Ottenhausen Malte, Rumalla Kavelin, La Corte Emanuele, Alalade Andrew, Nair Prakash, Forbes Jonathan, Ben Nsir Atef, Schwartz Theodore H
Department of Neurological Surgery, Weill Cornell Medical College, New York, NY, USA.
Department of Neurological Surgery, Weill Cornell Medical College, New York, NY, USA -
J Neurosurg Sci. 2019 Feb;63(1):83-87. doi: 10.23736/S0390-5616.17.04171-6. Epub 2017 Sep 4.
Craniopharyniomas arise from the sellar region and are particularly challenging because of their close proximity to critical neurovascular structures, including cranial nerves, brainstem, internal carotid arteries, posterior cerebral arteries, hypothalamus, and the pituitary gland. The tumors are benign on histology but can cause serious symptoms by compression of surrounding vital structures. While radical surgery and gross total resection (GTR) remains the first line treatment, choosing the optimal surgical approach is critical for the minimization of complications and postoperative morbidity. The extended endoscopic endonasal approach (EEEA) offers a direct route towards the sellar and suprasellar regions and has proven to be a safe and effective for GTR, with lower rates of complications and postoperative morbidity than open transcranial approaches. However, traditional transcranial approaches remain relevant in tumors with significant lateral extension. Radiotherapy can be considered in cases of subtotal resection (STR) or if surgery is not possible, although progressive growth of cysts with neurological deterioration has been demonstrated no infrequently. Newly developed chemotherapy for papillary craniopharygniomas might expand treatment options in the future.
颅咽管瘤起源于鞍区,由于其紧邻关键神经血管结构,包括颅神经、脑干、颈内动脉、大脑后动脉、下丘脑和垂体,因而极具挑战性。这些肿瘤组织学上为良性,但可通过压迫周围重要结构而引发严重症状。虽然根治性手术和全切除(GTR)仍是一线治疗方法,但选择最佳手术入路对于将并发症和术后发病率降至最低至关重要。扩大经鼻内镜入路(EEEA)为通向鞍区和鞍上区提供了直接路径,已被证明对GTR安全有效,与开颅经颅入路相比,并发症和术后发病率更低。然而,传统经颅入路在肿瘤有明显外侧扩展的情况下仍具有相关性。在次全切除(STR)病例或无法进行手术时可考虑放疗,尽管囊肿渐进性生长伴神经功能恶化的情况并不少见。新开发的针对乳头状颅咽管瘤的化疗可能在未来扩大治疗选择。