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Histopathologically, DF is characterized by the storiform proliferation of monomorphic fibroblast\u2010like spindle cells. In addition to neoplastic cells, secondary elements such as foamy histiocytes, Touton\u2010type giant cells, lymphoplasmacytes, and epidermal hyperplasia are characteristic histological features. Several histological variants, including atypical, cellular, aneurysmal, and lipidized variants, have been reported; cases with variant histologies are sometimes misdiagnosed as sarcomas. We present a case of metastasizing aneurysmal DF that was initially diagnosed as an angiosarcoma on biopsy. A 26\u2010year\u2010old woman was referred to our hospital with a gradually enlarging subcutaneous mass in her lower left leg. Positron emission tomography\u2010computed tomography revealed high fluorodeoxyglucose uptake not only in the tumor but also in the left inguinal region. On biopsy, ERG and CD31\u2010positive atypical spindle cells proliferated in slit\u2010like spaces with extravasation, leading to the diagnosis of angiosarcoma. Histology of the wide\u2010resection specimen was consistent with DF, and lymph node metastasis was also observed. Nanopore DNA sequencing detected <jats:italic>CD63::PRKCD<\/jats:italic> fusion and copy number gain, although CD63 was not included in the target region of adaptive sampling. This report highlights the importance of recognizing the unusual clinical, radiological, and pathological features of DF to avoid misdiagnosis, and the potential diagnostic utility of nanopore sequencer.<\/jats:p>","DOI":"10.1002\/gcc.23246","type":"journal-article","created":{"date-parts":[[2024,5,15]],"date-time":"2024-05-15T10:15:53Z","timestamp":1715768153000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Metastasizing aneurysmal dermatofibroma initially diagnosed as angiosarcoma confirmed by <scp><i>CD63::PRKCD<\/i><\/scp> fusion gene detection with nanopore sequencing"],"prefix":"10.1002","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-2362-7179","authenticated-orcid":false,"given":"Naoki","family":"Takeda","sequence":"first","affiliation":[{"name":"Division of Surgical Pathology Chiba Cancer Center  Chiba 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