颅咽管瘤（CP）是一种良性脑肿瘤，占儿童颅内肿瘤的 5 - 11%。这些肿瘤经常复发并可能导致严重的发病率。术后放疗可有效控制和预防病情进展和复发。尽管神经外科取得了进步，但内分泌、视觉和神经心理并发症很常见，并显着降低了患者的生活质量。我们进行了一项回顾性研究，包括 1989 年 7 月至 2022 年 8 月期间诊断为 CP 并在医院进行随访的所有年龄小于 16 岁的患者布鲁塞尔大学。纳入了 19 名 CP 儿童，首次出现症状时的中位年龄为 7 岁，诊断时的中位年龄为 7.5 岁。诊断时的常见症状是颅内压升高（63%）、视力障碍（47%）、生长障碍（26%）、多尿/多饮（16%）和体重增加（10.5%）。作为诊断时的临床体征，11/18 的患者观察到生长障碍，诊断前中位滞后 1 年零 4 个月。眼科检查显示，27%的患者出现乳头水肿，79%的患者出现视力障碍。当发现视力障碍时，平均术前体积较高（p=0.039）。只有 6/19 的患者进行了整体手术切除。第一次神经外科手术后，83% 的患者在中位时间 22 个月内出现肿瘤复发或进展。 11 名患者（73%）接受了术后放疗。诊断时，生长激素缺乏 (GHD) 是最常见的内分泌缺陷 (8/17)，术后一年，AVP 缺乏是最常见的内分泌缺陷 (14/17)。 13% 的患者在诊断时出现肥胖，40% 的患者在手术后 6 个月出现肥胖。术后前六个月后，体重指数随时间没有显着变化 (p=0.273)。CP 是一种具有挑战性的脑肿瘤，需要多模式治疗和终身多学科随访，包括激素替代治疗。早期识别症状对于及时进行手术治疗至关重要。长期后遗症和发病率的管理是患者临床路径的关键部分。这项研究的结果强调了在诊断时和随访期间进行完整评估（眼科、内分泌、神经认知）的根本重要性，以便患者能够从最好的护理中受益。版权所有 © 2024 Magerman, Boros,普雷齐奥西、洛尔、吉利斯、德维特、海因里希、萨蒙、弗里克斯、维尔穆伦、勒布伦、布拉谢特和罗德施。

Craniopharyngiomas (CPs) are benign brain tumors accounting for 5 - 11% of intracranial tumors in children. These tumors often recur and can cause severe morbidity. Postoperative radiotherapy efficiently controls and prevents progression and recurrence. Despite advancements in neurosurgery, endocrinological, visual, and neuropsychological complications are common and significantly lower the quality of life of patients.We performed a retrospective study, including all patients younger than sixteen diagnosed with CP between July 1989 and August 2022 and followed up in Hôpital Universitaire de Bruxelles.Nineteen children with CP were included, with median age of 7 years at first symptoms and 7.5 at diagnosis. Common symptoms at diagnosis were increased intracranial pressure (63%), visual impairment (47%), growth failure (26%), polyuria/polydipsia (16%), and weight gain (10.5%). As clinical signs at diagnosis, growth failure was observed in 11/18 patients, starting with a median lag of 1 year and 4 months before diagnosis. On ophthalmological examination, 27% of patients had papillary edema and 79% had visual impairment. When visual disturbances were found, the average preoperative volume was higher (p=0.039). Only 6/19 patients had gross total surgical resection. After the first neurosurgery, 83% experienced tumor recurrence or progression at a median time of 22 months. Eleven patients (73%) underwent postsurgical radiotherapy. At diagnosis, growth hormone deficiency (GHD) was the most frequent endocrine deficit (8/17) and one year post surgery, AVP deficiency was the most frequent deficit (14/17). Obesity was present in 13% of patients at diagnosis, and in 40% six months after surgery. There was no significant change in body mass index over time (p=0.273) after the first six months post-surgery.CP is a challenging brain tumor that requires multimodal therapy and lifelong multidisciplinary follow-up including hormonal substitution therapy. Early recognition of symptoms is crucial for prompt surgical management. The management of long-term sequelae and morbidity are crucial parts of the clinical path of the patients. The results of this study highlight the fundamental importance of carrying out a complete assessment (ophthalmological, endocrinological, neurocognitive) at the time of diagnosis and during follow-up so that patients can benefit from the best possible care.Copyright © 2024 Magerman, Boros, Preziosi, Lhoir, Gilis, De Witte, Heinrichs, Salmon, Fricx, Vermeulen, Lebrun, Brachet and Rodesch.