关键词: Case report Osteochondromyxoma Pediatric neurosurgery Spondyloepiphyseal dysplasia

Mesh : Female Humans Adolescent Osteochondrodysplasias / diagnostic imaging surgery Pituitary Neoplasms / surgery Endoscopy Bone Neoplasms / diagnostic imaging surgery Mucopolysaccharidosis IV Soft Tissue Neoplasms Cartilage Diseases Treatment Outcome

来  源:   DOI:10.1007/s00381-022-05786-z

Abstract:
Osteochondromyxomas (OMX) are rare congenital bone tumors that have only been described in the context of Carney complex syndrome (CNC). Data on OMX as a separate entity and in association with other disorders remain limited, making both diagnosis and treatment difficult.
A case report of a 17-year-old female diagnosed with sellar OMX is presented in the setting of spondyloepiphyseal dysplasia (SED). We discuss the radiographic and histopathological interpretations in addition to reviewing the current literature on OMX.
A successful gross total resection of the tumor was achieved via an endonasal endoscopic transsphenoidal approach. A diagnosis was established radiographically and pathologically.
The diagnosis and treatment of OMX are best achieved via tissue biopsy. Following confirmed osteochondromyxoma cases long term for recurrence and outcomes will be essential in understanding its natural tumor history and in establishing standard treatments.
摘要:
目的:骨软骨粘液瘤(OMX)是罕见的先天性骨肿瘤,仅在卡尼复杂综合征(CNC)的背景下进行了描述。关于OMX作为一个单独的实体并与其他疾病相关的数据仍然有限,诊断和治疗都很困难。
方法:一例17岁女性诊断为鞍区OMX,病例报告为脊柱骨发育不良(SED)。除了回顾有关OMX的现有文献外,我们还讨论了放射学和组织病理学解释。
结果:经鼻窦内镜经蝶入路成功切除肿瘤。在影像学和病理学上建立了诊断。
结论:OMX的诊断和治疗最好通过组织活检来实现。经过证实的骨软骨粘液瘤病例的长期复发和结果对于了解其自然肿瘤史和建立标准治疗至关重要。
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