Gorsi Hamza, Marupudi Neena I, Sood Sandeep, Altinok Deniz, Yankelevich Maxim
Division of Pediatric Hematology/Oncology, Children's Hospital of Michigan, Detroit, Michigan, USA.
Pediatric Neurosurgery, Children's Hospital of Michigan, Detroit, Michigan, USA.
Pediatr Neurosurg. 2020;55(1):51-53. doi: 10.1159/000503639. Epub 2019 Oct 29.
The mitogen-activated protein kinase (MAPK) pathway consists of the Ras/Raf/MEK/ERK signaling cascade, and its upregulation plays a major role in the pathogenesis of pediatric astrocytomas and molecular inhibitors of this pathway including trametinib and dabrafenib have been tested in early-phase clinical trials and used by pediatric oncologists in children with BRAF-mutated gliomas. We report a clinical case where a child with progressive BRAF-mutated glioma developed an uncommon and difficult to manage complication - pneumocephalus from intracranial air entry and trapping through dehisced surgical wounds and preexisting skull burr holes. The patient's wound breakdown coincided with skin toxicity from MEK inhibitor therapy. With increasing use of targeted molecular inhibitors in pediatric neuro-oncology, this case illustrates the potentially complicated course of MEK inhibitor therapy in patients with scalp surgical wounds and burr holes that were placed within few weeks from initiation of drug therapy, especially if patients have additional factors that can contribute to poor wound healing such as use of steroids and malnutrition.
丝裂原活化蛋白激酶（MAPK）通路由Ras/Raf/MEK/ERK信号级联组成，其上调在小儿星形细胞瘤的发病机制中起主要作用，该通路的分子抑制剂包括曲美替尼和达拉非尼已在早期临床试验中进行了测试，并被小儿肿瘤学家用于治疗BRAF突变型胶质瘤的儿童。我们报告了一例临床病例，一名患有进行性BRAF突变型胶质瘤的儿童出现了一种罕见且难以处理的并发症——通过裂开的手术伤口和先前存在的颅骨钻孔导致颅内空气进入和滞留而引起的气颅。患者的伤口裂开与MEK抑制剂治疗引起的皮肤毒性同时出现。随着靶向分子抑制剂在小儿神经肿瘤学中的使用增加，该病例说明了在开始药物治疗后几周内有头皮手术伤口和钻孔的患者中，MEK抑制剂治疗可能出现复杂的过程，特别是如果患者有其他可能导致伤口愈合不良的因素，如使用类固醇和营养不良。
