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.
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