Two cases of metastatic dermatofibrosarcoma protuberans treated with imatinib mesylate

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

“…The clinical outcomes of imatinib treatment for metastatic or advanced DFSP in Japanese cases that have been reported with English abstracts are summarized in Table. Although PDGFB fusion was not always consistent with a good response to imatinib, the obtained clinical outcomes were promising and similar to the results reported from other countries (12)(13)(14). Several clinical studies and case reports (3,(12)(13)(14)(15)(16)(17)(18) suggested that that a moderate dosage of 400-600 mg/day appeared to be as equally as effective as a higher dosage (800 mg/day) and was better tolerated. Since our case responded to 400 mg of imatinib without any toxicity, the therapy was continued with the same dosage.…”

“…Thus, imatinib therapy is a good option for unresectable, recurrent, or metastatic DFSP. Several clinical studies have demonstrated the efficacy of imatinib (12)(13)(14), however, little information is available regarding the usefulness of imatinib in Japanese patients with metastatic DFSP (15)(16)(17)(18). We encountered a case of abdominal wall DFSP that developed pulmonary and pancreatic metastases 5 years after complete resection.…”

“…Laboratory findings showed no abnormalities, including tumor markers. 18 FFluorodeoxy glucose positron emission tomography (FDG-PET) was performed and showed an accumulated uptake in the pulmonary and pancreas masses. No other areas showing an abnormal uptake on FDG-PET were observed.…”