ABSTRACT
Primary giant cell tumor of soft tissue (GCT-ST) is a rare entity that is considered the soft tissue equivalent of giant cell tumor of bone. It most commonly arises in soft tissues of the trunk and extremities, with occurrence in the head and neck being extremely rare. We report a case of GCT-ST of the carotid body, the first report of a tumor of this kind arising from this site in the neck. Giant cell tumor of soft tissue is generally considered a benign tumor with low malignant potential; thus, surgical excision is usually curative. However, due to the location and invasive nature of this patient's tumor, complete excision was not possible. We discuss the implication of this for long-term management of this patient, as well as similarities and differences in clinical presentation, histology, and biological behavior between this case and previously reported cases of GCT-ST.