目的:松果体区肿瘤(PRT)占脑肿瘤的1%以下。这些肿瘤的罕见和异质性反映在所采用的治疗方式的多样性中。
方法:对1996年11月至2021年6月间所有松果体区肿瘤患儿进行了单中心回顾性研究。对56例松果体肿瘤患者的年龄和症状进行了回顾,诊断方法,成像特性,组织学分类,治疗方式,复发,和死亡率。
结果:诊断时的平均年龄为11.3岁。大多数患者为男性(82.1%)和白种人(73.2%)。最常见的症状是头痛(n=38,67.9%)和视觉问题(n=34,60.7%)。49例患者存在脑积水(87.5%)。生殖细胞瘤(n=20,35.7%)和非生殖细胞瘤(n=17,30.4%)是最常见的肿瘤。54例(96.4%)患者接受化疗,辐射为49(87.5%),手术切除14例(25.0%)。平均治疗时间为5.9个月。5年无进展生存率为74.4%,10年无进展生存率为72.0%。5年总生存率为85.7%,10年总生存率为77.1%。
结论:松果体区肿瘤的治疗必须针对每位患者,子类型,脑积水的存在,和疾病的程度。通常不需要进行前期手术切除。随着肿瘤治疗的进展,治疗方式可能会继续提高疗效。
Pineal region tumors (PRT) represent less than 1% of brain neoplasms. The rare and heterogeneous nature of these tumors is reflected in the variety of treatment modalities employed.
A single-center retrospective review of all pediatric patients with pineal region tumors between November 1996 and June 2021 was performed. Fifty-six cases of pineal tumors were reviewed for age and symptoms upon presentation, diagnostic methods, imaging characteristics, histological classification, treatment modalities, recurrence, and mortality rates.
The average age at diagnosis was 11.3 years. The majority of patients were male (82.1%) and Caucasian (73.2%). The most common presenting symptoms were headache (n = 38, 67.9%) and visual problems (n = 34, 60.7%). Hydrocephalus was present in 49 patients (87.5%). Germinoma (n = 20, 35.7%) and non-germinomatous germ cell tumor (NGGCT) (n = 17, 30.4%) were the most common tumors. Chemotherapy was employed for 54 patients (96.4%), radiation for 49 (87.5%), and surgical resection for 14 (25.0%). The average duration of treatment was 5.9 months. Progression-free survival was 74.4% at 5 years and 72.0% at 10 years. Overall survival was 85.7% at 5 years and 77.1% at 10 years.
Treatment of pineal region tumors must be targeted to each patient based on presentation, subtype, presence of hydrocephalus, and extent of disease. Upfront surgical resection is usually not indicated. As advances in oncological care proceed, treatment modalities may continue to improve in efficacy.