Top

Published in:

Open Access 01-11-2017 | Case Report - Brain Tumors

Recurrent papillary craniopharyngioma with BRAFV600E mutation treated with neoadjuvant-targeted therapy

Authors: Elham Rostami, Petra Witt Nyström, Sylwia Libard, Johan Wikström, Olivera Casar-Borota, Olafur Gudjonsson

Published in: Acta Neurochirurgica | Issue 11/2017

Abstract

Craniopharyngiomas are histologically benign but locally aggressive tumors in the sellar region that may cause devastating neurological and endocrine deficits. They tend to recur following surgery with high morbidity; hence, postoperative radiotherapy is recommended following sub-total resection. BRAFV600E mutation is the principal oncogenic driver in the papillary variant of craniopharyngiomas. Recently, a dramatic tumor reduction has been reported in a patient with BRAFV600E mutated, multiply recurrent papillary craniopharyngioma using a combination therapy of BRAF inhibitor dabrafenib and MEK inhibitor trametinib. Here, we report on near-radical reduction of a growing residual BRAFV600E craniopharyngioma using the same neoadjuvant therapy.

Brastianos PK, Shankar GM, Gill CM, Taylor-Weiner A, Nayyar N, Panka DJ, Sullivan RJ, Frederick DT, Abedalthagafi M, Jones PS, Dunn IF, Nahed BV, Romero JM, Louis DN, Getz G, Cahill DP, Santagata S, Curry WT, Jr., Barker FG, 2nd (2016) Dramatic response of BRAF V600E mutant papillary craniopharyngioma to targeted therapy. J Natl Cancer Inst 108. https://doi.org/10.1093/jnci/djv310

Brastianos PK, Taylor-Weiner A, Manley PE, Jones RT, Dias-Santagata D, Thorner AR, Lawrence MS, Rodriguez FJ, Bernardo LA, Schubert L, Sunkavalli A, Shillingford N, Calicchio ML, Lidov HG, Taha H, Martinez-Lage M, Santi M, Storm PB, Lee JY, Palmer JN, Adappa ND, Scott RM, Dunn IF, Laws ER Jr, Stewart C, Ligon KL, Hoang MP, Van Hummelen P, Hahn WC, Louis DN, Resnick AC, Kieran MW, Getz G, Santagata S (2014) Exome sequencing identifies BRAF mutations in papillary craniopharyngiomas. Nat Genet 46:161–165. https://doi.org/10.1038/ng.2868 CrossRefPubMedPubMedCentral

Bulow B, Attewell R, Hagmar L, Malmstrom P, Nordstrom CH, Erfurth EM (1998) Postoperative prognosis in craniopharyngioma with respect to cardiovascular mortality, survival, and tumor recurrence. J Clin Endocrinol Metab 83:3897–3904. https://doi.org/10.1210/jcem.83.11.5240 PubMed

Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F, Bruner JM (1998) The descriptive epidemiology of craniopharyngioma. J Neurosurg 89:547–551. https://doi.org/10.3171/jns.1998.89.4.0547 CrossRefPubMed

Cushing H (1932) Intracranial Tumors. Notes upon series of two thousand verified cases with surgical mortality percentages pertaining thereto. Thomas, Springfield

Davies H, Bignell GR, Cox C, Stephens P, Edkins S, Clegg S, Teague J, Woffendin H, Garnett MJ, Bottomley W, Davis N, Dicks E, Ewing R, Floyd Y, Gray K, Hall S, Hawes R, Hughes J, Kosmidou V, Menzies A, Mould C, Parker A, Stevens C, Watt S, Hooper S, Wilson R, Jayatilake H, Gusterson BA, Cooper C, Shipley J, Hargrave D, Pritchard-Jones K, Maitland N, Chenevix-Trench G, Riggins GJ, Bigner DD, Palmieri G, Cossu A, Flanagan A, Nicholson A, Ho JW, Leung SY, Yuen ST, Weber BL, Seigler HF, Darrow TL, Paterson H, Marais R, Marshall CJ, Wooster R, Stratton MR, Futreal PA (2002) Mutations of the BRAF gene in human cancer. Nature 417:949–954. https://doi.org/10.1038/nature00766 CrossRefPubMed

Fernandez-Miranda JC, Gardner PA, Snyderman CH, Devaney KO, Strojan P, Suarez C, Genden EM, Rinaldo A, Ferlito A (2012) Craniopharyngioma: a pathologic, clinical, and surgical review. Head Neck 34:1036–1044. https://doi.org/10.1002/hed.21771 CrossRefPubMed

Sorbye H, Dragomir A, Sundstrom M, Pfeiffer P, Thunberg U, Bergfors M, Aasebo K, Eide GE, Ponten F, Qvortrup C, Glimelius B (2015) High BRAF mutation frequency and marked survival differences in subgroups according to KRAS/BRAF mutation status and tumor tissue availability in a prospective population-based metastatic colorectal cancer cohort. PLoS One 10:e0131046. https://doi.org/10.1371/journal.pone.0131046 CrossRefPubMedPubMedCentral

Sosman JA, Kim KB, Schuchter L, Gonzalez R, Pavlick AC, Weber JS, McArthur GA, Hutson TE, Moschos SJ, Flaherty KT, Hersey P, Kefford R, Lawrence D, Puzanov I, Lewis KD, Amaravadi RK, Chmielowski B, Lawrence HJ, Shyr Y, Ye F, Li J, Nolop KB, Lee RJ, Joe AK, Ribas A (2012) Survival in BRAF V600-mutant advanced melanoma treated with vemurafenib. N Engl J Med 366:707–714. https://doi.org/10.1056/NEJMoa1112302 CrossRefPubMedPubMedCentral

10.

Yang I, Sughrue ME, Rutkowski MJ, Kaur R, Ivan ME, Aranda D, Barani IJ, Parsa AT (2010) Craniopharyngioma: a comparison of tumor control with various treatment strategies. Neurosurg Focus 28:E5. https://doi.org/10.3171/2010.1.FOCUS09307 CrossRefPubMed

11.

Yasargil MG, Curcic M, Kis M, Siegenthaler G, Teddy PJ, Roth P (1990) Total removal of craniopharyngiomas. Approaches and long-term results in 144 patients. J Neurosurg 73:3–11. https://doi.org/10.3171/jns.1990.73.1.0003 CrossRefPubMed

Title: Recurrent papillary craniopharyngioma with BRAFV600E mutation treated with neoadjuvant-targeted therapy
Authors: Elham Rostami
Petra Witt Nyström
Sylwia Libard
Johan Wikström
Olivera Casar-Borota
Olafur Gudjonsson
Publication date: 01-11-2017
Publisher: Springer Vienna
Published in: Acta Neurochirurgica / Issue 11/2017
Print ISSN: 0001-6268
Electronic ISSN: 0942-0940
DOI: https://doi.org/10.1007/s00701-017-3311-0

At a glance: The STEP trials

Springer Medicine

Recurrent papillary craniopharyngioma with BRAFV600E mutation treated with neoadjuvant-targeted therapy

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

Please log in to get access to this content

Other articles of this Issue 11/2017

Adolescents with moyamoya disease: clinical features, surgical treatment and long-term outcomes

Health-related quality of life of cranial WHO grade I meningioma patients: are current questionnaires relevant?

Prolonged hypotension after carotid artery stenting: incidence, predictors and consequences

Delayed symptomatic haemorrhage from the remnants of a thalamic arteriovenous malformation after previous angiographic cure with radiotherapy

Microsurgical versus endoscopic transsphenoidal resection for acromegaly: a systematic review of outcomes and complications

Post-operative seizures after burr hole evacuation of chronic subdural hematomas: is prophylactic anti-epileptic medication needed?