■尤因肉瘤(ES)是一种由骨骼或软组织引起的恶性肿瘤,以原始的小而圆的蓝色细胞为特征。原发性ES通常发生在长骨中,椎骨,或者骨盆,在颅底极为罕见.
■一名14岁女孩表现为颈后疼痛和多个颅神经(CNs)功能障碍。放射学研究显示,岩斜骨的固体块延伸到蝶窦。患者接受内镜经蝶入路手术以诊断病理,部分切除是安全的。组织病理学,遗传,放射学检查证实了原发性ES的诊断。随后,患者接受了辅助化疗和放疗,随后临床症状得到缓解。在多模式治疗后达到完全反应。治疗后20个月,患者仍处于缓解状态,无复发或转移性疾病。文献中只有三例报道了岩骨的原发性ES。如本案所示,多个CNs的功能障碍是岩斜ES的最常见表现。诊断应通过考虑非特异性临床症状和放射学特征的组织病理学和遗传学检查来确认。
■多模式治疗,包括手术,化疗,和放射治疗,可以带来有利的结果。临床医生应在手术治疗期间考虑安全切除,以防止可能延迟术后多模式治疗的并发症。
UNASSIGNED: Ewing\'s sarcoma (ES) is a malignancy that arises from bones or soft tissue, characterized by primitive small and round blue cells. Primary ES typically occurs in the long bones, vertebrae, or pelvis, and is extremely rare in the skull base.
UNASSIGNED: A 14-year-old girl presented with posterior cervical pain and dysfunction of multiple cranial nerves (CNs). Radiological investigation revealed a solid mass of the petroclival bone extending into the sphenoid sinus. The patient underwent endoscopic transsphenoidal surgery for diagnosis of the pathology, and partial resection was safely achieved. Histopathological, genetic, and radiological examinations confirmed the diagnosis of primary ES. Subsequently, the patient underwent adjuvant chemotherapy and radiotherapy following which the clinical symptoms resolved. Complete response was achieved after multimodal treatment. Twenty months after treatment, the patient remains in remission without recurrence or metastatic disease. Primary ES of the petroclival bone has been reported in only three cases in the literature. As seen in the present case, dysfunction of multiple CNs is the most common manifestation of petroclival ES. Diagnosis should be confirmed by histopathological and genetic examinations considering the nonspecific clinical symptoms and radiological features.
UNASSIGNED: Multimodal treatment, including surgery, chemotherapy, and radiotherapy, can result in favorable outcomes. Clinicians should consider safe resection during surgical management to prevent complications that can delay postoperative multimodal treatment.