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

Abstract

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.

Case report of a female child with right nasal chondromyxoid fibroma

“…being more infiltrative than CMF, illustrates the lack of fibrous component. [10,11] Our case was crucial as it infiltrated the orbit, lamina papryacea, and hard palate.…”

Chondromyxoid Fibroma of the Skull Base: Our Experience with an Elusive Disease

“…4,5,7,9,[11][12][13][20][21][22][23] The treatment of choice, whenever possible, is surgical excision. 6,11,18,21,24,25 For lesions located in the skull base, surgery is often technically difficult and associated with a high risk of morbidity and cosmetic deformity. 5,11,18 For these reasons the surgical approach to a skull base CMF consists of the excision of the soft component of the lesion and perilesional bone drilling, avoiding the onset of new neurologic deficits and minimizing the risk of major complications.…”

“…As far as skull base CMFs are concerned, given the low possibility of gross total removal, many authors have suggested a local adjuvant treatment of high-dose RT to prevent recurrence. 4,5,18,25,27 To date, there is no incontrovertible evidence to support the use of RT for the treatment of residual disease in CMF 1,2 due to the risk of malignant transformation. 13,21,28 However, to the best of our knowledge, only two cases of sarcomatous transformation of CMF have been reported in the literature: The first was a 58-yearold woman with transformation 6 years after a 50-Gy treatment for a CMF of the proximal tibia, 16 and the second was a 20-year-old man who had cervical spine CMF that underwent transformation 7 years after RT.…”

Imaging features of cartilaginous tumors of the head and neck

“…CMF of the skull and facial bones are exceedingly rare, with less than 100 cases reported in English literature. [ 32 ] The previously reported locations of craniofacial CMF are skull base, sinonasal cavity, and, less frequently, the calvarium. [ 31 , 32 ] These tumors demonstrate slow growth and tend to invade surrounding bones and soft tissue structures.…”

“…[ 32 ] The previously reported locations of craniofacial CMF are skull base, sinonasal cavity, and, less frequently, the calvarium. [ 31 , 32 ] These tumors demonstrate slow growth and tend to invade surrounding bones and soft tissue structures. Radiologic findings of CMF are nonspecific.…”