Skull Base Chondromyxoid Fibroma: A Case Report and Review of the Literature

Introduction: Chondromyxoid fibromas are rare tumors that typically occur in metaphysis of long bones in adolescents. When occurring in the head and neck, they are elusive diagnostically and more challenging to treat. There is little information on these tumors in the literature, and further discussion is needed to aid in diagnosis and improve patient outcomes.

Case Description: A 44-year-old female presented to our emergency department with subacute left eye vision changes and was found to have a large, expansile soft tissue mass of the left sphenoethmoid region with skull base and lamina papyracea erosion. Biopsy revealed a spindle cell neoplasm with myxoid features. The patient was lost to follow up for 6 months, eventually returning with complete left eye vision loss. Repeat imaging demonstrated tumor compression of the optic nerve at the orbital apex. Endoscopic resection of the tumor and optic nerve decompression was performed. Final histologic examination demonstrated a benign chondromyxoid fibroma. Three weeks postoperatively, the patient developed a contralateral Bell's palsy that resolved with steroids. At 6 weeks of follow-up, the patient had not regained vision in the left eye.

Discussion: Diagnosis and treatment of these tumors remains challenging. One of the most common locations in the head and neck is in the ethmoid and sphenoid bones, as in this case. Diagnosis requires histopathologic examination, and radiographic evidence is not particularly specific in identification. On histology, chondromyxoid fibromas are typically lobular with cellular fibrous tissue at the periphery and loose myxoid tissue in the center. Positive margins are often present given the difficulty of gross total resection. The recurrence rate for all chondromyxoid fibromas is 20%, and potentially higher in the head and neck due to difficulty obtaining clear margins. Conversion to malignant disease is very rare, though the ability to be locally destructive causes significant morbidity for these patients.

Fig. 1 The chondromyxoid fibroma displays lobular/zonal arrangement composed of scattered tumor cells in the chondromyxoid matrix. The periphery of the lobules is relatively cellular along the thin-walled vasculature (A). The tumor cells have ovoid, spindled or stellate nuclei, and eosinophilic-to-amphophilic cytoplasm with multipolar, stellate extension (B). No mature cartilage is observed. There are small foci of tumor cells resembling cartilage lacunae (C, two arrows). The appearance of microcysts containing chondromyxoid material is seen (D).

Fig. 2 T1 coronal MRI image showing the intracranial extent of the tumor contacting the medial aspect of the left optic nerve. MRI, magnetic resonance imaging.

Fig. 3 Axial view CT showing obstruction of the left nasal cavity and infiltration of left ethmoid region. CT, computed tomography.

Fig. 4 Coronal view CT showing bony erosion of the planum sphenoidale and obliteration of left nasoethmoid region and maxillary sinus. CT, computed tomography.

 

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No conflict of interest has been declared by the author(s).

Publication History

Article published online:
12 February 2021