Bertalanffy Helmut, Gallieni Massimo, Mahmoodi Ramin, Fahlbusch Rudolf, Kar Souvik, Di Rocco Concezio
International Neuroscience Institute, Hannover, Germany.
Brain and Spinal Cord Injuries Research Center, Neuroscience Institute, Tehran University of Medical Science, Tehran, Iran.
Adv Tech Stand Neurosurg. 2025;55:17-45. doi: 10.1007/978-3-031-90762-3_2.
This paper provides an overview of optic pathway hypothalamic gliomas (OPHGs), neoplastic lesions that are mainly low-grade gliomas with predominance of pilocytic astrocytomas. They typically occur more frequently in children, but arise also in adults. While some tumors affect the optic nerve alone, the majority of them invade the hypothalamus and optic chiasm as well as adjacent structures. According to the pertinent literature, most authors consider extensive or curative tumor resection not feasible because of high risk for visual loss or severe hypothalamic side effects. Therefore, radiochemotherapy is a widely used modality to treat these challenging lesions. The authors of the present article describe a different strategy of selecting and treating surgically patients with OPHG. Similar to their management of low-grade gliomas in other intracranial location, they have treated a number of OPHGs microsurgically with curative intention. The present patient series comprises 56 individuals (42 pediatric and 14 adult patients). The authors achieved microsurgical gross total (99-100% tumor volume reduction) and near total tumor resection (90-98% volume reduction) in 42 patients (75.0%). There was no surgical mortality, and the rate of complications was low. Postoperatively, the patient's visual and endocrine function remained either intact or at a very satisfactory level in the vast majority of cases. These results differ from those found in previous publications dealing with OPHGs. In the author's experience, proper patient selection played an important role in achieving a good outcome. They conclude that microsurgical management should be considered an important part of the treatment plan in OPHGs. Patients should undergo surgery in an early stage before irreversible symptoms have occurred, and gross total tumor resection should be attempted in well-selected cases. Intentional partial OPHG resection and repeat surgery may help prolonging the symptom-free and tumor progression-free intervals.
本文概述了视路下丘脑胶质瘤（OPHG），这类肿瘤性病变主要是低级别胶质瘤，以毛细胞型星形细胞瘤为主。它们通常在儿童中更常见，但也可发生于成人。虽然有些肿瘤仅累及视神经，但大多数肿瘤会侵犯下丘脑、视交叉以及相邻结构。根据相关文献，由于存在视力丧失或严重下丘脑副作用的高风险，大多数作者认为广泛或根治性肿瘤切除不可行。因此，放化疗是治疗这些具有挑战性病变的广泛应用的方法。本文作者描述了一种选择和治疗OPHG手术患者的不同策略。与他们处理其他颅内部位低级别胶质瘤的方式类似，他们以根治为目的对一些OPHG进行了显微手术治疗。本患者系列包括56例个体（42例儿童患者和14例成人患者）。作者在42例患者（75.0%）中实现了显微手术全切除（肿瘤体积减少99 - 100%）和近全切除（体积减少90 - 98%）。无手术死亡，并发症发生率低。术后，绝大多数病例患者的视力和内分泌功能保持完整或处于非常满意的水平。这些结果与先前关于OPHG的出版物中所发现的结果不同。根据作者的经验，恰当的患者选择在取得良好结果中起着重要作用。他们得出结论，显微手术治疗应被视为OPHG治疗计划的重要组成部分。患者应在不可逆症状出现之前的早期接受手术，并且在精心选择的病例中应尝试进行肿瘤全切除。故意部分切除OPHG和再次手术可能有助于延长无症状和无肿瘤进展的间隔时间。
