Department of Neurosurgery, Huashan Hospital, 12 Wulumuqi Zhong Road, Fudan University, and Shanghai Neurosurgical Center, Shanghai 200040, China.
J Clin Neurosci. 2011 Oct;18(10):1419-21. doi: 10.1016/j.jocn.2011.01.035. Epub 2011 Jul 20.
Chondromyxoid fibroma (CMF) is an uncommon tumor that primarily develops in the long bones of young males. It is rarely seen in the skull and involvement of the skull base is rare. We report a patient with CMF arising in the region of the sella turcica. A literature review regarding the clinical and histological features of CMF, as well as recommended modalities of treatment, is presented. A 55-year-old male was admitted with polyuria and headache. A CT scan showed a well-defined expansive lesion with a sclerotic margin measuring approximately 2 cm in diameter in the sellar region. T1-weighted MRI revealed a well-circumscribed, lobulated and strongly enhancing lesion. On the T2-weighted MRI, the lesion showed high heterogeneous signal intensity. Using the trans sphenoidal approach, surgical exploration revealed a well-defined tumor underneath the optic chiasm. The piecemeal tumor removal was considered complete. We conclude that sellar region CMF can cause severe disabilities due to tumor compression. CMF should be taken into consideration in the differential diagnosis of a solitary tumor mass in the sellar area. As much as possible, surgical resection of the tumor is the cornerstone of treatment. Although CMF are generally regarded as benign neoplasms, they may show an infiltrative pattern and may recur; particularly when they are in locations where complete surgical excision may be difficult or impossible.
软骨黏液样纤维瘤(CMF)是一种少见的肿瘤,主要发生于年轻男性的长骨。颅骨中很少见,颅底受累更为罕见。我们报告了一例发生于蝶鞍区的 CMF 患者。本文就 CMF 的临床和组织学特征以及推荐的治疗方式进行了文献回顾。一名 55 岁男性因多尿和头痛入院。CT 扫描显示蝶鞍区有一个直径约 2 厘米的边界清楚的膨胀性病变,边缘硬化。T1 加权 MRI 显示边界清楚的分叶状、强烈增强的病变。经蝶窦入路手术探查显示视交叉下方有一个界限清楚的肿瘤。肿瘤部分切除。我们的结论是,蝶鞍区 CMF 可因肿瘤压迫导致严重残疾。在蝶鞍区单发肿瘤的鉴别诊断中应考虑到 CMF。尽可能地进行肿瘤切除术是治疗的基石。尽管 CMF 通常被认为是良性肿瘤,但它们可能表现出浸润性模式并可能复发;尤其是当它们位于完全切除可能困难或不可能的部位时。