Department of Neurosurgery, Seoul National University College of Medicine, Seoul National University Hospital, 101 Daehangno, Jongno-gu, Seoul 110-744, Republic of Korea.
J Neurooncol. 2010 Sep;99(2):227-36. doi: 10.1007/s11060-010-0119-7. Epub 2010 Jan 20.
Germinoma in the basal ganglia (BG) is notorious for its diagnostic difficulty. Clinical and radiological features of this disease are quite diverse, but have not been well characterized with respect to prognosis. We retrospectively reviewed the clinical course and treatment outcomes of 17 patients with a BG germinoma. The initial magnetic resonance imaging (MRI) features were classified. Clinical features and treatment outcomes were then analyzed with this classification scheme. A Type 1 lesion was defined as a subtle lesion with faint or no contrast enhancement (six patients). Type 2, 3, and 4 lesions were defined as contrast-enhancing lesions and were differentiated by the lesion size and the presence of subependymal seeding (11 patients). Type 1 lesions were distinct from the other lesions. Patients with a Type 1 lesion had a significantly longer time from the initial MRI to diagnosis than patients with Type 2, 3, and 4 lesions (P = 0.012). The actuarial progression-free survival and overall survival of patients 5 years after diagnosis were 66 and 77%, respectively. The presence of a Type 1 lesion (P = 0.004), a longer time delay in the diagnosis (P = 0.038), and radiation therapy without complete ventricular coverage (P = 0.010) were significantly associated with tumor progression. Profound motor deficits at diagnosis were associated with deterioration in motor function after tumor remission (P = 0.035). Early diagnosis of BG germinomas could affect the ability to control a tumor and neurological outcomes. In particular, high clinical suspicion and active diagnostic procedures are recommended. For optimal treatment, radiation fields should include entire ventricles even if there is no subependymal seeding.
基底节生殖细胞瘤（BG）的诊断极具难度，恶名昭著。该疾病的临床表现和影像学特征多种多样，但与预后相关的特征尚未得到充分的描述。我们回顾性分析了 17 例 BG 生殖细胞瘤患者的临床病程和治疗结局。对初始磁共振成像（MRI）特征进行分类。然后，根据该分类方案分析临床特征和治疗结局。将 1 型病变定义为具有微弱或无对比增强的细微病变（6 例）。2 型、3 型和 4 型病变定义为增强病变，并根据病变大小和室管膜下播散的存在进行区分（11 例）。1 型病变与其他病变明显不同。与 2 型、3 型和 4 型病变相比，1 型病变患者从初始 MRI 到诊断的时间明显更长（P = 0.012）。诊断后 5 年患者的无进展生存率和总生存率分别为 66%和 77%。存在 1 型病变（P = 0.004）、诊断时时间延迟较长（P = 0.038）和放疗未完全覆盖脑室（P = 0.010）与肿瘤进展显著相关。诊断时存在严重运动障碍与肿瘤缓解后运动功能恶化相关（P = 0.035）。早期诊断 BG 生殖细胞瘤可能会影响控制肿瘤和神经学结局的能力。因此，强烈建议提高临床警惕并积极进行诊断性检查。为了获得最佳治疗效果，即使没有室管膜下播散，放疗野也应包括整个脑室。
