Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Auenbruggerplatz 8, 8036 Graz, Austria.
J Neurooncol. 2011 Aug;104(1):279-86. doi: 10.1007/s11060-010-0485-1. Epub 2010 Dec 22.
Approximately 30-50% of patients with intracranial primitive neuroectodermal tumors (PNETs) of the central nervous system (CNS) develop spinal metastases. In contrast, primary spinal CNS-PNETs are extremely uncommon. The database and study records of the German/Austrian brain tumor trials HIT 91, HIT SKK 92, and HIT 2000 were retrospectively reviewed to describe clinical features, treatment modalities, and outcome of children with primary CNS-PNETs of the spinal cord who were registered as observational patients. Out of 1,248 patients with medulloblastomas or CNS-PNETs registered in the HIT database four patients (female, n = 3) with primary CNS-PNETs of the spinal cord were identified. Age at diagnosis was 10, 16, 23, and 174 months. Location of primary tumors was medulla oblongata-T3, C2-T1, T10-L2, T7-T10. Two patients had metastatic disease at diagnosis. Complete and incomplete resection was performed in one patient each, whereas two patients underwent a biopsy only. Two patients received chemotherapy only, in accordance with the HIT 91 trial (sandwich chemotherapy arm). They developed disease progression and died six months after diagnosis. One patient was given chemotherapy in accordance with the HIT 2000 trial followed by craniospinal radiotherapy and four courses of maintenance chemotherapy. The patient is in complete remission almost four years after diagnosis. The fourth patient developed disease progression while receiving induction chemotherapy. Hence, chemotherapy was switched to a modified Head Start protocol. After three cycles he underwent double autologous stem cell transplantation and craniospinal irradiation. Forty months after diagnosis the patient is alive and well, but surveillance MRIs still show nodular enhancing lesions in the area of the primary tumor and intracranial meningeal enhancement. Primary CNS-PNETs of the spinal cord probably require multimodal treatment including radiotherapy to achieve sustained tumor control.
约 30-50%的颅内原始神经外胚层肿瘤（PNETs）中枢神经系统（CNS）患者会发生脊柱转移。相比之下，原发性脊髓 CNS-PNETs 极为罕见。对德国/奥地利脑肿瘤试验 HIT 91、HIT SKK 92 和 HIT 2000 的数据库和研究记录进行了回顾性分析，以描述作为观察性患者登记的原发性脊髓 CNS-PNETs 患儿的临床特征、治疗方式和结果。在 HIT 数据库中登记的 1248 例髓母细胞瘤或 CNS-PNETs 患者中，有 4 例（女性，n=3）被确定为原发性脊髓 CNS-PNETs。诊断时的年龄分别为 10、16、23 和 174 个月。原发肿瘤的位置分别为延髓-T3、C2-T1、T10-L2、T7-T10。2 例患者在诊断时已有转移疾病。1 例患者行完全切除，1 例患者行不完全切除，2 例患者仅行活检。2 例患者仅接受化疗，遵循 HIT 91 试验（夹心化疗组）。他们在诊断后 6 个月发生疾病进展并死亡。1 例患者遵循 HIT 2000 试验接受化疗，然后行颅脊髓放疗和 4 个疗程的维持化疗。该患者在诊断后近 4 年仍处于完全缓解状态。第 4 例患者在接受诱导化疗时发生疾病进展。因此，化疗方案改为改良的 Head Start 方案。3 个周期后，他接受了双自体干细胞移植和颅脊髓照射。诊断后 40 个月，患者仍存活且状况良好，但监测 MRI 仍显示原发肿瘤区域和颅内脑膜增强的结节性增强病变。原发性脊髓 CNS-PNETs 可能需要包括放疗在内的多模式治疗，以实现肿瘤的持续控制。
