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

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

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)571-583
Number of pages13
JournalPituitary
Volume21
Issue number6
DOIs
Publication statusPublished - Dec 1 2018
Externally publishedYes

Fingerprint

Craniopharyngioma
Hypopituitarism
Neoplasms
Length of Stay
Weights and Measures
Drug Therapy
Mutation

Keywords

  • BRAF
  • Craniopharyngioma
  • CTNNB1
  • Papillary
  • Suprasellar
  • Third ventricle

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

Cite this

La Corte, E., Younus, I., Pivari, F., Selimi, A., Ottenhausen, M., Forbes, J. A., ... Schwartz, T. H. (2018). BRAF V600E mutant papillary craniopharyngiomas: a single-institutional case series. Pituitary, 21(6), 571-583. https://doi.org/10.1007/s11102-018-0909-z

BRAF V600E mutant papillary craniopharyngiomas : a single-institutional case series. / La Corte, Emanuele; Younus, Iyan; Pivari, Francesca; Selimi, Adelina; Ottenhausen, Malte; Forbes, Jonathan A.; Pisapia, David J.; Dobri, Georgiana A.; Anand, Vijay K.; Schwartz, Theodore H.

In: Pituitary, Vol. 21, No. 6, 01.12.2018, p. 571-583.

Research output: Contribution to journalArticle

La Corte, E, Younus, I, Pivari, F, Selimi, A, Ottenhausen, M, Forbes, JA, Pisapia, DJ, Dobri, GA, Anand, VK & Schwartz, TH 2018, 'BRAF V600E mutant papillary craniopharyngiomas: a single-institutional case series', Pituitary, vol. 21, no. 6, pp. 571-583. https://doi.org/10.1007/s11102-018-0909-z
La Corte E, Younus I, Pivari F, Selimi A, Ottenhausen M, Forbes JA et al. BRAF V600E mutant papillary craniopharyngiomas: a single-institutional case series. Pituitary. 2018 Dec 1;21(6):571-583. https://doi.org/10.1007/s11102-018-0909-z
La Corte, Emanuele ; Younus, Iyan ; Pivari, Francesca ; Selimi, Adelina ; Ottenhausen, Malte ; Forbes, Jonathan A. ; Pisapia, David J. ; Dobri, Georgiana A. ; Anand, Vijay K. ; Schwartz, Theodore H. / BRAF V600E mutant papillary craniopharyngiomas : a single-institutional case series. In: Pituitary. 2018 ; Vol. 21, No. 6. pp. 571-583.
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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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AU - Selimi, Adelina

AU - Ottenhausen, Malte

AU - Forbes, Jonathan A.

AU - Pisapia, David J.

AU - Dobri, Georgiana A.

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N2 - 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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KW - Third ventricle

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