Differential sensitivity to imatinib of 2 patients with metastatic sarcoma arising from dermatofibrosarcoma protuberans

摘要: Dermatofibrosarcoma protuberans (DFSP) is a rare superficial sarcoma usually affecting the trunk, with significant risk of local recurrence. It characterized by presence ring chromosomes or chromosomal translocations fusing promoter collagen gene COL1A1 to platelet-derived growth factor beta-chain PDGFB, increasing production PDGF locally and promoting autocrine paracrine tumor growth. Fewer than 5% patients DFSP develop metastatic sarcoma, poor subsequent prognosis. Imatinib (STI-571) was developed as an inhibitor receptor tyrosine kinase has proven clinical activity against chronic myelogenous leukemia (expressing bcr-abl) gastrointestinal stromal tumors c-kit). We describe 2 unresectable metastases from treated imatinib. After confirmation negative CD117 status sarcomas arising DFSP, were given imatinib 400 mg po qd assessed at regular intervals for their tolerance response therapy. One patient had transient response, then progressed rapidly died disease. Another showed partial therapy after months, resolution superior vena cava syndrome shrinking lung lesions. His ongoing 6 months These data confirm findings models support use in setting DFSP. may be useful advanced when other options are limited.