Frič Radek, König Marton, Due-Tønnessen Bernt J, Ramm-Pettersen Jon, Berg-Johnsen Jon
Department of Neurosurgery, Oslo University Hospital, Oslo, Norway.
Department of Neurology, Oslo University Hospital, Oslo, Norway.
Br J Neurosurg. 2025 Feb;39(1):52-60. doi: 10.1080/02688697.2023.2179600. Epub 2023 Feb 17.
Treatment of craniopharyngiomas (CP) is challenging due to their proximity to critical neural structures, risk of serious complications and impaired quality of life after treatment. Recurrences may occur many years after surgical resection. However, long-term outcome data are still scarce. The purpose of this retrospective study was therefore to assess the long-term results after treatment of patients with CP.
Patients surgically treated for a histologically verified CP at Oslo University Hospital between 1992 and 2015 and with at least a 5-year follow-up were included. Patients' medical records and radiological studies were reviewed.
Sixty-one patients (mean age 35.8 ± 22.2 years) were included; 18 patients (30%) were children <18 years of age. The incidence for the study period and the referral population was 1.1 cases/million/year, with trimodal peak incidence at 6, 32 and 59 years of age. The commonest presenting symptoms were visual disturbances (62%), headache (43%) and endocrine dysfunction (34%). The transcranial approach was utilized in 79% of patients. Gross total resection (GTR) was achieved in 59%. The surgical complication rate was 20%. Three patients (5%) received radiotherapy or radiosurgery after primary resection. The mean follow-up was 139 ± 76 months, with no patients lost to follow-up. Postoperatively, 59% of patients had panhypopituitarism and 56% diabetes insipidus. Eighteen patients (30%) developed tumour recurrence after a mean follow-up of 26 ± 25 months. The 10-year overall survival (OS) rate was 75%, whereas the disease-specific survival (DSS) rate was 84%, and recurrence-free survival (RFS) 61%. Subtotal resection (STR) ( = .01) and systemic comorbidity ( = .002) were associated with worse DSS.
Surgical treatment of CP, even though combined with adjuvant radiotherapy in only selected cases, provides good long-time OS and DSS, and relatively good functional outcome in long-term survivors despite postoperative morbidity, particularly endocrine dysfunction. Systemic comorbidity and STR are individual negative prognostic factors.
颅咽管瘤(CP)的治疗具有挑战性,因为其靠近关键神经结构,存在严重并发症风险且治疗后生活质量受损。复发可能在手术切除多年后发生。然而,长期结果数据仍然稀缺。因此,本回顾性研究的目的是评估CP患者治疗后的长期结果。
纳入1992年至2015年间在奥斯陆大学医院接受手术治疗且组织学确诊为CP并至少随访5年的患者。对患者的病历和影像学研究进行了回顾。
纳入61例患者(平均年龄35.8±22.2岁);18例患者(30%)为18岁以下儿童。研究期间及转诊人群的发病率为1.1例/百万/年,发病高峰呈三峰模式,分别在6岁、32岁和59岁。最常见的症状为视力障碍(62%)、头痛(43%)和内分泌功能障碍(34%)。79%的患者采用经颅入路。59%的患者实现了全切除(GTR)。手术并发症发生率为20%。3例患者(5%)在初次切除后接受了放疗或放射外科治疗。平均随访时间为139±76个月,无失访患者。术后,59%的患者出现垂体功能减退,56%的患者出现尿崩症。18例患者(30%)在平均随访26±25个月后出现肿瘤复发。10年总生存率(OS)为75%,疾病特异性生存率(DSS)为84%,无复发生存率(RFS)为61%。次全切除(STR)(=0.01)和全身合并症(=0.002)与较差的DSS相关。
CP的手术治疗,即使仅在部分病例中联合辅助放疗,也能提供良好的长期OS和DSS,并且尽管术后存在并发症,尤其是内分泌功能障碍,但长期存活者的功能结局相对较好。全身合并症和STR是个体不良预后因素。