Initial Presentation of Papillary Craniopharyngioma with BRAF Mutation Treated with Adjuvant Chemotherapy (867)

Objective: We present a rare case of BRAFV600E positive papillary craniopharyngioma treated with adjuvant chemotherapy. Background: Craniopharyngiomas are rare solid or mixed solid-cystic suprasellar tumors that arise from Rathke’s pouch. Of the two histological variants, papillary craniopharyngiomas are predominantly seen in adults, and 95% have BRAFV600E mutations. They are slow growing with symptoms of visual impairment, endocrine abnormalities, and headache, often presenting a year before diagnosis. MRI Brain shows a cystic calcified parasellar lesion. Historically, treatment includes two approaches: aggressive surgery for total resection versus conservative subtotal resection followed by adjuvant radiotherapy. In terms of recurrence, total tumor removal provides favorable results, though if unachievable adjuvant radiotherapy is also beneficial. Radiotherapy can cause endocrine, visual, cognitive abnormalities, and vascular complications such as stroke. This leads to increased long-term mortality from treatment complications rather than tumor recurrence. Targeted chemotherapy with BRAF inhibition has been reported for recurrent tumors, but not for initial treatment of papillary craniopharyngiomas. Design/Methods: Case Report Results: A 44 year old male presents with fatigue, weight gain, polydipsia, polyuria, and undetectable testosterone levels. MRI brain showed a large solid-cystic appearing suprasellar mass with surrounding edema, pressing on the optic chiasm. Neurological and ophthalmologic exams were unremarkable. Patient underwent craniotomy for subtotal resection which revealed a WHO grade I papillary craniopharyngioma with BRAFV600E mutation. The case was discussed at tumor board; the decision was made to defer radiation therapy and proceed with targeted chemotherapy with dabrafenib and trametinib. Subsequent MRI brain showed interval decrease in size of the papillary craniopharyngioma. Conclusions: This case demonstrates the initial occurrence of papillary craniopharyngioma treated with targeted adjuvant chemotherapy, which has only been reported for tumor recurrence. Established treatment of initial tumor onset with radiotherapy also has unfavorable treatment complications. Further research is indicated to establish targeted chemotherapy as an initial treatment option. Disclosure: Dr. Gopal has nothing to disclose. Dr. Thakur has nothing to disclose. Dr. Puduvalli has nothing to disclose.