Craniopharyngioma

Abstract

An experienced multidisciplinary team is essential for the optimal treatment of patients with craniopharyngiomas and treatment strategies should be risk-adapted for prevention of serious morbidity and mortality. Surgery, possibly aiming to gross total resection, is the gold standard primary approach, but for unfavorably located tumors, with evidence of contact or infiltration of hypothalamus and optic pathways (OPs), a maximum safe refection followed by stereotactic radiosurgery (SRS) or radiotherapy (RT) provides the best benefit-to-risk profile in term of progression-free survival, overall survival, morbidity, and quality of life preservation. SRS can even be considered as an effective initial treatment option in selected patients. Considering the great precision in tumor targeting and steep fall of the irradiation dose, SRS may be associated with fewer long-term complications than other forms of RT delivery, especially when dealing with younger patients. Unfortunately, data on SRS and hypofractionated schedules is still very limited. The optimal dose for craniopharyngioma has not been defined: it probably lies between 9 and 12 Gy, but it has to be decided accordingly to tumor size, distance from OPs and previous irradiations. Single-type tumor (solid or cystic), smaller volume and higher prescription doses are associated with better results. Tumor control was 90-100%, 70-80% and 60-70% for solid, cystic and mixed tumors, respectively, in most single session and hypofractionated SRS series. Complications rate was acceptable and most frequently involved hypopituitarism e visual deterioration. When the dose to OPs cannot be kept <10-12 Gy in single session, hypofractionated SRS represent a valid alternative, if feasible; otherwise fractionated stereotactic radiotherapy should be considered. Cystic craniopharyngioma can be managed with several approaches: intracystic chemotherapy, brachytherapy or cystic fenestration/intermittent drainage; these approaches might have a complementary effect when associated with SRS in achieving the best clinical outcome. Novel radiotherapic techniques, as proton beam therapy, and oncological targeted therapy, as in BRAF-positive craniopharyngioma, might entail interesting perspectives for the next future.