Morshed Ramin A, Lau Darryl, Sun Peter P, Ostling Lauren R
Department of Neurological Surgery, University of California, San Francisco; and.
UCSF Benioff Children's Hospital Oakland, California.
J Neurosurg Pediatr. 2017 Nov;20(5):471-479. doi: 10.3171/2017.5.PEDS17130. Epub 2017 Aug 25.
Choroid plexus papillomas (CPPs) are typically benign tumors that can occur in any age group but are more commonly found in pediatric patients. Although these tumors are benign, there are several reports in adult patients of distant metastases present either at the time of diagnosis or occurring months to years after initial resection. Here, the authors report the case of a 14-year-old boy who presented with symptoms of elevated intracranial pressure due to obstructive hydrocephalus that was caused by a large fourth ventricular mass. Preoperative imaging included a full MRI of the spine, which revealed an intradural lesion that encased the distal sacral nerve roots at the tip of the thecal sac and was concerning for a drop metastasis. The patient underwent gross-total resection of both the fourth ventricular and sacral tumors with histology of both lesions consistent with benign CPP (WHO Grade I). In addition, the authors review prior reports of both pediatric and adult patients in whom benign CPPs have metastasized with either benign or atypical pathology found at a distant site. Taking into account this unusual case and reports in the literature, patients with even benign CPPs may warrant initial and routine follow-up imaging of the total neural axis in search of the rare, but possible, occurrence of drop metastasis.
脉络丛乳头状瘤(CPPs)通常是良性肿瘤,可发生于任何年龄组,但更常见于儿童患者。尽管这些肿瘤是良性的,但有几例成年患者的报告显示,在诊断时或初次切除数月至数年后出现远处转移。在此,作者报告了一例14岁男孩的病例,该男孩因第四脑室巨大肿块导致梗阻性脑积水而出现颅内压升高症状。术前影像学检查包括全脊柱MRI,结果显示硬膜内病变包绕了硬膜囊末端的骶神经根远端,高度怀疑为播散性转移瘤。患者接受了第四脑室和骶部肿瘤的全切术,两个病变的组织学检查均符合良性CPP(WHO I级)。此外,作者回顾了之前关于儿童和成年患者的报告,这些患者的良性CPP发生了转移,在远处部位发现了良性或非典型病理。考虑到这一罕见病例及文献报道,即使是患有良性CPP的患者,也可能需要对整个神经轴进行初始和常规的随访影像学检查,以寻找罕见但可能发生的播散性转移。