Craniopharyngiomas (CPs) are rare intracranial tumors arising from remnants of Rathke’s pouch. Despite their benign histology, CPs present considerable clinical challenges due to their tendency to exert mass effect and adherence to critical neurovascular structures. There remains no clear consensus on the most effective management of CPs. We explore the latest developments in targeted treatment approaches, examining how innovations in surgery, radiation therapy, and emerging therapies are improving outcomes and management for these challenging tumors. In addition to providing our experience, we reviewed previously reported case series and reviews relevant to CPs. Studies show a bimodal age distribution for CP diagnosis, with peak incidence occurring in children aged 5–14 years and in adults aged 50–74 years. Surgical resection is the typical initial treatment for CPs, and adjuncts, including radiation therapy and intracavitary treatments, have been proven effective for tumor control. Additionally, genetic mutations associated with CPs offer an opportunity for novel strategies that address the underlying molecular mechanisms driving tumor growth through targeting the Wnt/β-catenin and/or MAPK/ERK pathways to disrupt the aberrant signaling that promotes tumor proliferation and survival. Survival rates for CPs are generally favorable (five-year survival of 80%), with recent studies showing improved outcomes and higher survival rates in children. CPs remain rare and challenging tumors. Although surgical resection is the main treatment, surgeons must weigh the benefits of achieving a gross total resection with the risks of iatrogenic injury. Adjuncts, including intracavitary and radiation treatments, may assist with subtotal resections and recurrences, as well as approved BRAF inhibitor therapy for the papillary variant. Many improvements in diagnostic and therapeutic methods were made after Dr. Cushing coined the term “craniopharyngioma”. Ongoing experiments, investigations, and advances in radiation techniques and molecular targeted therapies will provide patients with promise for safer and more effective treatments.
Background: Tourette syndrome (TS) is a common neurological disorder characterized by frequent and disabling motor or vocal tics. There has been widely reported variation in patient responses to deep brain stimulation (DBS) for TS treatment. However, the potentially synergistic effects of multifocal DBS placements have not been extensively explored in younger patient populations. Case Description: Our patient is a 19-year-old male with a medical history significant for TS and comorbid psychological disorders. Despite medical treatment, the patient’s violent tics progressed from simple back extension motor tics to vigorous and aggressive behaviors. He received multiple opinions and trialed numerous pharmacological therapies without success. He was ultimately referred for neurosurgical evaluation for placement of DBS with selected targets of the bilateral nucleus accumbens (NAcc) and bilateral centromedian thalamus for lead implantation. The Yale Global Tic Severity Scale rated by the attending surgeon demonstrated a significant improvement in the patient’s baseline tics and overall quality of life from preoperatively to 56 months postoperatively. With multifocal DBS leads in place, the patient’s tics are well controlled on a low-moderate dosage of haloperidol. His emotional lability is now reported as less volatile and less extreme. He has become considerably more sociable and talkative. Conclusion: Following DBS placement, the patient experienced substantial improvement from his preoperative violent behavior and mood lability. This case provides evidence that dual electrode DBS, in conjunction with appropriate medical management, is a safe and effective way to improve life quality in individuals struggling with debilitating TS symptoms.
19 March 2020 in Current Otorhinolaryngology Reports