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Department of Radiation Oncology, University of Massachusetts Medical Center, Worcester, Massachusetts (J.V., P.R., T.F., J.G.); Penn State Hershey Medical Center, Department of Neurology, Hershey, Pennsylvania (C.D.); Massachusetts General Hospital, Department of Radiation Oncology, Boston, Massachusetts (C.G.); Pennsylvania State University College of Medicine, Hershey, Pennsylvania (M.T., M.P., C.S., D.N., M.G., J.S.); Penn State Hershey Cancer Institute, Hershey, Pennsylvania (H.M.); Penn State Medical Center, Department of Pathology, Hershey, Pennsylvania (C.S., D.N.); Penn State Hershey Medical Center, Department of Radiology, Hershey, Pennsylvania (D.N.); Penn State Neuroscience Institute, Hershey, Pennsylvania (D.N., M.G., J.S.); Northeast Radiation Oncology, Scranton, Pennsylvania (C.S.); University of Massachusetts Medical Center, Division of Neurologic Surgery, Worcester, Massachusetts (U.U., R.M.); Department of Medical Oncology, University of Massachusetts Medical Center, Worcester, Massachusetts (S.Y.).
Published: September 2016
AI Article Synopsis
Article Abstract
Craniopharyngioma is a rare tumor that is expected to occur in ∼400 patients/year in the United States. While surgical resection is considered to be the primary treatment when a patient presents with a craniopharyngioma, only 30% of such tumors present in locations that permit complete resection. Radiotherapy has been used as both primary and adjuvant therapy in the treatment of craniopharyngiomas for over 50 years. Modern radiotherapeutic techniques, via the use of CT-based treatment planning and MRI fusion, have permitted tighter treatment volumes that allow for better tumor control while limiting complications. Modern radiotherapeutic series have shown high control rates with lower doses than traditionally used in the two-dimensional treatment era. Intracavitary radiotherapy with radio-isotopes and stereotactic radiosurgery may have a role in the treatment of recurrent cystic and solid recurrences, respectively. Recently, due to the exclusive expression of the Beta-catenin clonal mutations and the exclusive expression of BRAF V600E clonal mutations in the overwhelming majority of adamantinomatous and papillary tumors respectively, it is felt that inhibitors of each pathway may play a role in the future treatment of these rare tumors.
