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...
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| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2024-01-01
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| Series: | Brain and Spine |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2772529424000304 |
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| Summary: | 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. |
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| ISSN: | 2772-5294 |