Department of Neurological Surgery, Oregon Health & Science University, Portland, Oregon, USA,
Department of Neurological Surgery, Oregon Health & Science University, Portland, Oregon, USA.
Pediatr Neurosurg. 2022;57(5):348-357. doi: 10.1159/000525746. Epub 2022 Jun 27.
Choroid plexus tumors are rare neuroectodermal tumors that arise from the choroid plexus. Choroid plexus papillomas (CPPs) represent the lowest grade of these types of tumors and have a WHO grade I designation. Despite their typical low grade, some CPPs can exhibit aggressive behaviors including parenchymal invasion and dissemination throughout the neuro-axis. Due to their association with the choroid plexus, patients with CPP commonly present with signs and symptoms of hydrocephalus and increased intracranial pressure.
A 2-year-old male presented in extremis with acute hydrocephalus and seizure. He was found to have a large left intraventricular mass with innumerable intraparenchymal and extra-axial cysts throughout his neuro-axis. A literature review revealed five similar disseminated CPP cases with innumerable lesions. This is the youngest reported patient with disseminated CPP and the first with multiple compressive lesions. Following cranial resection and thoracic decompression, the patient's lesions have remained stable (2 years of follow-up). A literature search of the PubMed/Medline databases was performed using the search terms ["disseminated choroid plexus papilloma" OR "choroid plexus papilloma" OR "metastatic choroid plexus papilloma"] up to March 2021. Articles were then screened for similar patient radiographic presentation and histological diagnosis. To mitigate publication bias, referenced articles were utilized to identify other case reports and case series.
DISCUSSION/CONCLUSION: We describe a rare case of a lateral ventricle CPP with widespread leptomeningeal dissemination causing acute obstructive hydrocephalus and compressive myelopathy requiring cerebrospinal fluid diversion and intracranial resection followed by thoracic spine decompression. This case report serves to broaden knowledge of disseminated CPP and to encourage complete neuro-axis imaging for choroid plexus tumors. Additionally, we propose a naming paradigm refinement that includes radiographic characteristics.
脉络丛肿瘤是一种罕见的神经外胚层肿瘤，起源于脉络丛。脉络丛乳头瘤（CPP）是此类肿瘤中分级最低的，WHO 分级为 I 级。尽管它们通常为低级别，但有些 CPP 可表现出侵袭性行为，包括实质侵犯和沿神经轴播散。由于与脉络丛有关，CPP 患者通常表现为脑积水和颅内压增高的症状和体征。
一名 2 岁男性因急性脑积水和癫痫发作而病危。他被发现有一个大型左脑室肿瘤，其神经轴内有无数脑实质内和脑外囊肿。文献复习显示，有 5 例类似的播散性 CPP 病例，有无数病变。这是报道的年龄最小的播散性 CPP 患者，也是第一个有多个压迫性病变的患者。行颅切除术和胸段减压术后，患者的病变保持稳定（随访 2 年）。在 2021 年 3 月之前，使用搜索词“播散性脉络丛乳头瘤”或“脉络丛乳头瘤”或“转移性脉络丛乳头瘤”，在 PubMed/Medline 数据库中进行文献检索。然后筛选文章，以找到具有类似患者放射学表现和组织学诊断的文章。为了减轻发表偏倚，利用参考文献确定了其他病例报告和病例系列。
讨论/结论：我们描述了一例罕见的侧脑室 CPP 伴广泛软脑膜播散，导致急性梗阻性脑积水和压迫性脊髓病，需要脑脊液引流和颅内切除，随后行胸段脊柱减压。本病例报告旨在拓宽对播散性 CPP 的认识，并鼓励对脉络丛肿瘤进行完整的神经轴成像。此外，我们提出了一种命名模式的改进，包括影像学特征。
