A rare trifocal presentation of a choroid plexus papilloma: Case report and review of the literature

Introduction

CPP's present as slow-growing intraventricular neoplasms arising from epithelium of choroid plexus. They account for approximately 0.5–4% of intracranial neoplasms in adults and children, respectively. A trifocal presentation is exceedingly rare.

Research question

We describe the case of a trifocal presentation of a CPP and explored the importance of genetic analyses.

Material and methods

We present the case of an 18-year old adolescent who was treated for a fourth ventricular and suprasellar neoplasm. Brain MRI revealed an intraventricular lesion in the fourth ventricle, as well as a suprasellar lesion and a lesion located in the left internal auditory meatus. An adult-subtype CPP (WHO grade 1) was confirmed by means of histological and genetic analyses in the first two regions.

Results

Optimal treatment strategy remains controversial, although it is accepted that surgical resection alone remains the gold standard, whereas chemoradiotherapy is reserved for specific cases. There are only a few articles reporting on a multifocal presentation or the coexistence of synchronous histologically different primary brain neoplasms. Reports on genetic examination are scarce.

Discussion and conclusion

CPP's should be included in the differential diagnosis of posterior fossa tumors, both in children and adults. Genetic analyses (TP53/TERT mutations) should be considered, since they entail important diagnostic, prognostic and therapeutic implications. When a TERT mutation is present, adjuvant radiotherapy should be used with caution, since it plays a role in tumorigenesis, even when GTR could not be achieved. There is an association between TERT methylation status and malignant transformation, indicating that these patients should be followed more closely.