D'Ambrosio Anthony L, O'Toole John E, Connolly E Sander, Feldstein Neil A
Department of Neurological Surgery, College of Physicians and Surgeons of Columbia University, New York, NY, USA.
Pediatr Neurosurg. 2003 Jul;39(2):91-6. doi: 10.1159/000071320.
Diffuse bilateral enlargement of the choroid plexus has long been appreciated as a cause of shunt-resistant hydrocephalus in infants, most often secondary to cerebrospinal fluid overproduction. Despite the fact that such lesions have interchangeably been termed 'villous hypertrophy' (VH) or 'bilateral choroid plexus papilloma' (CPP), little attention has been paid to the pathological distinctions that characterize these two entities. We present a case of presumed VH in a 2-week-old female presenting with cerebrospinal fluid production-dependent hydrocephalus that ultimately required bilateral surgical ablation of the choroid plexus. Although the preoperative radiological diagnosis was consistent with VH, postoperative histological analysis suggested bilateral CPP. The neoplastic nature of the lesion was confirmed by specific staining for the proliferation index, showing an MIB-1 labeling index of 4%, characteristic of papilloma. Routine imaging and histological grading of choroid plexus hypertrophy and papilloma have not provided a reliable means of predicting malignant behavior or recurrence after surgical resection. Moreover, none of the previously reported cases of VH in the literature have analyzed pathological specimens for cellular proliferative potential. Therefore, we review the literature on VH and bilateral CPP and discuss the diagnostic and possible prognostic implications of distinguishing these variants by utilizing the MIB-1 marker for the proliferation index.
长期以来，脉络丛双侧弥漫性增大一直被认为是婴儿分流抵抗性脑积水的一个原因，最常见的是继发于脑脊液分泌过多。尽管这种病变被交替称为“绒毛状肥大”（VH）或“双侧脉络丛乳头状瘤”（CPP），但很少有人关注区分这两种实体的病理差异。我们报告一例2周龄女性疑似VH的病例，该患者因脑脊液分泌依赖性脑积水就诊，最终需要双侧脉络丛手术切除。虽然术前影像学诊断与VH一致，但术后组织学分析提示为双侧CPP。通过增殖指数的特异性染色证实了病变的肿瘤性质，显示MIB-1标记指数为4%，这是乳头状瘤的特征。脉络丛肥大和乳头状瘤的常规影像学检查和组织学分级尚未提供预测手术切除后恶性行为或复发的可靠方法。此外，文献中先前报道的VH病例均未分析病理标本的细胞增殖潜能。因此，我们回顾了关于VH和双侧CPP的文献，并讨论了利用MIB-1标记增殖指数区分这些变体的诊断及可能的预后意义。
