Case Report
Progression of choroid plexus papilloma

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Abstract

Choroid plexus papillomas are rare neoplasms that arise from choroid plexus epithelium. The World Health Organization classification describes three histological grades. Grade I is choroid plexus papilloma, grade II is atypical choroid plexus papilloma and grade III is choroid plexus carcinoma. Progression between grades is rare but documented. We present two adult cases, a 53-year-old female and a 70-year-old male, who demonstrated clear interval histological progression from grade I choroid plexus papilloma to higher grades.

Introduction

Choroid plexus tumours are rare, comprising 0.3–0.6% of all intracranial tumours. Histologically, 80% are choroid plexus papillomas (CPP), 15% are atypical papillomas, and less than 5% are carcinomas.1 Progression from a benign CPP to a higher grade atypical CPP or choroid plexus carcinoma (CPC) is rare.2 We report two patients with interval histological progression.

Section snippets

Patient 1

A 53-year-old woman presented in 2005 with a history of headaches. Imaging showed a left lateral ventricular lesion (Fig. 1a, b). She underwent total resection via an interhemispheric transcallosal approach. Histopathology and immunohistochemistry were consistent with World Health Organization (WHO) grade I CPP (Fig. 2a). Yearly imaging between 2005 and 2009 showed no recurrence. In 2009, she re-presented with recurrent headaches. Imaging showed four new intraventricular lesions (Fig. 1c, d).

Discussion

CPPs are most common in childhood, accounting for around 4% of paediatric intracranial tumours.3 Fifty per cent are found in the lateral ventricles, 40% in the fourth ventricle and 5% in the third ventricle. Around 5% of patients have synchronous lesions at diagnosis. Clinically, CPP usually present with headaches secondary to hydrocephalus which is due to overproduction of CSF by the tumour, but can also be due to obstruction of CSF flow.1 CPP is best managed with gross total resection via a

Conclusions

CPP are benign tumours. However, there are reports of late recurrences, metastasis and histological progression. Hence, these patients need prolonged follow-up. Progression to atypical CPP and CPC can occur rarely, and warrants consideration of adjuvant treatment.

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    All the patients in the present study underwent long-term clinical follow-up and serial imaging. Benign CPPs infrequently progress to a higher grade APP or CPC.50 Jeibmann et al.51 reported that only 2 of 124 patients experienced a transition from a CPP and an APP to CPCs.

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    Choroid plexus papilloma (CPP) is a rare primary neuroectodermal tumor of the choroid plexus, accounting for less than 1% of all intracranial neoplasms and 2–4% of brain tumors in children [1]. Histologically, 80% of choroid plexus tumors are papillomas, 15% are atypical papillomas (ACPP), and less than 5% are carcinomas (CPC) [2]. These tumors, in adults, commonly present in the fourth ventricle, arising from the caudal roof with signs and symptoms of hydrocephalus, such as headache, diplopia and ataxia.

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