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05.09.2018

BRAF V600E mutant papillary craniopharyngiomas: a single-institutional case series

verfasst von: Emanuele La Corte, Iyan Younus, Francesca Pivari, Adelina Selimi, Malte Ottenhausen, Jonathan A. Forbes, David J. Pisapia, Georgiana A. Dobri, Vijay K. Anand, Theodore H. Schwartz

Erschienen in: Pituitary | Ausgabe 6/2018

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Abstract

Purpose

To describe the clinical, radiographic and surgical outcomes in a cohort of patients with BRAF V600E mutant papillary craniopharyngiomas.

Methods

A retrospective review was performed to identify all patients with a histological diagnosis of CP operated upon at a single institution between 2005 and 2017. All cases with adequate material were sequenced to confirm the presence of BRAF V600E mutation.

Results

Sixteen patients were included in the present study. Approach was endoscopic endonasal (EEA) in 14 and transcranial (TCA) in 2. All patients were adult with an average age of 50 years (24–88). Radiographic review demonstrated that the majority (93.7%) were suprasellar and twelve (75%) had third ventricular involvement. No tumor showed evidence of calcifications and 68.7% were mixed solid-cystic. All patients had some evidence of hypopituitarism and 62.5% had hypothalamic disturbances. GTR was achieved in 11/14 (78.6%) EEA and 0/2 (0%) TCA (p < 0.05). The mean length of stay was 17.5 days in the TCA group and 7.6 days in the EEA group (p < 0.05). There were no CSF leaks. Post-operatively, eleven (68.7%) developed new DI or new hypopituitarism. Nine increased their BMI with a mean increase of 12.3%, whereas six patients lost weight with a mean decrease of 5.3%.

Conclusions

BRAF V600E mutant papillary tumors represent a clearly distinct clinical-pathological entity of craniopharyngiomas. These are generally non-calcified suprasellar tumors that occur in adults. These distinct characteristics may someday lead to upfront chemotherapy. When surgery is necessary, EEA may be preferred over TCA.

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Titel: BRAF V600E mutant papillary craniopharyngiomas: a single-institutional case series
verfasst von: Emanuele La Corte
Iyan Younus
Francesca Pivari
Adelina Selimi
Malte Ottenhausen
Jonathan A. Forbes
David J. Pisapia
Georgiana A. Dobri
Vijay K. Anand
Theodore H. Schwartz
Publikationsdatum: 05.09.2018
Verlag: Springer US
Erschienen in: Pituitary / Ausgabe 6/2018
Print ISSN: 1386-341X
Elektronische ISSN: 1573-7403
DOI: https://doi.org/10.1007/s11102-018-0909-z

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