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Late recurrence of choroid plexus carcinoma.
Child's Nervous System ( IF 1.4 ) Pub Date : 2020-05-15 , DOI: 10.1007/s00381-020-04663-x
Shannon Hart 1 , Roger Avery 1, 2 , Jane Barron 1, 3
Affiliation  

BACKGROUND Choroid plexus carcinomas (CPC) are rare malignant brain tumours arising from the choroid plexus epithelium. CPC are most common in the paediatric population, particularly those under 2 years of age. Common presentations include headache, diplopia and signs of increased intracranial pressure such as nausea and vomiting. Infants may present with increased head circumference, bulging fontanelles, splayed cranial sutures and/or neurological delay. Diagnosis is made via radiological and histological analysis. MANAGEMENT AND PROGNOSIS Gross total resection (GTR) is the preferred treatment and infers the best survival rate, but despite this, prognosis remains poor. The utility of chemotherapy and/or radiation in CPC management remains controversial, and an optimal treatment regimen has not been identified. Even with GTR, recurrence is common and usually occurs within months after resection. Delayed recurrence is exquisitely rare and has been reported very few times to date. CASE PRESENTATION Here, we present a rare case of delayed CPC recurrence 10 years after initial presentation. A 2-month-old male was diagnosed with CPC and received GTR, chemotherapy and stem cell transplant. The patient presented with a recurrent CPC 10 years after the initial diagnosis. CONCLUSIONS This case demonstrates the importance of long-term surveillance and raises questions regarding the natural history, recurrence patterns and factors contributing to long-term relapse in CPC. Further research should be targeted at identifying patient factors contributing to increased risk of late recurrence and whether adjuvant treatments play any role in decreasing this.

中文翻译:

脉络丛癌晚期复发。

背景脉络丛癌(CPC)是起源于脉络丛上皮的罕见恶性脑肿瘤。CPC 在儿科人群中最为常见,尤其是 2 岁以下的人群。常见的表现包括头痛、复视和颅内压升高的迹象,如恶心和呕吐。婴儿可能会出现头围增加、囟门鼓起、颅缝张开和/或神经系统延迟。通过放射学和组织学分析进行诊断。管理和预后 大体全切除术 (GTR) 是首选治疗方法,可以推断出最佳生存率,但尽管如此,预后仍然很差。化疗和/或放疗在 CPC 管理中的效用仍然存在争议,并且尚未确定最佳治疗方案。即使使用 GTR,复发很常见,通常发生在切除后的几个月内。延迟复发极为罕见,迄今为止报告的次数很少。病例介绍 在这里,我们介绍了一个罕见的 CPC 延迟复发病例,该病例在初次就诊 10 年后复发。一名 2 个月大的男性被诊断出患有 CPC,并接受了 GTR、化疗和干细胞移植。患者在初次诊断 10 年后出现复发性 CPC。结论 该病例证明了长期监测的重要性,并提出了关于自然史、复发模式和导致 CPC 长期复发的因素的问题。进一步的研究应针对确定导致晚期复发风险增加的患者因素以及辅助治疗是否在降低这种风险方面发挥任何作用。
更新日期:2020-05-15
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