Effectiveness of Imatinib Mesylate Treatment in a Patient with Dermatofibrosarcoma Protuberans with Pulmonary and Pancreatic Metastases

• Published in: Internal Medicine Vol. 55; no. 17; pp. 2507 - 2511
• Main Authors: Tatai, Toshiharu, Gomi, Daisuke, Fukushima, Toshirou, Kobayashi, Takashi, Sekiguchi, Nodoka, Sakamoto, Akiyuki, Sasaki, Shigeru, Koizumi, Tomonobu, Sano, Kenji
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 01-01-2016
• Subjects: Abdomen; Antineoplastic Agents - therapeutic use; Dermatofibrosarcoma - pathology; Dermatofibrosarcoma - surgery; FISH; Humans; imatinib; Imatinib Mesylate - therapeutic use; In Situ Hybridization, Fluorescence; Lung Neoplasms - drug therapy; Lung Neoplasms - secondary; Male; metastatic DFSP; Middle Aged; Pancreatic Neoplasms - drug therapy; Pancreatic Neoplasms - secondary; PDGFB fusion gene; Platelet-Derived Growth Factor; Skin Neoplasms - pathology
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.55.6836

We herein encountered a case of abdominal wall dermatofibrosarcoma protuberans (DFSP) that developed pulmonary and pancreatic metastases 5 years after complete resection. Because specific rearrangements of the platelet-derived growth factor beta (PDGFB) locus by a novel fluorescence in situ hybridization method was detected, the patient was treated with imatinib mesylate at 400 mg/day. A partial response was achieved by imatinib without any specific toxicity. Although metastatic DFSP is an extremely rare disease, an evaluation of PDGFB fusion is essential and imatinib mesylate should be considered as an optimal therapeutic choice in patients with metastatic or locally advanced DFSP.