Pineal region tumors commonly present with non-communicating hydrocephalus. These heterogeneous histological entities require different therapeutic regimens. We evaluated our surgical experience concerning procurance of a histological diagnosis, management of hydrocephalus, and choice of antitumoral treatment. We analyzed the efficacy of neuroendoscopic biopsy and endoscopic third ventriculocisternostomy (ETV) in patients with pineal region tumors between 2006 and 2019 in a single-center retrospective cross-sectional study with regard to diagnostic yield, hydrocephalus treatment, as well as impact on further antitumoral management. Out of 28 identified patients, 23 patients presented with untreated hydrocephalus and 25 without histological diagnosis. One patient underwent open biopsy, and 24 received a neuroendoscopic biopsy with concomitant hydrocephalus treatment if necessary. Eighteen primary ETVs, 2 secondary ETVs, and 2 ventriculoperitoneal shunts (VPSs) were performed. Endoscopic biopsy had a diagnostic yield of 95.8% (23/24) and complication rates of 12.5% (transient) and 4.2% (permanent), respectively. ETV for hydrocephalus management was successful in 89.5% (17/19) with a median follow-up of more than 3 years. Following histological diagnosis, 8 patients (28.6%) underwent primary resection of their tumor. Another 9 patients underwent later-stage resection after either adjuvant treatment (n = 5) or for progressive disease during observation (n = 4). Eventually, 20 patients received adjuvant treatment and 7 were observed after primary management. One patient was lost to follow-up. Heterogeneity of pineal region tumor requires histological confirmation. Primary biopsy of pineal lesions should precede surgical resection since less than a third of patients needed primary surgical resection according to the German pediatric brain tumor protocols. Interdisciplinary decision making upfront any treatment is warranted in order to adequately guide treatment.

松果体区肿瘤通常伴有非交通性脑积水。这些异质的组织学实体需要不同的治疗方案。我们评估了我们在获得组织学诊断、脑积水处理和抗肿瘤治疗选择方面的手术经验。我们在一项单中心回顾性横断面研究中分析了 2006 年至 2019 年间神经内窥镜活检和内窥镜第三脑室胆管吻合术 (ETV) 对松果体区肿瘤患者的诊断率、脑积水治疗以及对进一步抗肿瘤治疗的影响的疗效。管理。在确定的 28 名患者中，23 名患者出现未经治疗的脑积水，25 名未进行组织学诊断。一名患者接受了开放活检，24 人接受了神经内窥镜活检，必要时同时进行脑积水治疗。进行了 18 次主要 ETV、2 次次要 ETV 和 2 次脑室腹腔分流术 (VPS)。内窥镜活检的诊断率为 95.8% (23/24)，并发症发生率分别为 12.5%（暂时性）和 4.2%（永久性）。ETV 治疗脑积水的成功率为 89.5% (17/19)，中位随访时间超过 3 年。在组织学诊断后，8 名患者 (28.6%) 接受了肿瘤的初次切除。另有 9 名患者在任一辅助治疗后接受了晚期切除术（分别为 5%（瞬态）和 4.2%（永久）。ETV 治疗脑积水的成功率为 89.5% (17/19)，中位随访时间超过 3 年。在组织学诊断后，8 名患者 (28.6%) 接受了肿瘤的初次切除。另有 9 名患者在任一辅助治疗后接受了晚期切除术（分别为 5%（瞬态）和 4.2%（永久）。ETV 治疗脑积水的成功率为 89.5% (17/19)，中位随访时间超过 3 年。在组织学诊断后，8 名患者 (28.6%) 接受了肿瘤的初次切除。另有 9 名患者在任一辅助治疗后接受了晚期切除术（n = 5) 或观察期间疾病进展 ( n = 4)。最终，20 名患者接受了辅助治疗，7 名患者在初级治疗后进行了观察。一名患者失访。松果体区肿瘤的异质性需要组织学确认。松果体病变的初次活检应在手术切除之前进行，因为根据德国儿科脑肿瘤协议，不到三分之一的患者需要进行初次手术切除。任何治疗都需要预先做出跨学科决策，以充分指导治疗。