Background:The proximity of craniopharyngiomas (CPs) to critical neurovascular structures can lead to a host of neurologic and endocrine complications that lead to difficulty with surgical management. In this review, we examine the molecular and genetic markers implicated in CP, their involvement in tumorigenic pathways, and their impact on CP prognosis and treatment. Methods: We undertook a focused review of relevant articles, clinical trials, and molecular summaries regarding CP. Results: Genetic and immunological markers show variable expression in different types of CP. BRAF is implicated in tumorigenesis in papillary CP (pCP), whereas CTNNB1 and EGFR are often overexpressed in adamantinomatous CP (aCP) and VEGF is overexpressed in aCP and recurrent CP. Targeted treatment modalities inhibiting these pathways can shrink or halt progression of CP. In addition, EGFR inhibitors may sensitize tumors to radiation therapy. These drugs show promise in medical management and neoadjuvant therapy for CP. Immunotherapy, including anti-interleukin-6 (IL-6) drugs and interferon treatment, are also effective in managing tumor growth. Ongoing clinical trials in CP are limited but are testing BRAF/MET inhibitors and IL-6 monoclonal antibodies. Conclusions: Genetic and immunological markers show variable expression in different subtypes of CP. Several current molecular treatments have shown some success in the management of this disease. Additional clinical trials and targeted therapies will be important to improve CP patient outcomes.
Introduction In pituitary adenomas (PAs), the use of postoperative steroid supplementation remains controversial, as it reduces peritumoral edema and sinonasal complaints but disrupts the detection of adrenal insufficiency (AI). It is unclear whether postoperative cortisol supplementation has a measurable effect on improving outcomes in patients with pituitary adenoma undergoing endoscopic transsphenoidal surgery (ETS). The objective of the study was to evaluate a postoperative steroid treatment protocol on various surgical outcomes in patients with PA undergoing ETS. Methods A retrospective cohort study was performed for patients undergoing ETS from 2005 to 2020 for PA at a single tertiary academic center. Patients were divided into two groups: those managed by a routine postoperative glucocorticoid supplementation protocol (steroid protocol) and those who received supplementation based on postoperative cortisol laboratory assessment (steroid sparing protocol). Management was otherwise the same between groups. Evaluation of length of stay (LOS), sinonasal outcomes, 30-day readmission, and perioperative complications, including AI, were performed. Results Among 535 patients, 21% (n = 111) received postoperative steroids, while the remainder (n = 424) did not. There were no differences in mean LOS (3 vs. 3 days, p = 0.72), sinonasal complaints (27 vs. 19%, p = 0.12), 30-day readmission (5% vs. 5%, p = 0.44), and perioperative complications (5 vs. 5%, p = 0.79) between both the groups. A multivariate model supported that both groups were comparable in predicting LOS, 30-day readmission, and complications. No reduction in readmission for AI was seen. Conclusion Routine administration of postoperative glucocorticoids did not significantly improve patient outcomes in patients with PA who underwent ETS.
Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA) are disabling primary headache disorders. The advent of advanced imaging technologies and surgical techniques has translated to a growing arsenal of interventional therapies capable of treating headache disorders. This literature review sheds light on the current evidence available for interventional therapies in medically intractable SUNCT/SUNA. PubMed and EMBASE were searched for publications between 1978 and 2022. Inclusion criteria were SUNCT/SUNA studies reporting outcomes following occipital nerve stimulation (ONS), pulsed radiofrequency (PRF) of sphenopalatine ganglion (SPG), stereotactic radiosurgery (SRS), deep brain stimulation (DBS) or microvascular decompression (MVD) of the trigeminal nerve. A greater than 50% reduction in severity or a greater than 50% reduction in the number of attacks was defined as a successful response. The rate of successful responses for the various treatment modalities were as follows: ONS 33/41 (80.5%), PRF of SPG 5/9 (55.6%), DBS of the ventral tegmental area 14/16 (86.7%), SRS to the SPG and/or trigeminal nerve 7/9 (77.8%) and MVD 56/73 (76.7%). Mean follow-up time in months was 42.5 (ONS), 24.8 (PRF), 25.3 (DBS), 20.8 (SRS) and 42.4 (MVD). A significant proportion of SUNCT/SUNA patients remain refractory to medical therapy (45%–55%). This review discusses existing literature on interventional approaches, including neuromodulation, radiofrequency ablation, gamma knife radiosurgery and MVD. The outcomes are promising, yet limited data exist, underscoring the need for further research to develop a robust surgical management algorithm.
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