Lafay-Cousin Lucie, Keene Daniel, Carret Anne-Sophie, Fryer Chris, Brossard Josee, Crooks Bruce, Eisenstat David, Johnston Donna, Larouche Valerie, Silva Marianna, Wilson Beverly, Zelcer Shayna, Bartels Ute, Bouffet Eric
Pediatric Hematology Oncology and Bone marrow transplantation program, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, Canada.
Childs Nerv Syst. 2011 Feb;27(2):259-64. doi: 10.1007/s00381-010-1269-9. Epub 2010 Aug 31.
Choroid plexus tumors (CPT) are rare pediatric tumors. A population-based study on choroid plexus carcinoma (CPC) and choroid plexus papilloma (CPP) was carried out to describe the incidence, demographic, and outcome data and to identify potential prognostic factors.
The CPT population from the Canadian databank of CNS tumor in children ≤ 36 months diagnosed between 1990 and 2005 was reviewed
Out of the 579 reported cases of CNS tumors, 37 were CPT. The annual age-adjusted incidence rate was 0.22 + 0.12 (95% CI 0.16-0.28)/100,000 children < 3 years. There were 21 (56.7%) CPP and 16 (43.3.5%) CPC. Twenty patients (54%) were males. Median age at diagnosis was 7 months(range 0-30). Ten patients(62.5%) with CPC and one with CPP were metastatic at diagnosis. Twenty patients with CPP (95%) had a complete resection, whereas 6/16 CPC (37.5%) achieved a resection >90%. Fourteen CPC patients received adjuvant chemotherapy. None of the 37 patients received adjuvant radiation. At completion of survey, all CPP and five CPC were alive. Median survival time for CPC patients was 15 months (0-120). One death was related to intraoperative hemorrhage, another to chemotherapy-induced toxicity, and one to secondary AML. Age at diagnosis, degree of resection and metastatic status were not significant prognostic factors for CPC.
By contrast to CPC, CPP have an excellent prognosis following surgery alone. Survival of CPC remains poor. However, these data may suggest adjuvant chemotherapy can alter the aggressive natural history of CPC. As with other rare CNS tumors, international collaboration is required to identify optimal therapy.
脉络丛肿瘤（CPT）是罕见的儿科肿瘤。开展了一项基于人群的脉络丛癌（CPC）和脉络丛乳头状瘤（CPP）研究，以描述发病率、人口统计学和结局数据，并确定潜在的预后因素。
回顾了1990年至2005年间诊断的加拿大儿童中枢神经系统肿瘤数据库中36个月及以下儿童的CPT人群。
在579例报告的中枢神经系统肿瘤病例中，37例为CPT。年龄调整后的年发病率为0.22±0.12（95%CI 0.16 - 0.28）/10万＜3岁儿童。有21例（56.7%）CPP和16例（43.3%）CPC。20例患者（54%）为男性。诊断时的中位年龄为7个月（范围0 - 30个月）。10例（62.5%）CPC患者和1例CPP患者在诊断时发生转移。20例CPP患者（95%）进行了全切，而16例CPC中有6例（37.5%）切除率＞90%。14例CPC患者接受了辅助化疗。37例患者均未接受辅助放疗。调查结束时，所有CPP患者和5例CPC患者存活。CPC患者的中位生存时间为15个月（0 - 120个月）。1例死亡与术中出血有关，1例与化疗引起的毒性有关，1例与继发性急性髓细胞白血病有关。诊断时的年龄、切除程度和转移状态不是CPC的显著预后因素。
与CPC相比，CPP仅手术治疗后预后良好。CPC的生存率仍然很低。然而，这些数据可能表明辅助化疗可以改变CPC的侵袭性自然病程。与其他罕见的中枢神经系统肿瘤一样，需要国际合作来确定最佳治疗方案。
