UNASSIGNED: A 12-year-old female with sickle cell disease and Jeavons syndrome presented with seizures. Imaging and partial resection revealed an expansile benign granular cell tumor (GCT) involving the sphenoid body, pterygoid process, and central skull base. The disease has remained stable after 36-month follow up.
UNASSIGNED: GCT primarily involving the osseous sphenoid/skull base has not been previously reported in a child. Although mostly benign, some are aggressive, with malignant transformation in 1-2%. Surgery is the mainstay of treatment, but in the skull base this may be limited by adjacent critical structures. Decision-making is guided by anatomic extent, histology, and clinical behavior.
■一名12岁女性,患有镰状细胞病和Jeavons综合征,出现癫痫发作。影像学检查和部分切除术显示,涉及蝶骨体的扩张性良性颗粒细胞瘤(GCT),翼状突起,和中央颅底。经过36个月的随访,该疾病保持稳定。
■GCT主要累及骨性蝶骨/颅底,以前没有在儿童中报道过。虽然大部分是良性的,有些是侵略性的,恶性转化在1-2%。手术是治疗的主要手段,但是在颅底,这可能受到相邻关键结构的限制。决策是由解剖范围指导的,组织学,和临床行为。