Department of Pediatric Neurosurgery, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA.
Department of Neurological Surgery, Carle BroMenn Medical Center, Normal, IL, USA.
Childs Nerv Syst. 2023 Sep;39(9):2307-2316. doi: 10.1007/s00381-022-05593-6. Epub 2022 Jul 14.
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.
松果体区域肿瘤（PRT）占脑肿瘤的比例不到 1%。这些肿瘤的罕见性和异质性反映在各种治疗方式的应用上。
对 1996 年 11 月至 2021 年 6 月期间在单一中心接受治疗的所有儿童松果体区域肿瘤患者进行回顾性研究。对 56 例松果体肿瘤患者的年龄和发病时的症状、诊断方法、影像学特征、组织学分类、治疗方式、复发和死亡率进行了回顾。
平均诊断年龄为 11.3 岁。大多数患者为男性（82.1%）和白种人（73.2%）。最常见的首发症状是头痛（n=38，67.9%）和视力问题（n=34，60.7%）。49 例患者（87.5%）存在脑积水。生殖细胞瘤（n=20，35.7%）和非生殖细胞瘤性生殖细胞肿瘤（NGGCT）（n=17，30.4%）是最常见的肿瘤。54 例患者（96.4%）接受了化疗，49 例（87.5%）接受了放疗，14 例（25.0%）接受了手术切除。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%。
针对每个患者的表现、亚型、脑积水存在情况和疾病程度，必须有针对性地制定松果体区域肿瘤的治疗方案。通常不建议进行初始手术切除。随着肿瘤学治疗的进展，治疗方式的疗效可能会继续提高。
