Chondromyxoid Fibroma of the Skull Base and Calvarium: Surgical Management and Literature Review.

Chondromyxoid fibroma (CMF) is an exceedingly rare tumor that represents less than 1% of all primary bone neoplasms. Occurrence in the facial and cranial bones is extremely rare and frequently misdiagnosed. Case Reports We report two cases of CMF, one in the sphenoclival skull base and the other involving the parietal bone in two young female patients. Excision was performed in both cases. Presenting symptoms, treatment, and follow-up are reported. Methods A retrospective review of the literature on cranial CMF was performed. The location, demographics, presenting symptoms, and treatment of all calvarial and skull base CMF cases published since 1990 are summarized. Discussion In our literature review, we found 67 published cases of cranial CMF. Mean age of all calvarial and skull base CMFs at diagnosis was 38.2 years old. Of the cases affecting the cranium, the sinonasal structures were most commonly involved. To our knowledge we report only the second case of CMF involving the parietal bone published in an English-language journal. Total resection is the best treatment, and should be the goal of surgical intervention. Curettage results in high recurrence rates. Radiotherapy in the setting of subtotal resection or recurrence cannot be definitively recommended and needs further investigation.