Lafay-Cousin Lucie, Mabbott Donald J, Halliday William, Taylor Michael D, Tabori Uri, Kamaly-Asl Ian D, Kulkarni Abhaya V, Bartels Ute, Greenberg Mark, Bouffet Eric
Department of Pediatric Oncology and Bone Marrow Transplantation, Alberta Children's Hospital, Calgary, Alberta, Canada.
J Neurosurg Pediatr. 2010 Jun;5(6):615-21. doi: 10.3171/2010.3.PEDS09354.
Choroid plexus carcinomas (CPCs) are rare pediatric tumors with a generally poor prognosis. Although the role of surgery is well recognized, the role of adjuvant chemotherapy and radiation therapy remains unclear. In this paper, the authors' goal was to assess the role of second-look surgery and neoadjuvant ifosfamide, carboplatin, etoposide (ICE) chemotherapy in the management of CPC and to study neurocognitive outcome.
The authors performed an institutional retrospective review of patients in whom CPC was diagnosed between 1985 and 2006 at the Hospital for Sick Children in Toronto. Fourteen patients (7 boys and 7 girls) were included. The median age at diagnosis was 18.6 months (range 1.1-65.3 months). Four patients had evidence of metastatic disease at diagnosis. Two of the 14 patients underwent gross-total resection during initial surgery; 12 of the patients received neoadjuvant chemotherapy, 10 of whom underwent second surgery. In total, of 12 patients who received chemotherapy with a curative intent, 11 underwent a greater than 95% resection. Neoadjuvant ICE chemotherapy was given prior to second surgery (median 4 cycles, range 2-5 cycles) and was continued after second resection for a median total of 7 cycles (range 4-16 cycles).
No tumor progression was observed during chemotherapy prior to second surgery. Five patients subsequently experienced tumor progression/relapse. At a median follow-up of 6.9 years (range 1.9-18.5 years), 8 patients are alive. None of the survivors received radiation therapy. However, 6 of 8 display significant neurocognitive and/or sensorial deficit.
In this experience, second surgery following neoadjuvant ICE chemotherapy led to a high rate of complete or near-complete resection. Chemotherapy appears to facilitate second-look surgery, in particular through a reduction of intraoperative blood loss. Despite radiation avoidance, the majority of survivors experienced significant neurocognitive impairment.
脉络丛癌（CPC）是一种罕见的儿科肿瘤，预后通常较差。虽然手术的作用已得到充分认可，但辅助化疗和放疗的作用仍不明确。在本文中，作者的目标是评估二次手术和新辅助异环磷酰胺、卡铂、依托泊苷（ICE）化疗在CPC治疗中的作用，并研究神经认知结局。
作者对1985年至2006年在多伦多病童医院诊断为CPC的患者进行了机构回顾性研究。纳入14例患者（7例男孩和7例女孩）。诊断时的中位年龄为18.6个月（范围1.1 - 65.3个月）。4例患者在诊断时有转移疾病的证据。14例患者中有2例在初次手术时进行了全切除；12例患者接受了新辅助化疗，其中10例接受了二次手术。总共，在12例接受根治性化疗的患者中，11例进行了大于95%的切除。新辅助ICE化疗在二次手术前进行（中位4个周期，范围2 - 5个周期），并在二次切除后继续进行，中位总疗程为7个周期（范围4 - 16个周期）。
在二次手术前的化疗期间未观察到肿瘤进展。5例患者随后出现肿瘤进展/复发。中位随访6.9年（范围1.9 - 18.5年），8例患者存活。所有幸存者均未接受放疗。然而，8例中有6例表现出明显的神经认知和/或感觉缺陷。
在本研究中，新辅助ICE化疗后的二次手术导致了高比例的完全或接近完全切除。化疗似乎有助于二次手术，特别是通过减少术中失血。尽管避免了放疗，但大多数幸存者仍经历了明显的神经认知损害。
