Dermatofibrosarcoma protuberans

Abstract

Dermatofibrosarcoma protuberans is an intermediate-grade sarcoma that arises in the dermis of the trunk, proximal extremities, and less commonly, in the head and neck area of adults. Dermatofibrosarcoma protuberans has a deceptively innocuous early clinical appearance and generally exhibits an indolent course for years before entering a rapid growth phase. Its locally invasive behavior results in early recurrence if prompt, wide excision is not performed. Dermatofibrosarcoma protuberans rarely metastasizes through the bloodstream or, less often, to locoregional lymph nodes after multiple local recurrences. Fibrosarcomatous areas within the tumor result in a more aggressive course. Although wide excision is the mainstay of therapy, recent trials using the Mohs' surgical approach suggest that this technique may have a role in the future treatment of this tumor.

Imatinib Mesylate in Advanced Dermatofibrosarcoma Protuberans: Pooled Analysis of Two Phase II Clinical Trials

“…1,2 It is believed that metastases develop more commonly in DFSP harboring areas of fibrosarcoma, known as fibrosarcomatous DFSP (DFSP-FS). [3][4][5][6] If metastases occur, they often are localized in the lungs and are less commonly localized in lymph nodes.…”

Dynamic imaging of cancer growth and invasion: a modified skin-fold chamber model

“…The dorsal skinfold chamber is a widely used in vivo model for preclinical cancer research for drug testing, angiogenesis and tumor progression studies, as well as vascular leakage and intratumoral pressure measurements (Asaishi et al 1981;Boucher et al 1996;Guba et al 2002;Leunig et al 1992;ReyesAldasoro et al 2008). A suitable tumor type for the monitoring of active invasion in this window model are HT-1080 Wbrosarcoma cells, that clinically originate from connective tissue and readily invade 3D collagenous matrices (Laskin 1992;Wolf et al 2003Wolf et al , 2007.…”

A rare cutaneous tumor: Dermatofibrosarcoma protuberans

“…It differs from most of the soft tissue tumors because of the fact that it is indeed slowly progressive, locally aggresive tumor and has a high rate of recurrence rates after surgical treatment. DFSP is a rare tumor, its exact incidence is not known [1][2][3][4][5]. Recently it has been reported that the overall incidence of DFSP in United States is 4.1 per million person-years [6].…”

“…Most of the time initially painless lesion turn out to be a painful tumor. Moreover, ulceration along with discharge have been described for DFSP [1][2][3][4].…”

“…Although it may arise anywhere on the body, typical site of involvement is the trunk and proximal extremities. DFSP is also an exceptional dermatological disease in that it is associated with a genetic translocation involving chromosomes 11 and 22 [t(17;22) (q22;q13)] [1][2][3][4][5].…”