Gilheeney Stephen W, Saad Ali, Chi Susan, Turner Christopher, Ullrich Nicole J, Goumnerova Liliana, Scott R Michael, Marcus Karen, Lehman Leslie, De Girolami Umberto, Kieran Mark W
Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA, USA.
J Neurooncol. 2008 Aug;89(1):89-95. doi: 10.1007/s11060-008-9589-2. Epub 2008 Apr 16.
Pineoblastomas are a category of supratentorial primitive neuroectodermal tumors (sPNETs) occurring in the pineal gland; some studies support the impression that patients with pineoblastomas have a worse prognosis than those with other sPNETs.
We reviewed the medical records and tissue sections of all patients with the diagnosis of pineoblastoma that were treated at the Dana-Farber Cancer Institute/Children's Hospital Boston Pediatric Brain Tumor Program between 1986 and 2005.
Thirteen patients with the pathologic diagnosis of pineoblastoma were treated at our Hospital; 11 of these cases had complete records suitable for study. The median age was 8 years 8 months (5 F, 6 M). Surgical, radiation and chemotherapeutic regimens varied from case to case. Three patients had gross total resection and are alive and free of disease, versus four of eight with subtotal resection or biopsy only. Patients who received CSI and multi-agent chemotherapy had improved overall survival.
Seven of eleven patients with pineoblastoma are currently alive and free of disease, reflecting an improved outcome and longer survival than previously appreciated. Gross total surgical resection appeared to correlate with improved survival, as did treatment with craniospinal irradiation and multi-agent chemotherapy. Further study of this group of patients as a distinct diagnostic entity will be necessary to determine optimal therapy.
松果体母细胞瘤是一类发生于松果体的幕上原始神经外胚层肿瘤（sPNETs）；一些研究支持这样的观点，即松果体母细胞瘤患者的预后比其他sPNETs患者更差。
我们回顾了1986年至2005年间在达纳-法伯癌症研究所/波士顿儿童医院儿科脑肿瘤项目接受治疗的所有诊断为松果体母细胞瘤患者的病历和组织切片。
我院共治疗了13例经病理诊断为松果体母细胞瘤的患者；其中11例病例有适合研究的完整记录。中位年龄为8岁8个月（5名女性，6名男性）。手术、放疗和化疗方案因病例而异。3例患者接受了全切除，目前存活且无疾病，而仅接受次全切除或活检的8例患者中有4例存活。接受全脑脊髓放疗和多药化疗的患者总体生存率有所提高。
11例松果体母细胞瘤患者中有7例目前存活且无疾病，这表明其预后有所改善，生存期比之前预期的更长。全切除似乎与生存率提高相关，全脑脊髓放疗和多药化疗也是如此。有必要对这组患者作为一个独特的诊断实体进行进一步研究，以确定最佳治疗方案。
