Chemaitilly Wassim, Merchant Thomas E, Li Zhenghong, Barnes Nicole, Armstrong Gregory T, Ness Kirsten K, Pui Ching-Hon, Kun Larry E, Robison Leslie L, Hudson Melissa M, Sklar Charles A, Gajjar Amar
Division of Endocrinology, Department of Pediatric Medicine, St. Jude Children's Research Hospital, Memphis, TN, USA.
Department of Epidemiology and Cancer Control, St. Jude Children's Research Hospital, Memphis, TN, USA.
Clin Endocrinol (Oxf). 2016 Mar;84(3):361-71. doi: 10.1111/cen.12964. Epub 2015 Nov 16.
To estimate the prevalence of central precocious puberty (CPP) after treatment for tumours and malignancies involving the central nervous system (CNS) and examine repercussions on growth and pubertal outcomes.
Retrospective study of patients with tumours near and/or exposed to radiotherapy to the hypothalamus/pituitary axis (HPA).
Patients with CPP were evaluated at puberty onset, completion of GnRH agonist treatment (GnRHa) and last follow-up. Multivariable analysis was used to test associations between tumour location, sex, age at CPP, GnRHa duration and a diagnosis of CPP with final height <-2SD score (SDS), gonadotropin deficiency (LH/FSHD) and obesity, respectively.
Eighty patients (47 females) had CPP and were followed for 11·4 ± 5·0 years (mean ± SD). The prevalence of CPP was 15·2% overall, 29·2% following HPA tumours and 6·6% after radiotherapy for non-HPA tumours. Height <-2SDS was more common at the last follow-up than at the puberty onset (21·4% vs 2·4%, P = 0·005). Obesity was more prevalent at the last follow-up than at the completion of GnRHa or the puberty onset (37·7%, 22·6% and 20·8%, respectively, P = 0·03). Longer duration of GnRHa was associated with increased odds of final height <-2SDS (OR = 2·1, 95% CI 1·0-4·3) and longer follow-up with obesity (OR = 1·3, 95% CI 1·1-1·6). LH/FSHD was diagnosed in 32·6%. There was no independent association between CPP and final height <-2SDS, and LH/FSHD and obesity in the subset of patients with HPA low-grade gliomas.
Patients with organic CPP experience an incomplete recovery of growth and a high prevalence of LH/FSHD and obesity. Early diagnosis and treatment of CPP may limit further deterioration of final height prospects.
评估中枢神经系统(CNS)肿瘤和恶性肿瘤治疗后中枢性性早熟(CPP)的患病率,并研究其对生长和青春期结局的影响。
对下丘脑/垂体轴(HPA)附近肿瘤和/或接受过放疗的患者进行回顾性研究。
对CPP患者在青春期开始时、促性腺激素释放激素激动剂(GnRHa)治疗结束时及末次随访时进行评估。多变量分析用于检验肿瘤位置、性别、CPP发病年龄、GnRHa治疗时长与CPP诊断分别与最终身高<-2标准差评分(SDS)、促性腺激素缺乏(LH/FSHD)和肥胖之间的关联。
80例患者(47例女性)患有CPP,随访时间为11.4±5.0年(均值±标准差)。CPP总体患病率为15.2%,HPA肿瘤后为29.2%,非HPA肿瘤放疗后为6.6%。末次随访时身高<-2SDS比青春期开始时更常见(21.4%对2.4%,P=0.005)。末次随访时肥胖比GnRHa治疗结束时或青春期开始时更普遍(分别为37.7%、22.6%和20.8%,P=0.03)。GnRHa治疗时间越长,最终身高<-2SDS的几率越高(OR=2.1,95%CI 1.0-4.3),随访时间越长,肥胖几率越高(OR=1.3,95%CI 1.1-1.6)。32.6%的患者被诊断为LH/FSHD。在HPA低级别胶质瘤患者亚组中,CPP与最终身高<-2SDS、LH/FSHD和肥胖之间无独立关联。
器质性CPP患者生长恢复不完全,LH/FSHD和肥胖患病率高。CPP的早期诊断和治疗可能会限制最终身高前景的进一步恶化。