关键词: Sphenoid granular cell tumor pediatric tumor

Mesh : Humans Female Child Granular Cell Tumor / pathology diagnosis surgery Sphenoid Bone / pathology Skull Base Neoplasms / pathology diagnosis surgery Anemia, Sickle Cell / complications pathology Skull Base / pathology diagnostic imaging

来  源:   DOI:10.1080/15513815.2024.2315455

Abstract:
UNASSIGNED: Granular cell tumors occur in all ages and many anatomic sites. In the craniofacial region, they typically arise in soft tissue, not bone. We present a primary intra-osseous granular cell tumor of the sphenoid and central skull base arising in a 12- year- old girl.
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.
摘要:
颗粒细胞瘤发生在所有年龄和许多解剖部位。在颅面区域,它们通常出现在软组织中,不是骨头。我们介绍了一名12岁女孩的蝶骨和中央颅底原发性骨内颗粒细胞肿瘤。
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。
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