Zhang Yi, Deng Kan, Zhu Huijuan, Lu Lin, Pan Hui, Ma Wenbin, Wang Renzhi, Yao Yong
Department of Neurosurgery, Peking Union Medical College Hospital, Beijing, China.
Department of Endocrinology, Peking Union Medical College Hospital, Beijing, China.
World Neurosurg. 2019 Feb;122:e472-e479. doi: 10.1016/j.wneu.2018.10.082. Epub 2018 Oct 24.
Sellar germ cell tumors (GCTs) occur more frequently in childhood. Some will present as malignancy with infiltration and metastasis. However, the association between the timeliness of diagnosis and outcome has been controversial. We investigated the clinical risk factors associated with a diagnostic delay in patients with sellar GCTs in China.
The data from 53 patients aged <18 years with histologically confirmed sellar GCTs at Peking Union Medical College Hospital treated from January 2008 to December 2016 were reviewed retrospectively.
The median interval between symptom onset and diagnosis was 25 months. Of the 53 patients, 44 (83%) had a delayed diagnosis. Most patients (86%) with a delayed diagnosis presented with polyuria or polydipsia. Of the 53 patients, 48 (91%) exhibited changes in the pituitary stalk. Patients with a germinoma (odds ratio, 4.1; 95% confidence interval, 2.4-6.9) and slow growth (odds ratio, 5.3; 95% confidence interval, 1.2-24.5) were more likely to have a delayed diagnosis. The overwhelming majority of patients with a delayed diagnosis (96%) had been seen by >1 doctor. No statistically significant differences were found in the mean survival time (P = 0.21) or mean progression-free survival time (P = 0.36) between patients with and without delay in diagnosis, respectively.
A significant proportion of patients with sellar GCTs will experience a delay in the time to diagnosis. Although a delay in diagnosis did not reduce the survival time or progression-free survival time for patients with sellar GCTs, it might increase the risk of short stature. Thus, a detailed medical history and an immediate radiological examination are important for the early diagnosis of sellar GCTs in childhood.
鞍区生殖细胞肿瘤(GCTs)在儿童期更为常见。有些会表现为恶性,伴有浸润和转移。然而,诊断及时性与预后之间的关联一直存在争议。我们调查了中国鞍区GCTs患者诊断延迟相关的临床危险因素。
回顾性分析2008年1月至2016年12月在北京协和医院接受治疗的53例年龄<18岁、组织学确诊为鞍区GCTs患者的数据。
症状出现至诊断的中位间隔时间为25个月。53例患者中,44例(83%)诊断延迟。大多数诊断延迟的患者(86%)表现为多尿或烦渴。53例患者中,48例(91%)垂体柄有改变。患生殖细胞瘤的患者(优势比,4.1;95%置信区间,2.4 - 6.9)和生长缓慢的患者(优势比,5.3;95%置信区间,1.2 - 24.5)更有可能诊断延迟。绝大多数诊断延迟的患者(96%)曾就诊于>1名医生。诊断有延迟和无延迟的患者之间,平均生存时间(P = 0.21)或平均无进展生存时间(P = 0.36)分别未发现统计学显著差异。
相当一部分鞍区GCTs患者会出现诊断延迟。虽然诊断延迟并未降低鞍区GCTs患者的生存时间或无进展生存时间,但可能会增加身材矮小的风险。因此,详细的病史和立即进行影像学检查对于儿童鞍区GCTs的早期诊断很重要。