Department of Pediatrics and Pediatric Hematology/Oncology, University Children's Hospital, Carl von Ossietzky University of Oldenburg, Klinikum Oldenburg AöR, Oldenburg, Germany.
Department of Neurosurgery, Nanfang Hospital, Southern Medical University, Guangzhou, China.
PLoS One. 2024 Jul 23;19(7):e0307395. doi: 10.1371/journal.pone.0307395. eCollection 2024.
Craniopharyngiomas (CP) are histologically benign (WHO grade 1), embryonal malformations which are related to remnants of the Rathke's pouch and are located in the (peri)sellar region. Already before CP diagnosis, many patients show a reduced growth velocity and tend to present with weight gain. However, it is unknown whether patients with CP develop an increased head circumference (HC) before CP diagnosis, which could be a useful early diagnostic indicator.
For a cohort of 83 patients recruited in the multicenter studies KRANIOPHARYNGEOM 2000 and HIT-ENDO data on HC could be analyzed, based on medical records assessed in developmental monitoring visits performed at defined time points before CP diagnosis.
When comparing HC standard deviation scores (SDS) before CP diagnosis in 83 patients at defined time points between birth and 4 years of age, all HC were in the upper normal range. However, CP patients diagnosed at an age ≤4 years with initial hypothalamic involvement presented with a tendency towards an increased HC SDS early before CP diagnosis at routine medical examinations during the first 7 months of life.
We conclude that monitoring of growth and weight development including HC can lead to early CP diagnosis and treatment. This might prevent higher grades of hypothalamic involvement and lead to an improvement of quality of life after CP. Further studies on the specific value of HC as a diagnostic marker are warranted.
颅咽管瘤（CP）是组织学上良性的（WHO 分级 1 级），胚胎性畸形，与 Rathke 囊的残留物有关，位于（鞍旁）区域。在 CP 诊断之前，许多患者的生长速度已经减慢，并且往往会出现体重增加。然而，目前尚不清楚 CP 患者在 CP 诊断之前是否会出现头围（HC）增加，这可能是一个有用的早期诊断指标。
在多中心研究 KRANIOPHARYNGEOM 2000 和 HIT-ENDO 中招募的 83 名患者队列中，根据在 CP 诊断前的特定时间点进行的发育监测就诊中评估的医疗记录，可以分析 HC 数据。
在 83 名患者中，比较 CP 诊断前在出生至 4 岁之间的特定时间点的 HC 标准差评分（SDS），所有 HC 均处于正常上限范围内。然而，在≤4 岁时诊断为 CP 且最初下丘脑受累的患者，在生命的前 7 个月期间的常规医疗检查中，HC SDS 早期出现了增加的趋势，提示早期 CP 诊断。
我们得出结论，监测生长和体重发育，包括 HC，可导致早期 CP 诊断和治疗。这可能防止更高等级的下丘脑受累，并改善 CP 后的生活质量。需要进一步研究 HC 作为诊断标志物的具体价值。
