Department of Hematology/Oncology, Mayo Clinic, Rochester, MN, USA.
Department of Radiology, Mayo Clinic, Rochester, MN, USA.
J Neurooncol. 2022 Nov;160(2):527-534. doi: 10.1007/s11060-022-04174-6. Epub 2022 Oct 27.
Pineal parenchymal tumor of intermediate differentiation (PPTID) is a neoplasm of pinealocyte origin and of intermediate differentiation (WHO grade 2 or 3). Treatment selection and prognostication is challenging for this rare, recently characterized tumor. In this single study, we review our clinical experience in patients with PPTID as well as pooled data from two other institutions.
This is a retrospective analysis of patients seen at Mayo Clinic, with additional data pooled from two similar studies at outside institutions for comparison and further analysis.
Ten adult patients (6 male) were identified. Median age at diagnosis was 36 years (range 13-73 years). Patients were followed between 3 and 88 months with no reported deaths. The most common presenting symptom was headache, followed by nausea. Nine patients had local disease without neuroaxial dissemination at diagnosis, one of which had tumor recurrence at 36 months. Two patients underwent gross total resection (GTR) without adjuvant radiation without recurrence (PFS 4 and 46 months). In our combined analysis, we reviewed 36 patients from three different institutions. Four patients presented with disseminated disease. GTR was achieved for 16 patients. Twenty-eight patients received adjuvant radiation therapy and 16 patients experienced disease recurrence. Median overall survival was 44 months.
Our single institution experience and combined multi-institution analysis suggest GTR is associated with improved outcomes. The role of adjuvant radiation therapy and utility of CSI compared to focal RT is less unclear. Prospective study is required to identify optimal adjuvant therapy selection.
松果体实质肿瘤中度分化（PPTID）是一种源自松果体细胞的肿瘤，分化程度为中度（WHO 分级 2 级或 3 级）。这种罕见的、最近才被确定的肿瘤，其治疗选择和预后评估颇具挑战性。在本单中心研究中，我们回顾了我们在 PPTID 患者中的临床经验，并汇总了另外两个机构的两项相似研究的数据进行比较和进一步分析。
这是对梅奥诊所患者进行的回顾性分析，另外还汇总了来自两个外部机构的两项相似研究的数据进行比较和进一步分析。
共确定了 10 例成年患者（6 例为男性）。诊断时的中位年龄为 36 岁（范围 13-73 岁）。患者的随访时间为 3 至 88 个月，无死亡报告。最常见的首发症状是头痛，其次是恶心。9 例患者诊断时无神经轴播散的局部疾病，其中 1 例在 36 个月时出现肿瘤复发。2 例患者接受了无辅助放疗的大体全切除（GTR），且无复发（无进展生存期分别为 4 个月和 46 个月）。在我们的联合分析中，我们回顾了来自三个不同机构的 36 例患者。4 例患者有播散性疾病。16 例患者实现了 GTR。28 例患者接受了辅助放疗，16 例患者出现疾病复发。中位总生存期为 44 个月。
我们的单中心经验和联合多中心分析表明，GTR 与改善结局相关。辅助放疗的作用以及全脑脊髓照射（CSI）与局部放疗相比的效用尚不清楚。需要前瞻性研究来确定最佳辅助治疗选择。
