Atypical fibrous histiocytomas and pleomorphic fibroma-type lesions in a patient with li-fraumeni syndrome

Abstract

Atypical fibrous histiocytomas are an uncommon but benign variant of dermatofibroma where the neoplastic cells can feature cytological atypia constituted by prominent nuclear enlargement, pleomorphism and multinucleated giant cells. Though these can have a degree of atypia, they have a benign course and a failure to recognize these tumors as benign can lead to misdiagnosis and overtreatment, particularly in patients with Li-Fraumeni syndrome who are known to be predisposed to various malignancies. Here, we report a patient with atypical fibrous histiocytomas and multiple pleomorphic fibroma-type lesions in the setting of Li-Fraumeni syndrome.