Dermatofibrosarcoma Protuberans, Version 1.2014

Background The incidence of rare cutaneous malignancies is unknown. Current estimates of rare cutaneous malignancy incidences are based on broad epidemiologic data or single institution experiences, not population-based data. Objective To determine the incidence of several rare non-melanoma skin cancers. Methods & Materials We conducted a retrospective chart review of a population-based cohort between the years 2000 and 2010. Residents of Olmsted County, Minnesota, who were diagnosed with a biopsy-proven non-melanoma skin cancer - excluding basal cell carcinoma and squamous cell carcinoma - were included in this study. The primary outcome was tumor incidence. Additionally, we extracted patient demographics, tumor characteristics, treatment modalities, and outcomes. Results The age- and sex-adjusted incidences per 100,000 persons of multiple rare cutaneous malignancies were: atypical fibroxanthoma (1.8), sebaceous carcinoma (0.8), dermatofibrosarcoma protuberans (0.4), microcystic adnexal carcinoma (0.7), eccrine carcinoma (0.4), eccrine porocarcinoma (0.2), and leiomyosarcoma (0.2). Conclusion We report population-based incidences and clinical characteristics for these rare cutaneous malignancies. The immune status and smoking status of patients and the treatment and outcomes of these tumors are reported. Additional studies in a broader population are needed to further define the epidemiology and outcomes of these malignancies.

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“…(36) DFSP has a low metastatic potential (1–5%)(37, 38); however, due to its recurrence potential, early surgical therapy is recommended. (39-43)…”

Section: Discussionmentioning

confidence: 99%

Incidence and Clinical Features of Rare Cutaneous Malignancies in Olmsted County, Minnesota, 2000 to 2010

et al. 2017

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“…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].…”

Section: Discussionmentioning

confidence: 99%

“…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].…”

Section: Introductionmentioning

confidence: 99%

A rare cutaneous tumor: Dermatofibrosarcoma protuberans

Allı¹,

Yorulmaz²,

Üstün³

2017

Our Dermatol Online

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“…3 The standard of care is wide local excision, however, local recurrence is not uncommon and reaches 7.3% in more recent studies. 4 In more than 90% of cases, this tumor is characterized by an unbalanced t(17:22) (q22:q13) translocation, which results in the fusion of alpha chain type 1 of collagen gene (COL1A1) and platelet-derived growth factor beta gene (PDGFB). 5 The fusion protein results in constitutively active tyrosine kinase-mediated growth factor signaling.…”

Section: Introductionmentioning

confidence: 99%

Dermatofibrosarcoma Protuberans in a Patient With Cowden Syndrome

Smith

Lan

et al. 2016

The American Journal of Dermatopathology

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PTEN hamartoma tumor syndrome, of which Cowden syndrome (CS) is the most recognized variant, is characterized by multiple benign and malignant tumors of ectodermal, mesodermal, and endodermal origins, secondary to germline mutation in the phosphatase and tensin homolog (PTEN) gene. Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive malignant fibroblastic/myofibroblastic tumor of the skin, characterized by the t(17:22)(q22:q13) translocation resulting in fusion of the COL1A1 and PDGFB genes. An association between CS and DFSP has not been reported in the literature to date. The authors have encountered a male patient with CS and a history of DFSP that developed adjacent to a sclerotic fibroma on the parietal scalp, both excised at age 7. He presented at age 21 with an enlarging pink nodule at the same site on the parietal scalp. Excision revealed a dermal and subcutaneous storiform spindle cell proliferation with fat entrapment and positive staining for CD34, consistent with DFSP. Fluorescence in situ hybridization confirmed PDGFB gene rearrangement. PTEN expression in the patient's recurrent DFSP was nearly absent when compared with that of sporadic DFSP. To our knowledge, this is the first report of DFSP in a patient with CS. Although the association is likely to be coincidental, the authors revisited the PTEN and the PDGF pathways to speculate any possible interplay of the 2 conditions on a molecular level.

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Dermatofibrosarcoma Protuberans, Version 1.2014

Cited by 31 publications

References 0 publications

Incidence and Clinical Features of Rare Cutaneous Malignancies in Olmsted County, Minnesota, 2000 to 2010

Incidence and Clinical Features of Rare Cutaneous Malignancies in Olmsted County, Minnesota, 2000 to 2010

A rare cutaneous tumor: Dermatofibrosarcoma protuberans

Dermatofibrosarcoma Protuberans in a Patient With Cowden Syndrome

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