Abstract

BACKGROUND

Intracranial germinoma is a rare malignant neoplasm, accounting for approximately two-thirds of central nervous system germ cell tumors, which occurs in the suprasellar region, pineal gland, or basal nuclei in children and young adolescents. Due to the rare occurrence, there is relatively high variability in treatment modalities. This article aims to assess the symptoms, and treatment outcomes of radiotherapy and chemotherapy for intracranial germinoma, based on tertiary center experience.

METHODS

The study includes retrospectively gathered data from 35 consecutive patients treated for intracranial germinoma between 1999–2020 at a single institution. The statistical analysis used basic statistical tools and the Kaplan-Meier method.

RESULTS

The median age at diagnosis was 17 years (IQR 14–22), 85.7% of the patients were male. The majority of the patients had histopathological confirmation of the diagnosis (88.5%), 76% of which were non-secreting germinomas. The primary tumor was most frequently localized in the area of the pineal gland (34.3%), followed by bifocal (25.7%), suprasellar (17.1%), and basal germinoma (8.6%). The remaining patients had disseminated disease (14.3%). The most common symptoms at the onset of the disease were headache (54.3%), visual impairment (45.7%), vomiting (25.7%), polydipsia (22.9%), weight loss (17.1%), or minor neurological deficits (11.4%). As much as 54.3% of the patients presented with neuroendocrine disorders at diagnosis, and 42.9% of the patients presented radiological symptoms of hydrocephalus. Each patient received radiotherapy, which was preceded by chemotherapy in 77.1%, and surgery in 31.4% of the cases. The majority of the patients received radiotherapy to the ventricular system (51.4%), craniospinal irradiation (34.3%), or whole-brain radiotherapy (8.6%) followed by a focal boost in 88%. The remaining patient was treated with focal radiotherapy only. The 5- and 10-year overall survival rate was found to be 97% and 87% respectively, including two patients who died despite disease control (pulmonary and ventricular embolism). The 5-year progression-free survival was 83%.

CONCLUSIONS

Despite variability in treatment methods, there is a relatively high cure and survival rate in patients with intracranial germinoma. However, the rare occurrence of the disease seems to negatively impact the diagnostic process. The tumor is often diagnosed at an advanced stage, presenting significant, often irreversible symptoms.

中文翻译：

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P06.04 三级中心治疗颅内生殖细胞瘤的经验

摘要

背景

颅内生殖细胞瘤是一种罕见的恶性肿瘤，约占中枢神经系统生殖细胞肿瘤的三分之二，好发于儿童和青少年的鞍上区、松果体或基底核。由于很少发生，治疗方式的可变性相对较高。本文旨在根据三级中心的经验，评估颅内生殖细胞瘤放疗和化疗的症状和治疗效果。

方法

该研究包括从 1999 年至 2020 年间在一个机构接受颅内生殖细胞瘤治疗的 35 名连续患者的回顾性收集数据。统计分析使用基本统计工具和 Kaplan-Meier 方法。

结果

诊断时的中位年龄为 17 岁（IQR 14-22），85.7% 的患者为男性。大多数患者的诊断得到组织病理学证实 (88.5%)，其中 76% 为非分泌性生殖细胞瘤。原发肿瘤最常位于松果体区域 (34.3%)，其次是双灶 (25.7%)、鞍上 (17.1%) 和基底生殖细胞瘤 (8.6%)。其余患者有播散性疾病（14.3%）。发病时最常见的症状是头痛 (54.3%)、视力障碍 (45.7%)、呕吐 (25.7%)、烦渴 (22.9%)、体重减轻 (17.1%) 或轻微神经功能缺损 (11.4%) ）。多达 54.3% 的患者在诊断时出现神经内分泌疾病，42.9% 的患者出现脑积水的放射学症状。每位患者接受放射治疗，77.1% 的病例先化疗，31.​​4% 的病例手术。大多数患者接受了心室系统放疗 (51.4%)、颅脊髓放疗 (34.3%) 或全脑放疗 (8.6%)，随后有 88% 的患者接受了局灶性放疗。其余患者仅接受局部放疗。发现 5 年和 10 年总生存率分别为 97% 和 87%，其中包括两名在疾病控制（肺栓塞和心室栓塞）后死亡的患者。5 年无进展生存率为 83%。其余患者仅接受局部放疗。发现 5 年和 10 年总生存率分别为 97% 和 87%，其中包括两名在疾病控制（肺栓塞和心室栓塞）后死亡的患者。5 年无进展生存率为 83%。其余患者仅接受局部放疗。发现 5 年和 10 年总生存率分别为 97% 和 87%，其中包括两名在疾病控制（肺栓塞和心室栓塞）后死亡的患者。5 年无进展生存率为 83%。

结论

尽管治疗方法存在差异，但颅内生殖细胞瘤患者的治愈率和存活率相对较高。然而，这种疾病的罕见发生似乎对诊断过程产生了负面影响。肿瘤通常在晚期被诊断出来，呈现出明显的、通常不可逆的症状。