Department of Neurological Surgery, University of Illinois at Chicago, Chicago, IL, USA.
Division of Neurosurgery, Ann and Robert H. Lurie Children's Hospital, 225 E. Chicago Ave, Box 28, Chicago, IL, 60611, USA.
Childs Nerv Syst. 2023 Apr;39(4):1083-1087. doi: 10.1007/s00381-022-05786-z. Epub 2023 Feb 1.
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.
骨软骨黏液瘤（OMX）是一种罕见的先天性骨肿瘤，仅在卡尼综合征（CNC）的背景下被描述过。作为一个独立实体以及与其他疾病相关的 OMX 的数据仍然有限，这使得诊断和治疗都变得困难。
报告了 1 例 17 岁女性患有鞍内 OMX 的病例，同时患有脊椎骨骺发育不良（SED）。我们讨论了影像学和组织病理学解释，并回顾了 OMX 的当前文献。
通过经鼻内镜经蝶窦入路成功进行了肿瘤的大体全切除。通过影像学和病理学建立了诊断。
通过组织活检可以最好地诊断和治疗 OMX。对经证实的骨软骨黏液瘤病例进行长期随访以了解复发情况和结果对于理解其自然肿瘤史并建立标准治疗方法至关重要。
