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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Elsevier
2024-01-01
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| author | Senne Broekx Mania De Praeter |
| author_facet | Senne Broekx Mania De Praeter |
| author_sort | Senne Broekx |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-6f2a479854944d41b2a3961c58209f09 |
| institution | OA Journals |
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| language | English |
| publishDate | 2024-01-01 |
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| spelling | doaj-art-6f2a479854944d41b2a3961c58209f092025-08-20T01:56:24ZengElsevierBrain and Spine2772-52942024-01-01410277410.1016/j.bas.2024.102774A rare trifocal presentation of a choroid plexus papilloma: Case report and review of the literatureSenne Broekx0Mania De Praeter1Department of Neurosurgery, Antwerp University Hospital, 2650, Edegem, Belgium; Corresponding author.Department of Neurosurgery, Antwerp University Hospital, 2650, Edegem, Belgium; Faculty of Medicine, Antwerp University, 2650, Edegem, BelgiumIntroduction: 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.http://www.sciencedirect.com/science/article/pii/S2772529424000304Case reportChoroid plexus papillomaGeneticsTERTTP53Trifocal |
| spellingShingle | Senne Broekx Mania De Praeter A rare trifocal presentation of a choroid plexus papilloma: Case report and review of the literature Brain and Spine Case report Choroid plexus papilloma Genetics TERT TP53 Trifocal |
| title | A rare trifocal presentation of a choroid plexus papilloma: Case report and review of the literature |
| title_full | A rare trifocal presentation of a choroid plexus papilloma: Case report and review of the literature |
| title_fullStr | A rare trifocal presentation of a choroid plexus papilloma: Case report and review of the literature |
| title_full_unstemmed | A rare trifocal presentation of a choroid plexus papilloma: Case report and review of the literature |
| title_short | A rare trifocal presentation of a choroid plexus papilloma: Case report and review of the literature |
| title_sort | rare trifocal presentation of a choroid plexus papilloma case report and review of the literature |
| topic | Case report Choroid plexus papilloma Genetics TERT TP53 Trifocal |
| url | http://www.sciencedirect.com/science/article/pii/S2772529424000304 |
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