Arnautovic Aska, Billups Catherine, Broniscer Alberto, Gajjar Amar, Boop Frederick, Qaddoumi Ibrahim
Pediatric Oncology Education Program, St. Jude Children's Research Hospital, Memphis, TN, 38105, USA.
Childs Nerv Syst. 2015 Jul;31(7):1067-77. doi: 10.1007/s00381-015-2670-1. Epub 2015 Mar 6.
Diagnosis of childhood brain tumors is delayed more than diagnosis of other pediatric cancers. However, the contribution of the most common pediatric brain tumors, lowgrade gliomas (LGG), to this delay has never been investigated.
We retrospectively reviewed cases of childhood LGG diagnosed from January 1995 through December 2005 at our institution. The pre-diagnosis symptom interval (PSI) was conservatively calculated, and its association with race, sex, age, tumor site, tumor grade, and outcome measures (survival, disease progression, shunt use, seizures, extent of resection) was analyzed. Cases of neurofibromatosis type 1 were reported separately.
The 258 children had a median follow-up of 11.1 years, and 226 (88 %) remained alive. Greater pre-diagnosis symptom interval (PSI) was significantly associated with grade I (vs. grade II) tumors (p = 0.03) and age >10 years at diagnosis (p = 0.03). Half of the 16 spinal tumors had a PSI > 6 months. PSI was significantly associated with progression (p = 0.02) in grade I tumors (n = 195) and in grade I tumors outside the posterior fossa (n = 134, p = 0.03). Among children with grade I tumors, median PSI was longer in those who had seizures (10.3 months) than in those who did not (2.5 months) (p = 0.09).
Delayed diagnosis of childhood LGG allows tumor progression. To reduce time to diagnosis, medical curricula should emphasize inclusion of LGG in the differential diagnosis of CNS neoplasm.
儿童脑肿瘤的诊断比其他儿科癌症的诊断延迟时间更长。然而,最常见的儿科脑肿瘤——低级别胶质瘤(LGG)对这种延迟的影响从未得到过研究。
我们回顾性分析了1995年1月至2005年12月在我们机构诊断的儿童LGG病例。保守计算诊断前症状间隔(PSI),并分析其与种族、性别、年龄、肿瘤部位、肿瘤分级和结局指标(生存、疾病进展、分流使用、癫痫发作、切除范围)之间的关联。1型神经纤维瘤病病例单独报告。
258名儿童的中位随访时间为11.1年,226名(88%)仍存活。诊断前症状间隔(PSI)较长与I级(与II级相比)肿瘤(p = 0.03)以及诊断时年龄>10岁(p = 0.03)显著相关。16例脊髓肿瘤中有一半的PSI>6个月。PSI与I级肿瘤(n = 195)以及后颅窝外I级肿瘤(n = 134,p = 0.03)的进展显著相关(p = 0.02)。在I级肿瘤患儿中,有癫痫发作的患儿中位PSI(10.3个月)比无癫痫发作的患儿(2.5个月)更长(p = 0.09)。
儿童LGG的诊断延迟会导致肿瘤进展。为了缩短诊断时间,医学课程应强调将LGG纳入中枢神经系统肿瘤的鉴别诊断中。