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.