本研究旨在通过分析单个机构 30 年的数据集来阐明松果体实质肿瘤 (PPT) 的临床特征、治疗策略和预后。我们回顾了 1990 年至 2020 年间在首尔国立大学医院诊断为 PPT 的 43 名患者的数据我们进行了生存分析并评估了预后因素。该队列包括 10 名松果体细胞瘤 (PC) 患者、13 名松果体中间分化实质肿瘤 (PPTID) 患者和 20 名松果体母细胞瘤 (PB) 患者。大多数患者在诊断时就出现脑积水。大多数患者接受了内镜下第三脑室造口术和活检，有些患者在诊断确认后接受了额外的切除术。放射治疗采用高流行率的伽玛刀放射外科治疗 PC 和 PPTID，以及颅脊髓照射治疗 PB。化疗对于治疗 3 级 PPTID 和 PB 至关重要。 PC、2级PPTID、3级PPTID和PB的5年无进展生存率分别为100%、83.3%、0%和40%，5年总生存率为100%，分别为 100%、40% 和 55%。高级别肿瘤组织学与较低的存活率相关。不同肿瘤类型的重要预后因素各不相同，对于 PPTID，世界卫生组织 (WHO) 分级和软脑膜种植 (LMS) 以及对于 PB 的切除范围和 LMS。三名患者经历了恶性转化。本研究强调了 PPT 中 WHO 分级的预后意义。需要根据肿瘤分级进行针对性治疗。 3级PPTID预后不良。潜在的 LMS 和恶性转化需要积极的多模式治疗和近距离筛查。版权所有 © 2023 韩国脑肿瘤学会、韩国神经肿瘤学会和韩国儿科神经肿瘤学会。

This study aims to elucidate clinical features, therapeutic strategies, and prognosis of pineal parenchymal tumors (PPT) by analyzing a 30-year dataset of a single institution.We reviewed data from 43 patients diagnosed with PPT at Seoul National University Hospital between 1990 and 2020. We performed survival analyses and assessed prognostic factors.The cohort included 10 patients with pineocytoma (PC), 13 with pineal parenchymal tumor of intermediate differentiation (PPTID), and 20 with pineoblastoma (PB). Most patients presented with hydrocephalus at diagnosis. Most patients underwent an endoscopic third ventriculostomy and biopsy, with some undergoing additional resection after diagnosis confirmation. Radiotherapy was administered with a high prevalence of gamma knife radiosurgery for PC and PPTID, and craniospinal irradiation for PB. Chemotherapy was essential in the treatment of grade 3 PPTID and PB. The 5-year progression-free survival rates for PC, grade 2 PPTID, grade 3 PPTID, and PB were 100%, 83.3%, 0%, and 40%, respectively, and the 5-year overall survival rates were 100%, 100%, 40%, and 55%, respectively. High-grade tumor histology was associated with lower survival rates. Significant prognostic factors varied among tumor types, with World Health Organization (WHO) grade and leptomeningeal seeding (LMS) for PPTID, and the extent of resection and LMS for PB. Three patients experienced malignant transformations.This study underscores the prognostic significance of WHO grades in PPT. It is necessary to provide specific treatment according to tumor grade. Grade 3 PPTID showed a poor prognosis. Potential LMS and malignant transformations necessitate aggressive multimodal treatment and close-interval screening.Copyright © 2023 The Korean Brain Tumor Society, The Korean Society for Neuro-Oncology, and The Korean Society for Pediatric Neuro-Oncology.