Abstract

Atypical fibroxanthoma (AFX) is a rare, low-grade malignant mesenchymal neoplasm of the dermis. It arises on sun-damaged skin in primarily older male patients as a solitary ulcerated nodule on the head and neck. Clinically, it grows rapidly without any pain or local invasion, and metastasis is rarely seen. Its appearance may be similar to that of other skin cancers, meaning that histopathology is necessary to confirm the diagnosis. In spite of its highly malignant histopathological features, it is widely considered as benign if strict criteria for diagnosis are followed to distinguish it from pleomorphic dermal sarcoma, which is currently considered a different entity, although both neoplasms were previously grouped together as the same pathological diagnosis [1,2]. Herein, we present a case of AFX that initially appeared malignant on the nose of a 115-year-old man. The aim of this report is to highlight the strict criteria involved in diagnosing AFX and to discuss the differential diagnosis of this benign lesion in contradistinction to the former definition as well as histopathological diagnosis, which might be of interest to clinicians.

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