Radiation Oncology, Catalan Institute of Oncology, H. Universitari Germans Trías, Badalona, Barcelona, Catalonia, Spain.
Clin Transl Oncol. 2012 Nov;14(11):827-34. doi: 10.1007/s12094-012-0869-0. Epub 2012 Aug 23.
To better define outcome and prognostic factors in primary pineal tumors.
Thirty-five consecutive patients from seven academic centers of the Rare Cancer Network diagnosed between 1988 and 2006 were included. Median age was 36 years. Surgical resection consisted of biopsy in 12 cases and resection in 21 (2 cases with unknown resection). All patients underwent radiotherapy and 12 patients received also chemotherapy.
Histological subtypes were pineoblastoma (PNB) in 21 patients, pineocytoma (PC) in 8 patients and pineocytoma with intermediate differentiation in 6 patients. Six patients with PNB had evidence of spinal seeding. Fifteen patients relapsed (14 PNB and 1 PC) with PNB cases at higher risk (p = 0.031). Median survival time was not reached. Median disease-free survival was 82 months (CI 50 % 28-275). In univariate analysis, age younger than 36 years was an unfavorable prognostic factor (p = 0.003). Patients with metastases at diagnosis had poorer survival (p = 0.048). Late side effects related to radiotherapy were dementia, leukoencephalopathy or memory loss in seven cases, occipital ischemia in one, and grade 3 seizures in two cases. Side effects related to chemotherapy were grade 3-4 leucopenia in five cases, grade 4 thrombocytopenia in three cases, grade 2 anemia in two cases, grade 4 pancytopenia in one case, grade 4 vomiting in one case and renal failure in one case.
Age and dissemination at diagnosis influenced survival in our series. The prevalence of chronic toxicity suggests that new adjuvant strategies are advisable.
更好地定义原发性松果体肿瘤的结果和预后因素。
纳入了来自 Rare Cancer Network 的七个学术中心的 35 例连续患者，这些患者均在 1988 年至 2006 年期间被诊断为原发性松果体肿瘤。中位年龄为 36 岁。手术切除包括活检 12 例和切除术 21 例（2 例手术切除范围未知）。所有患者均接受了放疗，12 例患者还接受了化疗。
组织学亚型为松果体母细胞瘤（PNB）21 例，松果体细胞瘤（PC）8 例，中间分化的松果体细胞瘤 6 例。6 例 PNB 患者有脊髓播种的证据。15 例患者复发（14 例 PNB 和 1 例 PC），其中 PNB 病例风险更高（p = 0.031）。中位生存时间未达到。中位无疾病生存时间为 82 个月（CI 50% 28-275）。在单因素分析中，年龄小于 36 岁是不利的预后因素（p = 0.003）。诊断时即有转移的患者生存较差（p = 0.048）。与放疗相关的晚期副作用包括 7 例痴呆、脑白质病或记忆力丧失、1 例枕叶缺血、2 例 3 级癫痫发作。与化疗相关的副作用包括 5 例 3-4 级白细胞减少症、3 例 4 级血小板减少症、2 例 2 级贫血症、1 例 4 级全血细胞减少症、1 例 4 级呕吐症和 1 例肾衰竭症。
在我们的系列研究中，年龄和诊断时的扩散影响了生存。慢性毒性的高发率表明，新的辅助治疗策略是可行的。
