EM. Erfurth*a (Prof), S. Kinhulta (Dr), P. Siesj√∂a (Prof)

a Clinical Sciences, Lund University, Lund Sweden, Lund, SWEDEN

* eva_marie.erfurth@med.lu.se

Introduction: In 2014 the oncogenic driver of papillary craniopharyngioma (PCP) was discovered as BRAF V600E (1). The first case report showed a successful treatment of a man with PCP and the mutation (2) using BRAF-inhibitor (Dabrafenid orally) and MEK -inhibitor (Trametinib on day 21 orally). On day 38 after considerable shrinkage of the PCP an endocopic transphenoidal surgery was performed, followed by Radiation 50 Gy. About 8 case reports with PCP and BRAF V600E mutation have been published with successful treatment with the BRAF- inhibitors (only) or together with MEK inhibitors. We now present 2 cases with PCP and BRAF V600E mutation on this treatment.

Case: 1. A 53 yrs healthy man, operated for traumatic subdural hematoma in 2017. On MRI an accidental finding of a suprasellar mass. A biopsy revealed a PCP with BRAF V600E mutation. After 3 years of follow-up a progression of PCP was recorded and decision on dab/tram treatments was made. During 12 months a shrinkage of the tumour from 21 to 6 mm /2220 mm3 till 90 mm3 was recorded. Decision was made on Sterotactic Radiation (50Gy). At present the patient has normal pituitary function and is without medication.

Case 2: A woman 57 yrs with PCP had had 4 operations due to recurrences (2017-2019). Retrospective diagnosed with a BRAF V600E mutation. She suffers from severe vision and visual field reduction on left eye and panhypopituitarism. Decision was made on dab/tram treatment due to a new recurrence of PCP. From September (2019) to February (2020) a reduction was recorded (cystic 12x9 to 11x7 mm and solid 3.5 mm to 2 mm; volume 6.7 ml). In June (2020) PCP volume is 0.48 ml. Thereafter stable and after 36 months of treatment the drugs were discontinued and she is now followed on MRI.

Conclusion:In the beginning of the treatment both patients had slight fever, arthralgia and headache which disappeared. Ongoing is now our Swedish study (Clin Trials, Gov) on 25 patients with PCP with BRAF V600E mutation (Swecranio1-21).

References: 1. Brastianos PK et al Nat. Gen. 2014. 2. Brastianos PC et al JNCI, 2015.

The author has declared no conflict of interest.