Current approaches to cutaneous sarcomas: Dermatofibrosarcoma protuberans and cutaneous leiomyosarcoma

摘要: Dermatofibrosarcoma protuberans (DFSP) and cutaneous leiomyosarcoma (LMS) are 2 rare dermal sarcomas that often locally aggressive but rarely metastasize. LMS accounts for less than 3% DFSP 1% of all soft tissue sarcomas. Both malignancies slow-growing tumors; however, has a propensity to afflict white individiuals. present as firm nodules on the skin with minimal symptoms. sarcomas, especially DFSP, tend grow in an asymmetric pattern fingerlike projections without pigmentation. On biopsy, it is highly recommended dermatopathologist or sarcoma pathologist evaluate specimen, because rarity these lesions. Staging both according American Joint Committee Cancer (AJCC) guidelines (Table 1). In general, tumors superficial deep fascia 5 cm greatest dimension; such, they staged T1aN0M0 lesions, provided there no evidence metastatic spread Most lesions characteristically low grade. However, very large exophytic fibrosarcomatous changes (DFSP-FS), high-grade LMSs stage II III. Our current treatment approach multidisciplinary. As described later, role meticulous pathologic review margin analysis by just important nature which surgeon resects tumors. Although radiotherapy used definitive LMS, certainly adjuvant therapy selected cases 2). Chemotherapy (ie, gemcitabine docetaxel) other small-molecule inhibitors imatinib sorafenib) may also have some activity patients unresectable high-level lacking