的恶性中枢神经系统肿瘤,属于生殖细胞肿瘤的一种。这些肿瘤大多沿中线发展,最常见于松果体,其次是鞍上池内发生的肿瘤。鞍上生殖细胞瘤通常表现为尿崩症、视力障碍和下丘脑 - 垂体功能衰竭。我们对来自PubMed数据库的文献进行了综述,并报告了2例鞍上生殖细胞瘤病例。
两名儿科患者均出现进行性视力丧失;其中一名未表现出内分泌功能损害,另一名则出现甲状腺功能减退、尿崩症和全身水肿。磁共振成像显示两例患者均有鞍上占位性病变,组织病理学检查确诊为生殖细胞瘤。尽管由于内分泌和术后并发症,两名患者的治疗结果不同,但他们接受了相同的手术方法。
鞍上生殖细胞瘤早期诊断时具有高度可治愈性。治疗仍存在争议,应个体化,但化疗和放疗联合使用可减少不良反应并取得良好效果。