CD34‐positive superficial myxofibrosarcoma: a potential diagnostic pitfall

BACKGROUND: Myxofibrosarcoma (MFS) arises most commonly in the proximal extremities of the elderly, where it may involve subcutaneous and dermal tissues and masquerade as benign entities in limited biopsy samples. We encountered such a case, in which positivity for CD34 and morphologic features were initially wrongly interpreted as a 'low-fat/fat-free' spindle cell/pleomorphic lipoma. Case series have not assessed prevalence of CD34 reactivity among cutaneous examples of MFS. METHODS: We performed a systematic review of our institution's experience, selecting from among unequivocal MFS resection specimens those superficial cases in which a limited biopsy sample might prove difficult to interpret. These cases were immunostained for CD34 and tabulated for clinicopathologic characteristics. RESULTS: After review of all MFS diagnoses over 5 years (n = 56), we identified a study group of superficial MFS for comparison to the index case (total n = 8). Of these, the index and three additional cases (4 of 8, 50%; 2 low, 2 high grade) demonstrated positive staining for CD34, with diffuse staining of spindled cells including cellular processes. Four additional cases showed no or equivocal/rare staining. CONCLUSIONS: CD34 positivity should be recognized as prevalent among such cases and should not be inappropriately construed as inveighing against a diagnosis of MFS in favor of benign entities.