A choroid plexus carcinoma in the lateral ventricle of an adult

Clinical History

A 34-year-old male patient with an unremarkable medical history was referred to the emergency department because of an increasing headache and persistent neck pain, combined with intermittent confusion and disorientation. The clinical examination revealed a drowsy and sleepy patient without any other abnormalities.

Imaging Findings

The CT examination shows a large irregular cauliflower-like hyperdense mass in the trigone region of the left lateral ventricle. There is surrounding vasogenic oedema affecting multiple projection and association fibres (including the radiatio optica). The occipital and temporal horn of the left lateral ventricle are encysted and show signs of obstructive hydrocephalus. There is a mass effect on the supratentorial ventricles and on the medial temporal structures with a right-sided midline deviation. On the contrast-enhanced images this lesion shows a strong and heterogeneous enhancement.
A ventricle drain was placed in the occipital horn of the left lateral ventricle because of the obstructive hydrocephalus, and additional magnetic resonance (MR) images were acquired. On the MR images this lesion showed a heterogeneous signal on both the T2 and FLAIR-weighted images and a hypo to isointense T1 signal. The lesion showed restricted diffusion and a strong and heterogeneous contrast enhancement.

Discussion

A choroid plexus carcinoma is a rare malignant WHO grade III tumour that represents 20% of all choroid plexus neoplasms [1, 2]. It is most often seen in infants and young children and only very rarely seen in adults. This tumour arises from the epithelium of the choroid plexus and a small percentage is thought to be a malignant progression from an (atypical) choroid plexus papilloma [3]. These lesions grow fast and are invasive, and in 12-30% of cases there is already metastatic disease at presentation due to CSF seeding [4]. Patients typically present with symptoms due to hydrocephalus (nausea, vomiting, headache and obtundation) and brain invasion (focal neurological symptoms) [5]. There is an association with the Li Fraumeni syndrome [6]. The 5 year survival is poor, estimated to be less than 50%. The prognosis is very poor is there is CSF seeding at presentation [7]. MR imaging is the first choice of imaging modality [7]. On MR imaging these lesions show a hypo to isointense signal on the T1-weighted images and a heterogeneous signal on T2 and FLAIR-weighted images. Typically there is restricted diffusion in the solid parts of the tumour. Contrast enhancement shows a hypervascular lesion and may also reveal possible metastatic disease. Vasogenic oedema in the surrounding white matter is suggestive for invasion. There is also transependymal CSF flow due to the obstructive hydrocephalus [7, 8]. On CT images these lesions show calcifications in 20-25% of cases [9]. On angiography images an enlarged choroidal artery and vascular stain can be noted [7, 8]. MR imaging may not be able to distinguish a choroid plexus carcinoma from a papilloma, but heterogeneity, brain invasion and/or CSF seeding favour the diagnosis of a carcinoma [7]. In the adult one must also include the differential diagnosis of a hypervascular metastatic papillary adenocarcinoma, typically originating from a thyroid or renal cell malignancy [7, 8].
Surgical resection is the first choice of treatment and prior to surgery MR imaging of the neuroaxis must be performed to exclude metastatic disease [8]. Additional treatment with chemotherapy or radiotherapy is based on expert opinion.

Our patient underwent a surgical resection of the lesion after MRI of the neuroaxis was negative for metastasic disease. Six months after the surgery the patient experienced a hypoesthesis of the left thorax. New MRI of the neuroaxis revealed new brain and leptomeningeal metastases for which he is currently being treated with radiotherapy.

Differential Diagnosis List

Final Diagnosis

Choroid plexus carcinoma in the lateral ventricle of an adult

URL:	https://eurorad.org/case/14804
DOI:	10.1594/EURORAD/CASE.14804
ISSN:	1563-4086