Pang Brandi W, Mazur-Hart David J, Yaghi Nasser K, Han Seunggu Jude, Liu Jesse J
Department of Neurological Surgery, Oregon Health & Science University, Portland, OR, USA.
Department of Neurological Surgery, Stanford University, Palo Alto, CA, USA.
Brain Tumor Res Treat. 2024 Oct;12(4):221-229. doi: 10.14791/btrt.2024.0021.
Papillary tumor of the pineal region (PTPR) is a rare neuroepithelial tumor with Central Nervous System (CNS) World Health Organization (WHO) grade II or III classification. Due to its rarity, there is no clear census on treatment. The purpose of this study is to identify the optimal treatment plan focused on extending overall survival (OS).
This is an institutional case series with review of the literature. Fifty-three publications were analyzed. Only cases with histological diagnosis of PTPR were included. Data collected included demographics, treatment modalities, disease progression, and OS.
The analysis included 105 patients from the literature and 3 new cases (54 female, 50%) with an average age of 33.1 years (range 1-73 years). The average lesion size was 26.4 mm (range 5-50 mm) in longest axis. All patients underwent an initial resection. There were 46 cases of surgery alone. The remaining cases received adjuvant therapy including radiation (RT), stereotactic radiosurgery (SRS), chemotherapy (CT), or RT and CT. The average follow-up was 61.4 months (range 1-240 months). OS at 1 year was 96.9%, at 5 years was 87.5%, and at 10 years was 80.2%. Overall progression-free survival (PFS) was 57.4%. Statistical significance was observed in PFS in the surgery plus SRS group and surgery plus CT and RT group. Surgery with SRS had the best PFS (75%), and OS at 1 year (100%) and 5 years (88.9%). Surgery with CT and RT had the best OS at 10 years (85.7%).
We describe a case series and literature review of PTPR to help guide the most effective treatment strategies for this rare disease entity. We recommend surgery followed by SRS as the treatment of choice because of its best PFS and 5-year survival rates. We would also recommend adding chemotherapy in the event of disease progression or recurrence as adjuvant radiation and chemotherapy provided the best 10-year survival.
松果体区乳头状瘤（PTPR）是一种罕见的神经上皮肿瘤，世界卫生组织（WHO）对其的中枢神经系统（CNS）分级为II级或III级。由于其罕见性，目前尚无明确的治疗统计数据。本研究的目的是确定以延长总生存期（OS）为重点的最佳治疗方案。
这是一项机构病例系列研究，并对文献进行了综述。分析了53篇出版物。仅纳入组织学诊断为PTPR的病例。收集的数据包括人口统计学、治疗方式、疾病进展和总生存期。
分析纳入了文献中的105例患者和3例新病例（54例女性，占50%），平均年龄为33.1岁（范围1 - 73岁）。病变最长径平均为26.4 mm（范围5 - 50 mm）。所有患者均接受了初次手术。单纯手术治疗的有46例。其余病例接受了辅助治疗，包括放疗（RT）、立体定向放射外科治疗（SRS）、化疗（CT）或放疗及化疗联合。平均随访时间为61.4个月（范围1 - 24个月）。1年总生存率为96.9%，5年为87.5%，10年为80.2%。总体无进展生存期（PFS）为57.4%。手术联合SRS组和手术联合CT及RT组的PFS具有统计学意义。手术联合SRS的PFS最佳（75%），1年总生存率（100%）和5年总生存率（88.9%）也最佳。手术联合CT及RT的10年总生存率最佳（85.7%）。
我们描述了一组PTPR病例系列及文献综述，以帮助指导针对这种罕见疾病实体的最有效治疗策略。我们推荐手术联合SRS作为首选治疗方法，因为其PFS和5年生存率最佳。我们还建议在疾病进展或复发时添加化疗，因为辅助放疗和化疗可提供最佳的10年生存率。
