Acral FibroChondroMyxoid tumor: imaging features of a new entity

“Acral FibroChondroMyxoid tumor” (AFCMT) is a recently described distinctive subtype of acral soft tissue tumor that typically arises on the fingers and toes. We herein present the unreported imaging features of AFCMT in a 44-year-old woman. This otherwise healthy patient was referred for a painful, slow-growing, soft tissue mass in the middle finger of her right hand. Initial radiographs and computed tomography showed a small lesion centered in the soft tissue of the ulnar aspect of the proximal phalanx, associated with scalloping of the underlying bone. Magnetic resonance imaging confirmed the presence of a well-circumscribed soft tissue tumor that exhibited relatively high T2-weighted signal intensity and marked enhancement after contrast administration. Subsequent excisional biopsy was performed. Histologically, the tumor was characterized by an abundant stroma displaying fibrous, chondroid, and myxoid areas. By immunohistochemistry, tumor cells stained for CD34 , ERG , and focally S100 protein. RNA-sequencing allowed detection of THBS1-ADGFR5 gene fusion which confirmed the diagnosis of AFCMT. At 2-year follow-up, the patient remains free of recurrence. AFCMT is a previously unrecognized entity that may mimic chondroma and should be considered in the differential diagnosis of soft tissue tumors with cartilaginous or myxoid stroma in the extremities.