Dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans is a rare, low- to intermediate-grade cutaneous soft tissue sarcoma originating in the dermis and subcutaneous tissues Most frequently developing on the trunk and proximal extremities of young to middle-aged adults, it typically manifests as an indurated, slow-growing cutaneous plaque that eventually progresses into multinodular, violaceous or flesh-colored protuberant masses

Dermatofibrosarcoma protuberans is a rare, low- to intermediate-grade cutaneous soft tissue sarcoma originating in the dermis and subcutaneous tissues

Dermatofibrosarcoma protuberans is a rare, low- to intermediate-grade cutaneous soft tissue sarcoma originating in the dermis and subcutaneous tissues Histologically characterized by CD34-positive spindle cells arranged in a distinctive storiform pattern, the neoplasm demonstrates infiltrative, tentacle-like local growth patterns that yield high rates of local recurrence despite a low propensity for distant metastasis

Most frequently developing on the trunk and proximal extremities of young to middle-aged adults, it typically manifests as an indurated, slow-growing cutaneous plaque that eventually progresses into multinodular, violaceous or flesh-colored protuberant masses

Pathophysiologically, more than 90% of cases are driven by a chromosomal translocation, t(17;22)(q22;q13), or supernumerary ring chromosomes that fuse the *COL1A1* and *PDGFB* genes, generating continuous autocrine activation of the platelet-derived growth factor receptor-beta pathway

Pathophysiologically, more than 90% of cases are driven by a chromosomal translocation, t(17;22)(q22;q13), or supernumerary ring chromosomes that fuse the *COL1A1* and *PDGFB* genes, generating continuous autocrine activation of the platelet-derived growth factor receptor-beta pathway Histologically characterized by CD34-positive spindle cells arranged in a distinctive storiform pattern, the neoplasm demonstrates infiltrative, tentacle-like local growth patterns that yield high rates of local re...