Medulloblastoma in children - the Ottawa experience

被引:26
作者
Modha, A
Vassilyadi, M
George, A
Kuehn, S
Hsu, E
Ventureyra, ECG
机构
[1] Childrens Hosp Eastern Ontario, Div Neurosurg, Ottawa, ON K1H 8L1, Canada
[2] Univ Ottawa, Childrens Hosp Eastern Ontario, Dept Psychol, Mental Hlth Patient Serv Unit, Ottawa, ON, Canada
[3] Univ Ottawa, Childrens Hosp Eastern Ontario, Dept Oncol, Ottawa, ON, Canada
关键词
medulloblastoma; primitive neuroectodermal tumor (PNET);
D O I
10.1007/s003810050529
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
A retrospective review of 36 children diagnosed with medulloblastoma in the Ottawa area between 1974 and 1997 was completed (mean age 7.8+/-4.2 years, range 1.2-15.3 years). Via a suboccipital approach, complete tumor resection was achieved in 75% and subtotal resection (>90%) in 25%, without any operative mortality. The tumor was located in the vermis in 39% and in the cerebellar hemisphere in 11%; it occupied both locations in 50%. In 47% of the children a ventriculoperitoneal shunt was required. Postoperatively, craniospinal radiation at 3600 cGy with a boost to the posterior fossa was administered. Chemotherapy was used in 56%. The 1-year survival rate was 92%, and survival plateaued at 54% at 5 years. Children less than 3 years of age fared worse than those over 3 years old. While the male-to-female ratio was 1.6:1, there was no gender difference in survival. Chang's classification was used to grade the tumors. T stage did not have an impact on survival, but M stage did. No statistically significant difference in survival was found between the patients who had a total resection and those who had a subtotal resection. There was no difference in survival in terms of tumor location, hydrocephalus or ventriculoperitoneal shunt. Chemotherapy showed no survival benefit. The recurrence rate was 26%, and its timing followed Collin's law. Recurrence led to death within 1-9 months. GH deficiency was diagnosed in 5 patients and hypothyroidism in 4 patients. The mean follow-up time was 4.4+/-3.7 years, with a range of 2.5 months to 16.5 years. Fourteen patients died, 5 were lost to follow-up, and 7 were transferred to adult care without persistent disease. Ten children are presently being followed up by the Neuro-oncology Clinic. Four children continue to be followed through psychology services. Our results are comparable to those in larger series, and are similar to those of the Montreal Children's Hospital.
引用
收藏
页码:341 / 350
页数:10
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