Abstract
Dermatofibrosarcoma protuberans (DFSP) is a slow growing, low- to intermediate-grade dermal soft-tissue tumor. It has a high local recurrence rate but low metastatic potential. It is characterized by a uniform spindle cell arrangement, classically with a storiform pattern and CD34 immunoreactivity. The histomorphology and immunophenotype overlap with a broad range of other neoplasms. The standard treatment is complete surgical excision. The surgical procedures include wide local excision (WLE) with tumor free margins, Mohs micrographic surgery (MMS) and amputation. Unresectable DFSPs are treated with radiation therapy and/or targeted therapy. DFSP has characteristic t(17; 22) (q22; q13), resulting in a COL1A1- PDGFB fusion transcripts in more than 90% of DFSPs. Molecular detection of the gene rearrangement or fusion transcripts is helpful for the diagnosis of patients with atypical morphology and for screening candidates for targeted therapy with tyrosine kinase inhibitors. The aims of the present review are to update the clinical presentation, tumorigenesis and histopathology of DFSP and its variants for diagnosis and differential diagnosis from other benign and malignant tumors, to compare the advantages and drawbacks of WLE and MMS, to propose the baseline for selecting surgical procedure based on tumor’s location, size, stage and relationship with surrounding soft tissue and bone structures, and to provide a biologic rationale for the systemic therapy. We further propose a modified clinical staging system of DFSP and a surveillance program for the patients after surgical excision.
Highlights
Dermatofibrosarcoma protuberans (DFSP), derived from dermal fibroblasts, was initially characterized as keloid sarcoma
DFSP is characterized by stellate or spindle cells, with long, slender, ramified cell processes joined by primitive junctions, which are similar to dermal dendrocytes [2]
Since Programmed cell death 1 ligand (PD-L1) expression in the tumor cells is involved in immune escape from T cell attack [124], PD-L1 signal pathway may be a potential target for metastatic FS-DFSP
Summary
Dermatofibrosarcoma protuberans (DFSP), derived from dermal fibroblasts, was initially characterized as keloid sarcoma. It is a slow-growing, low- to intermediate-grade malignant sarcoma and frequently occurs in middle-aged adults It is composed of uniform spindle cell fascicles growing in a storiform pattern with multiple variants and with strong and diffuse CD34 immunoreactivity. Molecular detection of gene rearrangements or fusion transcripts is beneficial for the diagnosis in cases without typical morphology, and for screening patients who are candidates for using imatinib mesylate (a tyrosine kinase inhibitor that affects PDGFβR). This provides neoadjuvant targeted therapy for the patients with unresectable, recurrent or metastatic DFSP [3]. We propose a modified staging system of DFSP for clinical practice and a surveillance program to monitor local recurrence and metastasis after surgical excision
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