J Endocrinol Invest. 2014 Mar;37(3):219-28. doi: 10.1007/s40618-013-0050-9.
Craniopharyngiomas are rare epithelial tumours arising along the path of the craniopharyngeal duct. Their pathogenesis remains uncertain and they can present with a variety of manifestations attributed to pressure effects to surrounding structures. The optimal management of craniopharyngiomas remains challenging mainly due to their sharp, irregular borders and their tendency to adhere to vital neurovascular structures making surgical manipulations potentially hazardous to vital brain areas. Non-aggressive surgery followed by radiotherapy is currently the most widely used option possibly achieving the most optimal long-term outcome. Other treatment modalities including intracystic irradiation, intracystic instillation of antineoplasmatic agents and stereotactic radiotherapy are also available in our armamentarium. The long-term morbidities related with the craniopharyngiomas and their treatment remain significant, with hypothalamic damage playing the protagonist role and requiring further studies to identify measures that will improve the prognosis of the patients.
颅咽管瘤是沿着颅咽管发生的罕见上皮性肿瘤。其发病机制尚不确定,可表现为多种因周围结构受压所致的症状。颅咽管瘤的最佳治疗方法仍然具有挑战性,主要是因为其边界锐利、不规则,且有向重要的神经血管结构黏附的倾向,使得手术操作对重要的脑区可能存在潜在危险。目前,最广泛应用的治疗方法是采用非侵袭性手术联合放疗,这种方法可能获得最佳的长期效果。其他治疗方法包括囊内放疗、囊内抗肿瘤药物灌注和立体定向放疗也可应用于我们的治疗方案中。颅咽管瘤及其治疗相关的长期并发症仍然很显著,其中下丘脑损伤起着主要作用,需要进一步研究以确定能改善患者预后的措施。