Department of Pediatrics and Pediatric Hematology/Oncology, University Children's Hospital, Klinikum Oldenburg AöR, Rahel-Straus-Strasse 10, 26133, Oldenburg, Germany.
Department of Neuroradiology, University Hospital Würzburg, 97080, Würzburg, Germany.
Pituitary. 2019 Oct;22(5):532-541. doi: 10.1007/s11102-019-00983-7.
Childhood-onset craniopharyngiomas (CP) are diagnosed due to clinical symptoms (symCP) or incidentally (incCP). We investigated clinical manifestations and outcome in incCPs and symCPs.
IncCP were discovered in 4 (3 m/1 f) and symCP in 214 (101 m/113 f) CP recruited 2007-2014 in KRANIOPHARYNGEOM 2007. Age, sex, height, body mass index (BMI), tumor volume, degree of resection, pre- and postsurgical hypothalamic involvement/lesions, pituitary function and outcome were compared between both subgroups.
Reasons for imaging in incCP were cerebral palsy, head trauma, nasal obstruction, and tethered-cord syndrome, whereas headache (44%), visual impairment (25%), and growth retardation (17%) lead to imaging in symCP. Tumor volume at diagnosis was smaller in incCP (median 2.39 cm; range 0.14-4.10 cm) when compared with symCP (15.86 cm; 0.002-286.34 cm). Age, gender, BMI, height, hydrocephalus, tumor location, and hypothalamic involvement at diagnosis of incCP were within the range of these parameters in symCP. Complete resections were achieved more frequently (3/4 patients) in incCP when compared with symCP (20%). Surgical hypothalamic lesions were distributed similar in incCP and symCP. Irradiation was performed only in symCP (33%). No noticeable differences were observed concerning survival rates, endocrine deficiencies, BMI, height, functional capacity and quality of life of the 4 incCP cases when compared with the symCP cohort.
IncCP are rare (1.8%) and characterized by lack of endocrine deficiencies, resulting in normal height and BMI, no hydrocephalus, and smaller tumor volume at diagnosis when compared with symCPs. Outcome of the observed incCP is similar with symCP.
NCT01272622.
儿童期颅咽管瘤（CP）因临床症状（symCP）或偶然发现（incCP）而确诊。我们研究了 incCP 和 symCP 的临床表现和结局。
2007 年至 2014 年期间，KRANIOPHARYNGEOM 2007 招募了 4 例（3 男/1 女）incCP 和 214 例（101 男/113 女）symCP。比较了两组患者的年龄、性别、身高、体重指数（BMI）、肿瘤体积、切除程度、术前和术后下丘脑受累/损伤、垂体功能和结局。
incCP 进行影像学检查的原因是脑瘫、头部外伤、鼻塞和脊髓栓系综合征，而 symCP 进行影像学检查的原因是头痛（44%）、视力障碍（25%）和生长迟缓（17%）。incCP 的诊断肿瘤体积较小（中位数 2.39cm；范围 0.14-4.10cm），而 symCP 的肿瘤体积较大（15.86cm；0.002-286.34cm）。incCP 的年龄、性别、BMI、身高、脑积水、肿瘤位置和下丘脑受累与 symCP 患者的这些参数范围一致。incCP 患者更常进行完全切除（3/4 例），而 symCP 患者仅进行部分切除（20%）。incCP 和 symCP 患者的手术下丘脑损伤分布相似。仅对 symCP 患者进行放疗（33%）。与 symCP 队列相比，4 例 incCP 患者的生存率、内分泌缺陷、BMI、身高、功能能力和生活质量无明显差异。
incCP 较为罕见（1.8%），其特征为缺乏内分泌缺陷，导致身高和 BMI 正常，无脑积水，诊断时肿瘤体积较小。观察到的 incCP 结局与 symCP 相似。
NCT01272622。
