Wieczorek Aleksandra, Balwierz Walentyna, Wyrobek Łukasz, Garus Katarzyna, Kwiatkowski Stanisław, Kwiecińska Kinga, Walicka-Soja Katarzyna
Klinika Onkologii i Hematologii Dzieciecej, Polsko-Amerykański Instytut Pediatrii, UJ CM, Kraków.
Przegl Lek. 2010;67(6):399-403.
Central nervous system (CNS) involvement in the course of neuroblastoma (NBL) in children is relatively rare. However, it seems to become serious clinical problem in the group of patients from high risk group. They presently achieve longer time of survival caused by employment of more intensive treatment modalities. The aim of the study was clinical evaluation of the patients over 1 year of age with stage 4 NBL with CNS involvement, both at diagnosis and at relapse. From 1997 to 2007, 117 patients (age 0.2-13.5 years) started NBL treatment. In 58 children over 1 year, stage 4 of disease was diagnosed. In 4 (6.9%) cases the CNS involvement was found at diagnosis. In 5 patients (8.6%) the isolated relapse in brain was diagnosed. The clinical symptoms caused by increased intracranial pressure were observed in all patients at relapse. In the case of initial involvement no neurological symptoms were observed. All 5 children with CNS involvement as isolated relapse did not present with infiltration of skull bones, whereas at initial diagnosis the brain lesions were continuous with bone metastases. Among 5 patients with isolated relapse 4 died because of NBL progression. Among 4 children with initial CNS involvement 1 died due to haemorrhage to CNS. Probably brain involvement at initial NBL diagnosis is not an additional negative prognostic factor. Because of extremely poor prognosis in patients with CNS relapse it should be advised to consider possible implementation of preventive treatment. It is also necessary to invent new more effective treatment methods.
中枢神经系统(CNS)受累于儿童神经母细胞瘤(NBL)病程中相对少见。然而,在高危组患者中这似乎成为了一个严重的临床问题。由于采用了更强化的治疗方式,他们目前获得了更长的生存时间。本研究的目的是对1岁以上伴有CNS受累的4期NBL患者在诊断时及复发时进行临床评估。1997年至2007年,117例患者(年龄0.2 - 13.5岁)开始接受NBL治疗。在58例1岁以上儿童中,诊断为疾病4期。4例(6.9%)在诊断时发现有CNS受累。5例患者(8.6%)诊断为脑孤立性复发。所有复发患者均观察到由颅内压升高引起的临床症状。在初始受累的情况下,未观察到神经症状。所有5例CNS受累为孤立性复发的儿童均未出现颅骨浸润,而在初始诊断时脑病变与骨转移相连。在5例孤立性复发患者中,4例因NBL进展死亡。在4例初始有CNS受累的儿童中,1例因CNS出血死亡。可能在NBL初始诊断时脑受累并非额外的不良预后因素。鉴于CNS复发患者预后极差,建议考虑可能实施预防性治疗。发明新的更有效的治疗方法也很有必要。