Introduction: Myxofibrosarcoma (MFS) is a rare subtype of a malignant soft tissue tumor that occurs mainly in adults, and peaks at the age of 70. It typically presents as a slow growing, painless mass in the proximal part of the extremities. It is characterized with a high recurrence rate and a low rate of distant metastases; the most common metastases site is the lungs, and in some extremely rare cases it was mentioned that there was metastases to the head and neck region. We here report the first case of a myxofibrosarcoma metastasized from the gluteal region to the pterygopalatine fossa, which is the first report in the literature of this rare metastatic spread of myxofibrosarcoma.
Case presentation: a 70 year-old male presented with diplopia and limited right eye movement. His medical history was significant for myxofibrosarcoma in his gluteus maximus. Magnetic resonance imaging showed a low signal mass in the pterygopalatine fossa. The tumor neither invaded the maxillary bone nor the maxillary sinus; the therapy plan was resection of the mass by partial maxillectomy followed by adjuvant radiotherapy.
Conclusion: Metastasis to the pterygopalatine fossa should be considered in a patient with myxofibrosarcoma history presents with neuro-opthalmic symptoms. Partial maxillectomy in tumors that do not infiltrate into adjacent structures should be considered as a minimally invasive therapy.
Keywords: CT, Computed tomography; Fibrosarcoma; MFH, Malignant fibrous histocytoma; MFS, Myxofibrosarcoma; MRI, magnetic resonance imaging; Metastasis; Myxofibrosarcoma; Partial maxillectomy; Pterygopalatine fossa.
© 2020 The Authors.